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Doke R, Lokhande R, Chande K, Vinchurkar K, Prajapati BG. Recent advances in therapeutic strategies of Erdheim-Chester disease. NAUNYN-SCHMIEDEBERG'S ARCHIVES OF PHARMACOLOGY 2025; 398:6407-6428. [PMID: 39836251 DOI: 10.1007/s00210-024-03769-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/18/2024] [Accepted: 12/26/2024] [Indexed: 01/22/2025]
Abstract
Erdheim-Chester disease (ECD) is a rare form of non-LCH characterized by excessive accumulation of histiocytes in various tissues, leading to significant morbidity. The estimated prevalence of ECD is low, with fewer than 1000 cases reported globally, yet it presents considerable clinical challenges due to its heterogeneous manifestations, which include bone pain, cardiovascular complications, and neurological symptoms. Traditional treatment approaches, primarily involving corticosteroids and chemotherapy, have limitations, including inconsistent responses and significant side effects. Recent advances in understanding the pathogenesis of ECD, particularly the role of the BRAF V600E mutation, have led to the exploration of novel therapeutic strategies, such as targeted BRAF inhibitors, MEK and mTOR inhibitors, and other immunotherapies, which offer promise in improving patient outcomes. The review further explores clinical manifestations, and radiographic features of Erdheim-Chester disease, and discusses treatment strategies, current clinical studies in the field of ECD. By integrating these aspects, this review aims to provide a thorough understanding of ECD and its evolving treatment landscape, ultimately contributing to improved patient outcomes.
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Affiliation(s)
- Rohit Doke
- Jaihind College of Pharmacy, Vadgaon Sahani, Pune, Maharashtra, 412401, India
| | - Rahul Lokhande
- Samarth Institute of Pharmacy, Belhe, Pune, Maharashtra, 412410, India
| | - Kalyani Chande
- Dr. DY Patil College of Pharmacy Akurdi, Pune, Maharashtra, 411044, India
| | - Kuldeep Vinchurkar
- Sandip Foundation's Sandip Institute of Pharmaceutical Sciences (SIPS), Nashik, Maharashtra, 422213, India.
| | - Bhupendra G Prajapati
- Department of Pharmaceutical Technology, Shree S K Patel College of Pharmaceutical Education and Research, Ganpat University, 384012, Mahesana, Gujarat, India.
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Dong ZY, Zhu HB. Idiopathic retroperitoneal fibrosis arising from peritoneal space: A case report and review of literature. World J Gastroenterol 2025; 31:105443. [DOI: 10.3748/wjg.v31.i18.105443] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2025] [Revised: 03/27/2025] [Accepted: 04/24/2025] [Indexed: 05/13/2025] Open
Abstract
BACKGROUND Retroperitoneal fibrosis is a rare fibro-inflammatory condition which can be classified into idiopathic (accounting for over 75%) and secondary types (due to malignancies, infections, medications, radiotherapy or other conditions). Idiopathic retroperitoneal fibrosis (IRPF) typically affects the abdominal aorta and iliac arteries along with the surrounding retroperitoneal area. This case review aims to summarize the imaging characteristics of IRPF arising from the peritoneal space.
CASE SUMMARY An abdominal mass was discovered in a 52-year-old man during a routine physical examination, he had not complained of abdominal pain, distension, nausea, vomiting, diarrhea, fever, and had no significant past medical or family history. Abdominal magnetic resonance imaging revealed a soft tissue mass with poorly defined margins surrounding the duodenum, exhibiting slight to moderate high signal intensity on both T1-weighted and T2-weighted images. Diffusion-weighted imaging with a β value of 800 mm²/second demonstrated slightly to moderate high signal intensity. Dynamic contrast enhanced images showed uneven enhancement on the arterial phase, with significant enhancement observed on the delayed phase. The mass infiltrated adjacent structures, including the head of the pancreas, the hepatic flexure of the colon, and part of the intestine, raising suspicion for malignant tumors such as sarcoma or lymphoma. However, surgery confirmed the diagnosis of IRPF. The patient underwent routine follow-up for one year, with no recurrence.
CONCLUSION IRPF is a rare condition that presents considerable diagnostic challenges when lesions arise from the peritoneal space. In cases where imaging findings are atypical, a further puncture biopsy may be necessary to confirm the diagnosis.
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Affiliation(s)
- Zhen-Yu Dong
- Department of Radiology, Qingdao Huangdao District People’s Hospital, Qingdao 266400, Shandong Province, China
| | - Hai-Bin Zhu
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Radiology, Peking University Cancer Hospital and Institute, Beijing 100142, China
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Peyronel F, Della-Torre E, Maritati F, Urban ML, Bajema I, Schleinitz N, Vaglio A. IgG4-related disease and other fibro-inflammatory conditions. Nat Rev Rheumatol 2025; 21:275-290. [PMID: 40195520 DOI: 10.1038/s41584-025-01240-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/07/2025] [Indexed: 04/09/2025]
Abstract
IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder usually characterized by multi-organ involvement. Its pathogenesis is complex and involves genetic and environmental factors, while immune responses usually mediate organ damage and promote fibrosis, which is a key feature of the disease. IgG4 responses, however, are not exclusive to IgG4-RD and can be encountered in other diseases with phenotypes that partially overlap that of IgG4-RD. Although IgG4-RD has clinical and histological hallmarks, the lack of validated diagnostic criteria often makes the diagnosis challenging, requiring a multi-dimensional approach that integrates clinical, radiological and serological data. The present Review covers recent advances in the understanding of disease drivers and its clinical phenotypes, mainly focusing on the differential diagnosis with potential IgG4-RD mimickers, namely histiocytoses, lymphoproliferative disorders, systemic vasculitides and other immune-mediated conditions. The Review also provides a schematic approach to IgG4-RD treatment, including a brief overview of glucocorticoid-sparing agents and emerging therapies, from B cell-depleting monoclonal antibodies to cytokine-targeting drugs, the majority of which are currently under investigation in randomized clinical trials.
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Affiliation(s)
- Francesco Peyronel
- Nephrology and Dialysis Unit, Meyer Children's Hospital IRCCS, Florence, Italy
- Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
| | - Emanuel Della-Torre
- University Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Federica Maritati
- Nephrology, Dialysis and Kidney Transplant Unit, IRCCS-Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - Maria L Urban
- Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
| | - Ingeborg Bajema
- Department of Pathology and Medical Biology, University Medical Centre Groningen, Groningen, The Netherlands
| | - Nicolas Schleinitz
- Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Université, Department of Internal Medicine Hôpital Timone, Marseille, France
| | - Augusto Vaglio
- Nephrology and Dialysis Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
- Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Florence, Florence, Italy.
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Ji Z, Sun Y, Zhang L, Kong X, Jiang L. Predictors of irreversible renal dysfunction in patients with idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 2025; 64:2855-2861. [PMID: 39460947 DOI: 10.1093/rheumatology/keae565] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2024] [Revised: 09/10/2024] [Accepted: 09/22/2024] [Indexed: 10/28/2024] Open
Abstract
OBJECTIVES Idiopathic retroperitoneal fibrosis (iRPF) can lead to irreversible kidney damage. This study aimed to investigate predictors of irreversible renal dysfunction in patients with iRPF. METHODS Eighty-three patients with newly diagnosed iRPF were enrolled between January 2010 and September 2022 at Zhongshan Hospital of Fudan University, including 60 in the training set and 23 in the validation set. They were regularly contacted or followed up via outpatient examinations by specialist doctors, who documented their condition and treatment progress. Predictors of irreversible renal dysfunction were identified using univariate and multivariate regression, logistic model, and receiver operating curve analyses. RESULTS In the training set, over a median follow-up of 29 months, 16.7% of patients had an estimated glomerular filtration rate (eGFR) of <60 ml/min/1.73 m2 at the last follow-up, and 25% had hydronephrosis or required prolonged double-J stents. A prognostic score was developed by assigning 1, 1, and 2 points for peripheral CD19+ B cells <9.3%, serum creatinine (sCr) ≥120 µmol/L, and no response at 6 months, respectively. A score of ≥2 for predicting irreversible renal dysfunction had sensitivity and specificity of 100% and 92%, respectively. In the validation set, 21.7% of patients suffered from irreversible renal dysfunction. The sensitivity and specificity for predicting irreversible renal dysfunction were 100% and 94.4%, respectively. CONCLUSIONS A prognostic score based on factors including CD19+ B cells <9.3% and sCr ≥120 µmol/L at baseline, and no response at 6 months, is suitable for predicting irreversible renal dysfunction in iRPF.
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Affiliation(s)
- Zongfei Ji
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Ying Sun
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Lijuan Zhang
- Department of Rheumatology, Xiamen Branch, Zhongshan Hospital, Fudan University, Xiamen, China
| | - Xiufang Kong
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Lindi Jiang
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
- Evidence-Based Medicine Center, Fudan University, Shanghai, China
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Czerniak S, Mathur M. Multimodality imaging review of retroperitoneal fibrosis. Abdom Radiol (NY) 2025:10.1007/s00261-025-04847-6. [PMID: 40035807 DOI: 10.1007/s00261-025-04847-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2024] [Revised: 02/10/2025] [Accepted: 02/11/2025] [Indexed: 03/06/2025]
Abstract
Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disease with idiopathic and secondary causes. Idiopathic disease is more common and is believed to be immune mediated; associations with autoimmune diseases and/or inflammatory disorders such as IgG4 related disease are often present. Common complications include hydronephrosis and venous stenosis and/or thrombosis. Due to its nonspecific clinical presentation, imaging is vital for diagnosis; in addition, imaging may help distinguish idiopathic from secondary causes and can aid in distinguishing early/active disease from chronic/inactive disease. Magnetic resonance imaging is the preferred imaging modality to stage and monitor the disease, though CT and PET/CT imaging may also be of value. While the imaging findings can overlap with other diseases, there are some characteristic findings which can favor RPF. However, a biopsy is needed for a definitive diagnosis.The following article discusses the clinical features, imaging appearances across modalities, associated complications, potential diagnostic pitfalls, and treatment approaches for RPF. The role of advanced imaging techniques, such as diffuse weighted imaging and 18F-FDG PET/MRI, in the evaluation of RPF will also be included.
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Affiliation(s)
- Suzanne Czerniak
- Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, USA
| | - Mahan Mathur
- Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, USA.
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Steimer A, Becker MO. Treatment approaches for idiopathic retroperitoneal fibrosis: a systematic review with meta-analysis. BMC Rheumatol 2025; 9:12. [PMID: 39910636 PMCID: PMC11800648 DOI: 10.1186/s41927-024-00445-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/29/2024] [Accepted: 12/05/2024] [Indexed: 02/07/2025] Open
Abstract
BACKGROUND Currently, there is no standard therapy for idiopathic retroperitoneal fibrosis, so a systematic review was undertaken to assess the effectiveness of different treatment approaches. METHODS A comprehensive search of English and German literature from 1980 to 2021 was conducted using PubMed, Embase, and PreMedline. To be included, studies must have had a minimum of two patients employing the same treatment approach and reporting relevant treatment outcomes. A meta-analysis with a subgroup analysis was conducted for the primary outcomes "regression of fibrosis," "freedom from ureteric stents" and "relapse rate," and the secondary outcome "clinical improvement." The lack of homogeneous data prevented a subgroup analysis for the primary outcome "improvement in renal function." RESULTS The search resulted in a total of 3818 articles, of which 108 were selected for qualitative analysis involving a total of 1408 patients. For the meta-analysis 83 studies were included involving 1044 patients. The summary effect size of the outcomes "regression of fibrosis," "freedom from ureteric stent" and "clinical improvement" was high with values between 80-97.9%. The summary relapse rate across studies was 18.1%. Subgroup analysis revealed no statistically significant differences in the effectiveness of treatment approaches for the outcomes "regression of fibrosis" (QM = 2.72, p = 0.74), "freedom from ureteric stent" (QM = 7.21, p = 0.13), "relapse rate" (QM = 11.34, p = 0.08) and "clinical improvement" (QM = 9.54, p = 0.15). CONCLUSIONS Considering the lack of clear evidence indicating that one drug is more effective than the other, the treatment choice should depend on factors such as the potential side effects of different drug therapies, patient comorbidities, and clinician expertise. The review protocol is registered on PROSPERO under the identification number CRD42019115744.
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Affiliation(s)
- Annik Steimer
- Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
- Center for Experimental Rheumatology, University of Zurich, Zurich, Switzerland
| | - Mike O Becker
- Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
- Center for Experimental Rheumatology, University of Zurich, Zurich, Switzerland.
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Hu JQ, Jin ZY, Yu YY, Min DM, Cai Q, Gao J. Clinical characteristics of IgG4-related retroperitoneal fibrosis in a cohort of 117 patients with idiopathic retroperitoneal fibrosis: a retrospective study. Clin Rheumatol 2025; 44:757-766. [PMID: 39755817 DOI: 10.1007/s10067-024-07276-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2024] [Revised: 10/22/2024] [Accepted: 12/11/2024] [Indexed: 01/06/2025]
Abstract
OBJECTIVE Retroperitoneal fibrosis (RPF) is a rare condition marked by inflammation and fibrosis affecting the peritoneal and retroperitoneal soft tissues. In recent years, the identification of IgG4-related diseases has brought to light a significant association with fibrous disorders, including RPF, which were once considered independent. In this comprehensive cohort study, we performed a comparative analysis of the demographic, clinical, laboratory, histopathological, and therapeutic characteristics between patients with IgG4-related RPF and those with idiopathic retroperitoneal fibrosis (iRPF). METHODS We performed a retrospective analysis of 117 patients diagnosed with RPF at the First Affiliated Hospital of Naval Medical University between July 2007 and July 2023. RESULTS Demographic, clinical, laboratory, histopathological, and therapeutic characteristics of 70 iRPF patients and 47 IgG4-related patients were systematically compared. The IgG4-related group exhibited an older age of onset, with a predominant occurrence among adult males. Significantly elevated levels of eosinophilia and IgE were observed in the IgG4-related group. Most patients across both groups displayed elevated CRP and ESR levels. Furthermore, at the time of diagnosis, the IgG4-related group had higher serum creatinine and lower levels of complement. The most prevalent clinical manifestation in both groups was flank pain. The proportion of lymphoplasmic infiltration and storiform fibrosis in IgG4-related RPF group was significantly higher. The IgG4-related RPF group had significantly higher IgG4-positive plasma cell count, IgG4/total IgG ratio, and eosinophils count than that in iRPF group. CONCLUSION We conducted a comparative analysis of demographic, clinical, laboratory, histopathological, and therapeutic differences between the iRPF patients and the IgG4-related patients. Clarifying the distinctive characteristics of these two groups will contribute to a better understanding of the condition and facilitate the development of specific treatment strategies tailored to each group. Key Points • Identification of distinct clinical features and outcomes between IgG4-related and iRPF patients in a large retrospective study.
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Affiliation(s)
- Jia-Qi Hu
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China
| | - Zheng-Yi Jin
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China
| | - Yi-Yi Yu
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China
| | - Du-Mu Min
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China
| | - Qing Cai
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China.
| | - Jie Gao
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China.
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Huang Z, Liu Y, Xiao Y, Hu H, Xu T. Idiopathic Retroperitoneal Fibrosis-Related Hydronephrosis: Evaluation of Comprehensive Management and Prediction of Inflammatory Markers for Stent-Free Outcomes. Int J Gen Med 2025; 18:113-121. [PMID: 39816639 PMCID: PMC11733166 DOI: 10.2147/ijgm.s490245] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2024] [Accepted: 12/28/2024] [Indexed: 01/18/2025] Open
Abstract
Objective This study investigated the efficacy of comprehensive management and predictable inflammatory markers for idiopathic retroperitoneal fibrosis (iRPF)-related hydronephrosis outcomes. Methods Patients with iRPF-related hydronephrosis underwent surgical (ureteral stent and/or nephrostomy tube placement) and medical (corticosteroid-based multiple immunosuppressants) management were classified according to stent-indwelling outcomes. Univariate analysis of clinical profiles was conducted to screen possible predictors of hydronephrosis remission. Results In a series of 38 patients, 52.6% achieved hydronephrosis remission and stent/tube removal (stent-free group). The median indwelling time in the stent-free group (12 months) was significantly lower than that in the treatment-failure group (37 months, p<0.05). Mean retroperitoneal mass diameters was significantly reduced (anteroposterior by 11.66 mm (95% CI 2.31-21.01), transverse by 15.41 mm (95% CI 3.37-27.46), suprainferior by 30.53 mm (95% CI 4.87-56.19); p<0.05) during the treatment course, in line with mean renal pelvis width (by 36.2%) and renal function parameters (serum creatinine by 16.9%, blood urea nitrogen by 12.9%). Renal function improved (36.9%) or remained stable (44.7%) in most patients, the mean estimated glomerular filtration rate increasing by 8.7% (from 55.4 mL/min/1.73 m2 to 60.2 mL/min/1.73 m2). At the initial diagnosis, median serum immunoglobulin IgG and CRP levels were significantly higher in the stent-free group than in the treatment-failure group (IgG 17.55 g/L vs. 13.50 g/L, CRP 19.60 mg/L vs. 3.15 mg/L; p<0.05). Decline in serum IgG (-5.80 g/L vs. -2.30 g/L), CRP (-18.93 mg/L vs. -1.72 mg/L) and erythrocyte sedimentation rate (-22.00 mm/h vs. -1.50 mm/h) levels in the stent-free group surpassed those in the treatment-failure group (p<0.05). Conclusion Comprehensive management benefits iRPF patients with hydronephrosis by preserving renal function. The 24-month scale might guide stent/tube removal. Elevated inflammatory markers (IgG and CRP) at the initial iRPF diagnosis and IgG, CRP, and erythrocyte sedimentationrate (ESR) variations associated with hydronephrosis outcomes.
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Affiliation(s)
- Zixiong Huang
- Department of Urology, Peking University People’s Hospital, Beijing, 100044, People’s Republic of China
- Applied Lithotripsy Institute, Peking University, Beijing, 100044, People’s Republic of China
- Department of Urology, Michigan Medicine, Ann Arbor, MI, 48109, USA
| | - Yanying Liu
- Department of Rheumatology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, People’s Republic of China
- Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, 100044, People’s Republic of China
| | - Yunshu Xiao
- Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, 100044, People’s Republic of China
| | - Hao Hu
- Department of Urology, Peking University People’s Hospital, Beijing, 100044, People’s Republic of China
- Applied Lithotripsy Institute, Peking University, Beijing, 100044, People’s Republic of China
| | - Tao Xu
- Department of Urology, Peking University People’s Hospital, Beijing, 100044, People’s Republic of China
- Applied Lithotripsy Institute, Peking University, Beijing, 100044, People’s Republic of China
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Cheng S, Wu S, Wang N, Xu W, Wei F, Ao W, Yuan L, Ning X, Mao Y, Zhang X, Mao G. Systemic lupus erythematosus complicated by retroperitoneal fibrosis: A case report and literature review. Medicine (Baltimore) 2025; 104:e41208. [PMID: 40184101 PMCID: PMC11709181 DOI: 10.1097/md.0000000000041208] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/16/2024] [Revised: 12/04/2024] [Accepted: 12/17/2024] [Indexed: 04/05/2025] Open
Abstract
RATIONALE Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems. Retroperitoneal fibrosis (RPF) is a rare condition characterized by the development of fibrous tissue in the retroperitoneal space. The coexistence of SLE and RPF is extremely uncommon, and this report aims to enhance understanding of this complex relationship. PATIENT CONCERNS A 32-year-old woman presented with sudden-onset syncope. Her medical history revealed a 5-year history of SLE, and imaging studies identified a retroperitoneal mass. DIAGNOSES A comprehensive diagnostic workup, including magnetic resonance imaging (MRI) and biopsy, confirmed retroperitoneal fibrosis secondary to SLE. INTERVENTIONS The patient was treated with high-dose corticosteroids, immunosuppressive therapy, and the biologic agent rituximab. OUTCOMES The patient's symptoms markedly improved, and follow-up MRI demonstrated significant regression of the retroperitoneal lesion. LESSONS RPF associated with SLE is exceptionally rare. This case underscores the importance of early diagnosis and a coordinated multidisciplinary approach in managing such complex conditions. Glucocorticoid therapy remains the cornerstone of treatment, augmented by immunosuppressants and biologic agents when necessary.
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Affiliation(s)
- Song Cheng
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Sikai Wu
- Zhejiang Chinese Medical University, Hangzhou, Zhejiang Province, China
| | - Neng Wang
- Zhejiang Chinese Medical University, Hangzhou, Zhejiang Province, China
| | - Wenjie Xu
- Zhejiang Chinese Medical University, Hangzhou, Zhejiang Province, China
| | - Fuquan Wei
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Weiqun Ao
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Li Yuan
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Xiaoxiang Ning
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Yichuan Mao
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Xianzhen Zhang
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Guoqun Mao
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
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Badshah MB, Khan QA, Kazi N, Ansari RA, Verma R. IgG4-related retroperitoneal fibrosis presenting as a peripancreatic mass: a case series. Ann Med Surg (Lond) 2025; 87:36-42. [PMID: 40109592 PMCID: PMC11918540 DOI: 10.1097/ms9.0000000000002749] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2024] [Accepted: 11/05/2024] [Indexed: 03/22/2025] Open
Abstract
Introduction IgG4-related disease (IgG4-RD) is a chronic, immune-mediated disorder characterized by widespread inflammation and fibrosis, leading to potential organ dysfunction if untreated. Often underdiagnosed due to subtle and varied symptoms, the disease can affect multiple organ systems. This case series highlights two patients who were diagnosed as cases of IgG4-related retroperitoneal fibrosis. Methods A total of two patients were included in this prospective case series who presented to the gastroenterology department of a tertiary care hospital with the same signs and symptoms and were diagnosed with IgG4-related retroperitoneal fibrosis (IgG4-RPF). Case summary Two patients were included in our case series, aged 25 and 26 years. The chief complaints included dull, radiating epigastric pain, other symptoms include diffuse abdominal pain, intensified, accompanied by nausea, vomiting, and episodic diarrhea, and a history of B-cell lymphoproliferative disorder. Endoscopic ultrasound (EUS)-)-guided biopsies were performed, showing findings consistent with (IgG4-RPF). Both patients were started on a regimen of prednisolone, pantoprazole, and vitamin D, which they tolerated well without adverse effects. They were advised to follow up with a CT scan after one month. Conclusion IgG4-related disease (IgG4-RD) is a rare, chronic condition often presenting as retroperitoneal fibrosis (RPF) and affecting multiple organs. Serum IgG4 levels can be normal in certain cases, histopathological and radiological investigations are necessary for the correct diagnosis. Early initiation of immunosuppressive drugs are necessary for the disease control.
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Affiliation(s)
| | | | - Naima Kazi
- Khyber Girls Medical College, Peshawar, Pakistan
| | | | - Ravina Verma
- St. George's University School of Medicine, True Blue, Grenada
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Liu H, Gao H, Zhao JX, Wong UK, Liu SB, Liu J, Zhang G, Wang KT, Wang Y, Zhao L, Ma XB, Lu YW, Zhang XW. Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients. Front Med (Lausanne) 2024; 11:1435870. [PMID: 39717179 PMCID: PMC11663637 DOI: 10.3389/fmed.2024.1435870] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2024] [Accepted: 11/15/2024] [Indexed: 12/25/2024] Open
Abstract
Objective Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes have not been well elucidated. Methods A total of 115 RPF-related hydronephrosis patients have been recruited from 9 clinical centers in China since March 2010. They were followed up until death or September 2021, whichever came first. Results The mean age at diagnosis was 58.83 ± 12.13 years, and 80 patients (69.57%) were men. The median disease duration was 3.00 (1.00, 9.00) months. Renal impairment was observed in 88.35% of the patients, and 49.57% showed bilateral ureteral involvement. Elevated ESR and CRP were presented in 80.28 and 62.02% of the patients, respectively. Overall, 28.21% (11/39) of the patients had increased IgG4 levels, and 41.38% (12/29) showed positive pathological IgG4 staining (IgG4+/IgG+ ≥ 40% or IgG4+ ≥ 10/HPF). Among them, three patients were diagnosed as IgG4RD. After 60.43 ± 34.53 months of follow-up, 36 patients had poor prognosis, which was associated with severe kidney impairment, bilateral hydronephrosis and inflammation status (elevated ESR and IgG) at diagnosis by case-control study. eGFR and creatinine were independent risk factors after adjusting for all other significant associations (p = 0.002 and p = 0.067, respectively). Glucocorticoid-based therapy could reduce the time of stenting, decrease the need for long-term ureteral stenting/percutaneous nephrostomy (PNS)/ureterolysis, increase the rate of mass shrinkage, and reduce the new requirement of hemodialysis compared to surgery-only strategy for RPF-related hydronephrosis patients in need of renal drainage, but did not reduce new-onset renal atrophy. Conclusion Severity of kidney dysfunction and inflammation status were related to the poor prognosis of hydronephrosis induced by RPF. More efficient interventions and strategies are needed to further improve outcomes.
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Affiliation(s)
- Hongyan Liu
- Department of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Hui Gao
- Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China
| | - Jin-xia Zhao
- Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China
| | - Ut-kei Wong
- Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China
| | - Shi-bo Liu
- Department of Retroperitoneal Tumor Surgery, Peking University International Hospital, Beijing, China
| | - Jian Liu
- Department of Rheumatology and Immunology, Aerospace Center Hospital, Peking University Aerospace School of Clinical Medicine, Beijing, China
| | - Ge Zhang
- Department of Rheumatology and Immunology, Beijing Haidian Hospital, Beijing Haidian Section of Peking University Third Hospital, Beijing, China
| | - Kuan-ting Wang
- Department of Rheumatology and Immunology, Peking University Shougang Hospital, Beijing, China
| | - Yan Wang
- Department of Rheumatology and Immunology, The Fifth Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Lin Zhao
- Department of Endocrinology, Rheumatology, and Immunology, The Fourth Clinical College of Xinxiang Medical University, Xinxiang, China
| | - Xiang-bo Ma
- Department of Rheumatology and Immunology, Handan First Hospital, Handan, China
| | - Yue-wu Lu
- Department of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Xue-wu Zhang
- Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China
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12
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Lanzillotta M, Culver E, Sharma A, Zen Y, Zhang W, Stone JH, Della-Torre E. Fibrotic phenotype of IgG4-related disease. THE LANCET. RHEUMATOLOGY 2024; 6:e469-e480. [PMID: 38574746 DOI: 10.1016/s2665-9913(23)00299-0] [Citation(s) in RCA: 8] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/08/2023] [Revised: 10/13/2023] [Accepted: 10/31/2023] [Indexed: 04/06/2024]
Abstract
A prompt response to glucocorticoids is a clinical hallmark of IgG4-related disease. However, manifestations characterised by prominent tissue fibrosis on histological examination can be less responsive to glucocorticoid therapy than other types of IgG4-related disease. These manifestations include retroperitoneal fibrosis, fibrosing mediastinitis, Riedel thyroiditis, orbital pseudotumor, and hypertrophic pachymeningitis, among others. To explain this discrepancy, a preliminary distinction into proliferative and fibrotic phenotypes of IgG4-related disease has been proposed on the basis of clinical presentation, pathological features, and response to immunosuppressive therapy. Implications of this classification for patient management remain an important area of investigation. In this Series paper, we aim to dissect the pathophysiology of tissue fibrosis in IgG4-related disease and discuss how clinicians should approach the management of fibrotic manifestations of IgG4-related disease based on the most recent diagnostic and therapeutic developments.
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Affiliation(s)
- Marco Lanzillotta
- Università Vita-Salute San Raffaele, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Emma Culver
- Oxford University Hospitals NHS Foundation Trust, University of Oxford, Oxford, UK
| | - Amita Sharma
- Thoracic Imaging and Intervention Division, Massachusetts General Hospital, Boston, MA, USA
| | - Yoh Zen
- Institute of Liver Studies, King's College Hospital and King's College London, London, UK
| | - Wen Zhang
- Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China
| | - John H Stone
- Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA
| | - Emanuel Della-Torre
- Università Vita-Salute San Raffaele, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy.
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13
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Laminette PJ, Hardie RJ. Subcutaneous ureteral bypass for treatment of proximal ureteral obstruction secondary to retroperitoneal fibrosis after renal transplantation in a cat. JFMS Open Rep 2024; 10:20551169241265244. [PMID: 39184328 PMCID: PMC11342331 DOI: 10.1177/20551169241265244] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/27/2024] Open
Abstract
Case summary A 5.5-year-old male neutered domestic shorthair cat was presented with a 2-year history of progressive chronic kidney disease. Abdominal ultrasonography revealed bilateral chronic renal degeneration, nephrolithiasis, cortical hyperechogenicity and infarction. Left orthotopic renal transplantation was performed using the Synovis vascular coupling system for end-to-end anastomosis of the renal arteries and veins. Two months after transplantation, renal values were elevated, and abdominal ultrasonography revealed hydronephrosis and hydroureter of the transplanted kidney. Fluoroscopic antegrade pyelography identified a proximal ureteral stricture. Proximal neoureterocystostomy was performed and renal values normalized postoperatively. The cat was re-evaluated for acute stranguria and severe azotemia 12 months later. Contrast-enhanced CT revealed severe hydronephrosis of the transplanted kidney, obstruction of the proximal ureter and adhesions to the urinary bladder. Upon exploration, retroperitoneal fibrosis was found covering the transplanted kidney. Given the clinical situation, a subcutaneous ureteral bypass device (SUB) was placed. Clinicopathologic analyses, trough cyclosporine levels, aerobic urine cultures and ultrasonographic evaluations of the transplanted kidney were monitored every 1-3 months. Patency of the SUB was reassessed every 3-6 months. At 15 months after placement, the SUB occluded due to kinking of the cystostomy catheter and was replaced. At 28 months after SUB placement, renal function and clinical status deteriorated, and the cat was euthanized. Relevance and novel information To the authors' knowledge, this is the first report of a SUB device being used for management of ureteral obstruction in a transplanted kidney in a cat.
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Affiliation(s)
- Peter J Laminette
- University of Wisconsin-Madison, School of Veterinary Medicine, Department of Surgical Sciences, Madison, WI, USA
| | - Robert J Hardie
- University of Wisconsin-Madison, School of Veterinary Medicine, Department of Surgical Sciences, Madison, WI, USA
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14
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Khalil Z, Hanna RA, Maher S. A Case of Idiopathic Retroperitoneal Fibrosis Triggering Recurrent Gastrointestinal Bleeding. Cureus 2024; 16:e65039. [PMID: 39165438 PMCID: PMC11335034 DOI: 10.7759/cureus.65039] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/21/2024] [Indexed: 08/22/2024] Open
Abstract
Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by fibrous tissue proliferation in the retroperitoneal space, commonly affecting the ureters and other abdominal structures. This case report describes a previously undocumented presentation of IRF in a 52-year-old female, who presented with recurrent gastrointestinal bleeding and severe anemia over six months. Diagnostic workup included endoscopy, colonoscopy, abdominal computed tomography (CT), and biopsy, revealing fibrous encasement of the mesenteric vessels leading to ischemic damage and gastrointestinal bleeding. Treatment involved high-dose corticosteroids and surgical resection of the fibrotic tissue, which resulted in complete resolution of symptoms. The aim of this case report is to highlight this unique presentation of IRF, discuss the diagnostic challenges, and explore effective treatment strategies for managing this rare but significant complication.
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Affiliation(s)
- Zeyad Khalil
- College of Medicine, October 6 University, Cairo, EGY
| | | | - Soheir Maher
- Emergency Medicine, Al-Fares Crystal Medical Complex, Makkah, SAU
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15
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Chahrour MA, Sharafuddin MJ. Infective native arterial aneurysms and inflammatory abdominal aortic aneurysms: An overview with a focus on emergency settings. Semin Vasc Surg 2024; 37:258-276. [PMID: 39152004 DOI: 10.1053/j.semvascsurg.2023.04.019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2023] [Revised: 04/25/2023] [Accepted: 04/27/2023] [Indexed: 08/19/2024]
Abstract
Infective native arterial aneurysms and inflammatory aortic aneurysms are rare but morbid pathologies seen by vascular surgeons in the emergency setting. Presentation is not always clear, and a full workup must be obtained before adopting a management strategy. Treatment is multidisciplinary and is tailored to every case based on workup findings. Imaging with computed tomography, magnetic resonance, or with fluorodeoxyglucose-positron emission tomography aids in diagnosis and in monitoring response to treatment. Open surgery is traditionally performed for definitive management. Endovascular surgery may offer an alternative treatment in select cases with acceptable outcomes. Neither technique has been proven to be superior to the other. Physicians should consider patient's anatomy, comorbidities, life expectancy, and goals of care before selecting an approach. Long-term pharmacological treatment, with antibiotics in case of infective aneurysms and immunosuppressants in case of inflammatory aneurysms, is usually required and should be managed in collaboration with infectious disease specialists and rheumatologists.
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Affiliation(s)
- Mohamad A Chahrour
- Division of Vascular Surgery, University of Iowa Carver College of Medicine, Iowa City, IA
| | - Mel J Sharafuddin
- Memorial Hospital Central, University of Colorado Healthcare, 1400 E Boulder St, Colorado Springs, CO 80909.
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16
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Kunow A, Freyer Martins Pereira J, Chenot JF. Extravertebral low back pain: a scoping review. BMC Musculoskelet Disord 2024; 25:363. [PMID: 38714994 PMCID: PMC11075250 DOI: 10.1186/s12891-024-07435-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2023] [Accepted: 04/11/2024] [Indexed: 05/12/2024] Open
Abstract
BACKGROUND Low back pain (LBP) is one of the most common reasons for consultation in general practice. Currently, LBP is categorised into specific and non-specific causes. However, extravertebral causes, such as abdominal aortic aneurysm or pancreatitis, are not being considered. METHODS A systematic literature search was performed across MEDLINE, Embase, and the Cochrane library, complemented by a handsearch. Studies conducted between 1 January 2001 and 31 December 2020, where LBP was the main symptom, were included. RESULTS The literature search identified 6040 studies, from which duplicates were removed, leaving 4105 studies for title and abstract screening. Subsequently, 265 publications were selected for inclusion, with an additional 197 publications identified through the handsearch. The majority of the studies were case reports and case series, predominantly originating from specialised care settings. A clear distinction between vertebral or rare causes of LBP was not always possible. A range of diseases were identified as potential extravertebral causes of LBP, encompassing gynaecological, urological, vascular, systemic, and gastrointestinal diseases. Notably, guidelines exhibited inconsistencies in addressing extravertebral causes. DISCUSSION Prior to this review, there has been no systematic investigation into extravertebral causes of LBP. Although these causes are rare, the absence of robust and reliable epidemiological data hinders a comprehensive understanding, as well as the lack of standardised protocols, which contributes to a lack of accurate description of indicative symptoms. While there are certain disease-specific characteristics, such as non-mechanical or cyclical LBP, and atypical accompanying symptoms like fever, abdominal pain, or leg swelling, that may suggest extravertebral causes, it is important to recognise that these features are not universally present in every patient. CONCLUSION The differential diagnosis of extravertebral LBP is extensive with relatively low prevalence rates dependent on the clinical setting. Clinicians should maintain a high index of suspicion for extravertebral aetiologies, especially in patients presenting with atypical accompanying symptoms.
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Affiliation(s)
- Anna Kunow
- Department of General Practice, University Medicine Greifswald, 17475, Fleischmannstraße, Greifswald, Germany.
| | | | - Jean-François Chenot
- Department of General Practice, University Medicine Greifswald, 17475, Fleischmannstraße, Greifswald, Germany
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17
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Besson FL, Nocturne G, Noël N, Gheysens O, Slart RHJA, Glaudemans AWJM. PET/CT in Inflammatory and Auto-immune Disorders: Focus on Several Key Molecular Concepts, FDG, and Radiolabeled Probe Perspectives. Semin Nucl Med 2024; 54:379-393. [PMID: 37973447 DOI: 10.1053/j.semnuclmed.2023.10.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2023] [Revised: 10/19/2023] [Accepted: 10/23/2023] [Indexed: 11/19/2023]
Abstract
Chronic immune diseases mainly include autoimmune and inflammatory diseases. Managing chronic inflammatory and autoimmune diseases has become a significant public health concern, and therapeutic advancements over the past 50 years have been substantial. As therapeutic tools continue to multiply, the challenge now lies in providing each patient with personalized care tailored to the specifics of their condition, ushering in the era of personalized medicine. Precise and holistic imaging is essential in this context to comprehensively map the inflammatory processes in each patient, identify prognostic factors, and monitor treatment responses and complications. Imaging of patients with inflammatory and autoimmune diseases must provide a comprehensive view of the body, enabling the whole-body mapping of systemic involvement. It should identify key cellular players in the pathology, involving both innate immunity (dendritic cells, macrophages), adaptive immunity (lymphocytes), and microenvironmental cells (stromal cells, tissue cells). As a highly sensitive imaging tool with vectorized molecular probe capabilities, PET/CT can be of high relevance in the management of numerous inflammatory and autoimmune diseases. Relying on key molecular concepts of immunity, the clinical usefulness of FDG-PET/CT in several relevant inflammatory and immune-inflammatory conditions, validated or emerging, will be discussed in this review, together with radiolabeled probe perspectives.
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Affiliation(s)
- Florent L Besson
- Department of Nuclear Medicine-Molecular Imaging, Hôpitaux Universitaires Paris-Saclay, AP-HP, DMU SMART IMAGING, CHU Bicêtre, Paris, France; Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France; Université Paris-Saclay, Commissariat à l'énergie Atomique et aux Énergies Alternatives (CEA), Centre National de la Recherche Scientifique (CNRS), INSERM, BioMaps, Le Kremlin-Bicêtre, France.
| | - Gaetane Nocturne
- Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France; Department of Rheumatology, Hôpital Bicêtre Assistance Publique -Hôpitaux de Paris, Le Kremlin-Bicêtre, France; Center for Immunology of Viral Infections and Auto-Immune Diseases (IMVA), Université Paris-Saclay, Institut pour la Santé et la Recherche Médicale (INSERM) UMR 1184, Le Kremlin Bicêtre, Paris, France
| | - Nicolas Noël
- Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France; Center for Immunology of Viral Infections and Auto-Immune Diseases (IMVA), Université Paris-Saclay, Institut pour la Santé et la Recherche Médicale (INSERM) UMR 1184, Le Kremlin Bicêtre, Paris, France; Department of Internal Medicine, Hôpital Bicêtre Assistance Publique -Hôpitaux de Paris, Le Kremlin-Bicêtre, Paris, France
| | - Olivier Gheysens
- Department of Nuclear Medicine, Cliniques Universitaires St-Luc and Institute for Experimental and Clinical Research (IREC), Université Catholique de Louvain, Brussels, Belgium
| | - Riemer H J A Slart
- Department of Nuclear Medicine and Molecular Imaging, University Medical Centre Groningen, Groningen, The Netherlands; Biomedical Photonic Imaging Group, Faculty of Science and Technology, University of Twente, Enschede, The Netherlands
| | - Andor W J M Glaudemans
- Department of Nuclear Medicine and Molecular Imaging, University Medical Centre Groningen, Groningen, The Netherlands
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18
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Gagliardo CM, Giammanco A, Vaglio A, Pegoraro F, Cefalù AB, Averna M, Noto D. Erdheim-Chester disease as complex clinical presentation and diagnosis: A case report and concise review of literature. Medicine (Baltimore) 2024; 103:e37870. [PMID: 38669404 PMCID: PMC11049690 DOI: 10.1097/md.0000000000037870] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/11/2023] [Accepted: 03/21/2024] [Indexed: 04/28/2024] Open
Abstract
RATIONALE Erdheim-Chester disease (ECD) is a rare multisystemic disease characterized by the infiltration of multiple organs by foamy CD68 + CD1a-histiocytes. The genetic background consists of gain-of-function somatic mutations in the mitogen-activated protein kinase pathway. The purpose of the present paper is to make a contribution to the scientific literature on ECD by reporting our experience with a complex clinical case report, along with a concise review of the literature. We discussed the unusual clinical presentation, the complex diagnostic process and the comparison with other published cases. PATIENT CONCERNS A 70-year-old man presented with arthralgia due to multiple bone areas of sclerosis, first diagnosed with metastases of a prostatic neoplasm. Sequential thorax-abdomen, femoral and homer contrast-enhanced computed tomography (CT) showed pericardial effusion, pulmonary fibrosis, and perirenal fibrous tissue as "hairy kidneys." He underwent. Three bone biopsies were unsuccessful to reach diagnosis. DIAGNOSES A xanthelasma biopsy showed histopathological signs compatible with ECD; genetic analysis showed the mutation BRAFV600E. INTERVENTIONS The patient underwent targeted therapy with vemurafenib (BRAF-inhibitor), discontinued 2 weeks later due to the onset of a diffuse erythematous papular rash on the trunk and limbs. OUTCOMES At the 1-year follow-up, there was only progression of chronic kidney disease (CKD). LESSONS The present case report describes how ECD diagnosis could represent a challenge for clinicians, owing to its heterogeneous clinical presentation. Early diagnosis followed by prompt therapy is essential for modifying the natural history of the disease.
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Affiliation(s)
- Carola M. Gagliardo
- Department of Health Promotion, Maternal and Child Health, Internal and Specialized Medicine of Excellence “G. D. Alessandro” (PROMISE), University of Palermo, Palermo, Italy
| | - Antonina Giammanco
- Department of Health Promotion, Maternal and Child Health, Internal and Specialized Medicine of Excellence “G. D. Alessandro” (PROMISE), University of Palermo, Palermo, Italy
| | - Augusto Vaglio
- Nephrology and Dialysis Unit, Meyer Children’s Hospital IRCCS, Florence, Italy
- Department of Biomedical, Experimental and Clinical Sciences “Mario Serio,” University of Florence, Florence, Italy
| | - Francesco Pegoraro
- Department of Health Science, University of Florence, Florence, Italy
- Pediatric Hematology and Oncology Unit, Meyer Children’s University Hospital IRCCS, Florence, Italy
| | - Angelo B. Cefalù
- Department of Health Promotion, Maternal and Child Health, Internal and Specialized Medicine of Excellence “G. D. Alessandro” (PROMISE), University of Palermo, Palermo, Italy
| | - Maurizio Averna
- Department of Health Promotion, Maternal and Child Health, Internal and Specialized Medicine of Excellence “G. D. Alessandro” (PROMISE), University of Palermo, Palermo, Italy
| | - Davide Noto
- Department of Health Promotion, Maternal and Child Health, Internal and Specialized Medicine of Excellence “G. D. Alessandro” (PROMISE), University of Palermo, Palermo, Italy
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19
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Achrak E, Manville E, Ayyat M, Toribio RD. Atypical Presentation of Retroperitoneal Fibrosis Causing Colonic Obstruction: A Case Report. Cureus 2024; 16:e55621. [PMID: 38586788 PMCID: PMC10995894 DOI: 10.7759/cureus.55621] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/05/2024] [Indexed: 04/09/2024] Open
Abstract
Retroperitoneal fibrosis (RPF), also referred to as Ormond's disease, is a rare fibroinflammatory condition characterized by abnormal fibrous tissue deposition in the retroperitoneal space, which traditionally presents with ureteral obstruction. Nonetheless, our case report showcases an exceptional instance involving a 70-year-old female patient who presented with symptoms suggestive of colonic obstruction, an unusual presentation that is not commonly associated with RPF. Although RPF has established associations with autoimmune conditions such as immunoglobulin G4-related disease and systemic lupus erythematosus, its connection to colonic obstruction remains undocumented in the medical literature. Our patient is a 70-year-old female who presented with constipation, anemia, and fecal occult blood. Her past medical history included a hysterectomy due to fibroids, right breast lumpectomy, type 2 diabetes mellitus, subclinical hyperthyroidism, hypertension, and obesity. Upon physical examination, the patient's abdomen appeared protuberant but was non-tender to palpation. Bowel sounds were normal, and there was no distension. Notably, there was no tenderness in the right or left costovertebral angles, nor was there any guarding. Workup with colonoscopy could not be completed due to the inability to pass a colonoscope beyond the rectosigmoid junction. Further workup with barium enema confirmed an apple core lesion seen in the rectosigmoid concerning for a neoplastic or inflammatory process. Finally, a computed tomography scan of the abdomen and pelvis showed a 7.1 cm right pelvic mass attached to the bladder and cecum, moderate right hydroureteronephrosis, and a 5.2 cm left adnexal mass with soft tissue changes narrowing the sigmoid colon. The next step was to take the patient for an exploratory laparotomy. During exploratory laparotomy, extensive adhesions and desmoplastic reactions were observed in the pelvic region, involving the sigmoid colon, bladder, cecum, and appendix. Two firm masses were identified in the retroperitoneum, one located in the left lower quadrant (LLQ) adherent to the posterior wall of the sigmoid colon and one in the right lower quadrant (RLQ) adherent to the posterior wall of the cecum. Three specimens were sent to pathology for further examination: a portion of the sigmoid colon, a resection from the RLQ mass, and a resection from the LLQ mass. Pathology reported dense fibrotic masses with abscess-like formation, reactive in nature and of unclear etiology, and negative for malignancy. They were negative for fibromatosis (β-catenin negative), and IgG4+/IgG+ was approximately 5%. Interestingly, the LLQ mass also contained remnants of the fallopian tube and ovary and benign cystic changes. This case report presents a unique and atypical presentation of RPF, deviating from the conventional presentation of ureteral obstruction. The patient's initial symptoms suggested colonic obstruction, a clinical scenario rarely linked to RPF. This case underscores the significance of considering diverse clinical presentations when diagnosing RPF, thereby expanding our comprehension of the condition's clinical spectrum and ultimately refining patient care and management.
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Affiliation(s)
- Eleonora Achrak
- Department of Surgery, Touro College of Osteopathic Medicine, New York, USA
| | - Emily Manville
- Department of Surgery, Touro College of Osteopathic Medicine, New York, USA
| | - Mumen Ayyat
- Surgery, Brookdale University Hospital and Medical Center, Brooklyn, USA
| | - Ruben D Toribio
- Surgery, Brookdale University Hospital and Medical Center, Brooklyn, USA
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20
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Gagliardi AP, Rotunno S, Romanello D. Retroperitoneal Fibrosis: A Puzzle of Elusive Causal Link. Cureus 2024; 16:e56220. [PMID: 38618340 PMCID: PMC11016232 DOI: 10.7759/cureus.56220] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/15/2024] [Indexed: 04/16/2024] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of fibro-inflammatory tissue surrounding the abdominal aorta and iliac arteries, often leading to the involvement of adjacent organs such as the ureters and inferior vena cava. We present a case report of a 56-year-old Caucasian woman with a complex medical history, including Hodgkin's lymphoma treated with chemotherapy and radiotherapy (31 years before), a significant smoking history, and a current presentation of acute kidney injury with oliguria, edema, and hypertension. Initial diagnostic considerations included rapidly progressive glomerulonephritis, supported by clinical and imaging findings. However, the absence of specific autoantibodies and the presence of bilateral calyx-pelvic dilation raised questions regarding alternative diagnoses. Imaging studies, including CT, contrast-enhanced CT, and subsequent MRI, revealed periaortic and paracaval adipose tissue thickening suggestive of periaortitis, leading to a diagnosis of retroperitoneal fibrosis. The multifactorial etiology, including previous radiation therapy, lymphoma history, and significant smoking, posed challenges in establishing a definitive causal link. Despite extensive investigations, including laboratory tests and imaging modalities, no single etiological factor could be conclusively identified. This case underscores the diagnostic complexity of RPF, especially in the presence of multiple potential risk factors, and highlights the importance of considering this condition in the differential diagnosis of patients presenting with renal dysfunction and obstructive uropathy.
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Affiliation(s)
| | - Sara Rotunno
- Internal Medicine, Ospedale San Pietro Fatebenefratelli, Rome, ITA
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21
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Gilani SI, Buglioni A, Cornell LD. IgG4-related kidney disease: Clinicopathologic features, differential diagnosis, and mimics. Semin Diagn Pathol 2024; 41:88-94. [PMID: 38246802 DOI: 10.1053/j.semdp.2023.12.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/14/2023] [Accepted: 12/11/2023] [Indexed: 01/23/2024]
Abstract
IgG4-related kidney disease (IgG4-RKD) encompasses all forms of kidney disease that are part of IgG4-related disease (IgG4-RD). First recognized as IgG4-related tubulointerstitial nephritis (IgG4-TIN), and then IgG4-related membranous glomerulonephritis (IgG4-MGN), we now recognize additional patterns of interstitial nephritis, glomerular disease, and vascular disease that can be seen as part of IgG4-RKD. The clinical presentation is variable and can include acute or chronic kidney injury, proteinuria or nephrotic syndrome, mass lesion(s), and obstruction. While usually associated with other organ involvement by IgG4-RD, kidney-alone involvement is present in approximately 20 % of IgG4-RKD. Compared to IgG4-RD overall, patients with IgG4-RKD are more likely to show increased serum IgG4 or IgG, and more likely to have hypocomplementemia. In this review, we extensively cover other types of autoimmune and plasma cell-rich interstitial nephritis, mass forming inflammatory diseases of the kidney, and other mimics of IgG4-TIN, in particular ANCA-associated disease.
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Affiliation(s)
- Sarwat I Gilani
- Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at San Antonio, TX, USA
| | - Alessia Buglioni
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
| | - Lynn D Cornell
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
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22
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Ren L, Fei X, Zhao Y, Xie F, Xue S, Li N, Nie F, Luo Y. Diagnostic value of contrast-enhanced ultrasound in the activity of idiopathic retroperitoneal fibrosis: a retrospective study. Clin Rheumatol 2024; 43:1207-1215. [PMID: 38329616 DOI: 10.1007/s10067-023-06855-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2023] [Revised: 11/01/2023] [Accepted: 11/24/2023] [Indexed: 02/09/2024]
Abstract
OBJECTIVES To explore the diagnostic value of contrast-enhanced ultrasound (CEUS) in the disease activity of idiopathic retroperitoneal fibrosis (IRPF). METHODS This retrospective study included 148 CEUS examinations from 63 patients with IRPF treated in our hospital from April 2016 to September 2021. They were divided into two groups: IRPF active group (69 examinations) and inactive group (79 examinations). Uni- and multivariable analyses were used to identify independent risk factors for IRPF activity. Receiver operating characteristic (ROC) curves were drawn to establish different diagnostic models to evaluate the diagnostic value of IRPF activity. The z test was used to compare the differences of the area under the curves (AUCs). The value of CEUS in evaluating the variation of disease activity over time was also investigated between repeat patient studies. RESULTS Univariate and multivariate logistic regression analyses revealed the thickness [odds ratio (OR) = 14.125, 95% confidence interval (CI) = 3.017-66.123] was the most significant independent risk factor for IRPF activity (P < 0.01). The best diagnostic model was model 3, which was established by CEUS score combined with thickness. The AUC was 0.944 (95%CI = 0.912-0.977), and the sensitivity and specificity were 89.86% and 86.08%, respectively. The diagnostic performance was not significantly improved after combining clinical symptom (back pain) and laboratory indicators [erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)]. Compared with before treatment, the CEUS score and thickness were significantly decreased after treatment (x2 = 14.580, P < 0.001 and z = 4.708, P < 0.001, respectively). CONCLUSION CEUS has good diagnostic value for IRPF disease activity. Key points • Thickness and contrast-enhanced ultrasound score were significantly higher in the active group than those in inactive group (P < 0.001). • With thickness of 4 mm and contrast-enhanced ultrasound 2 score as optimal cut-off values, the sensitivity and specificity were 89.86%, 81.01% and 52.17%, 100.00%, respectively. • During follow-up, when the disease progressed, the change of CEUS score was earlier than the change of thickness.
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Affiliation(s)
- Ling Ren
- Department of Ultrasound, First Medical Center, Chinese PLA General Hospital, Haidian District, No. 28 Fuxing Road, Beijing, 100853, China
- The Second Medical College of Lanzhou University, Chengguan District, No.222 Tianshui South Road, Lanzhou, 730030, Gansu, China
| | - Xiang Fei
- Department of Ultrasound, First Medical Center, Chinese PLA General Hospital, Haidian District, No. 28 Fuxing Road, Beijing, 100853, China
| | - Yurong Zhao
- Department of Rheumatology, First Medical Center, Chinese PLA General Hospital, Haidian District, No. 28 Fuxing Road, Beijing, 100853, China
| | - Fang Xie
- Department of Ultrasound, First Medical Center, Chinese PLA General Hospital, Haidian District, No. 28 Fuxing Road, Beijing, 100853, China
| | - Shaowei Xue
- Department of Ultrasound, First Medical Center, Chinese PLA General Hospital, Haidian District, No. 28 Fuxing Road, Beijing, 100853, China
| | - Nan Li
- Department of Ultrasound, First Medical Center, Chinese PLA General Hospital, Haidian District, No. 28 Fuxing Road, Beijing, 100853, China
| | - Fang Nie
- The Second Medical College of Lanzhou University, Chengguan District, No.222 Tianshui South Road, Lanzhou, 730030, Gansu, China.
| | - Yukun Luo
- Department of Ultrasound, First Medical Center, Chinese PLA General Hospital, Haidian District, No. 28 Fuxing Road, Beijing, 100853, China.
- The Second Medical College of Lanzhou University, Chengguan District, No.222 Tianshui South Road, Lanzhou, 730030, Gansu, China.
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23
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Nishimura M, Kimizuka Y, Ogawa T, Tsuchiya M, Kato Y, Matsukida A, Igarashi S, Ito K, Serizawa Y, Tanigaki T, Fujikura Y, Katsurada Y, Ogata S, Kawana A. IgG4-related retroperitoneal fibrosis induced by nivolumab and ipilimumab in a patient with non-small cell lung cancer: A case report. Thorac Cancer 2024; 15:104-107. [PMID: 38098256 PMCID: PMC10761607 DOI: 10.1111/1759-7714.15155] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2023] [Revised: 10/24/2023] [Accepted: 10/25/2023] [Indexed: 01/04/2024] Open
Abstract
IgG4-related diseases are adverse events that occur after receiving treatment with immune checkpoint inhibitors (ICI). This study reports the first case of IgG4-related retroperitoneal fibrosis after the administration of chemotherapy with nivolumab and ipilimumab (NI therapy). An 80-year-old man developed lower abdominal pain eight months after NI therapy was initiated. Although the primary lesion maintained its reduced size on computed tomography, there was an increase in the soft tissue shadows intensity around the abdominal aorta, bladder, and seminal vesicles, suggesting retroperitoneal fibrosis. Blood tests showed elevated IgG4 levels. Computed tomography-guided biopsy of the retroperitoneum showed B cell-dominant lymphocyte infiltration consistent with IgG4-related retroperitoneal fibrosis and characteristic CD8-positive lymphocyte infiltration, suggestive of the involvement of cytotoxic T cells. Based on the clinical, imaging, and pathological findings, the patient was diagnosed with IgG4-related retroperitoneal fibrosis due to ICI. Immunotherapy discontinuation alone did not result in improvement; therefore, steroid therapy was initiated. In clinical practice, IgG4-related retroperitoneal fibrosis can occur as an immune-related adverse event when administering anti-PD-1 and anti-CTLA-4 antibodies for cancer immunotherapy. Early steroid therapy could be effective in controlling this immune-related adverse event.
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Affiliation(s)
- Masashi Nishimura
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Yoshifumi Kimizuka
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Takunori Ogawa
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Motohiro Tsuchiya
- Department of Pathology and Laboratory MedicineNational Defense Medical CollegeSaitamaJapan
| | - Yoshiki Kato
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Akira Matsukida
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Shunya Igarashi
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Koki Ito
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Yusuke Serizawa
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Tomomi Tanigaki
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Yuji Fujikura
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
| | - Yuka Katsurada
- Department of Pathology and Laboratory MedicineNational Defense Medical CollegeSaitamaJapan
| | - Sho Ogata
- Department of Pathology and Laboratory MedicineNational Defense Medical CollegeSaitamaJapan
| | - Akihiko Kawana
- Division of Infectious Diseases and Respiratory Medicine, Department of Internal MedicineNational Defense Medical CollegeSaitamaJapan
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24
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Harne PS, Soni U, Albustamy A, Rivera AS, Zamir A. Liposarcoma Masquerading as Immunoglobulin G4-Related Disease. ACG Case Rep J 2024; 11:e01249. [PMID: 38179263 PMCID: PMC10766308 DOI: 10.14309/crj.0000000000001249] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2023] [Accepted: 11/27/2023] [Indexed: 01/06/2024] Open
Abstract
This case report highlights the challenge of differentiating between immunoglobulin G4 (IgG4)-related disease (IGRD) and liposarcoma, which have similar symptoms and serological markers. A 40-year-old woman presented with right upper-quadrant pain and a retroperitoneal mass. Initial biopsy indicated increased IgG4-positive cells, leading to an IGRD diagnosis. However, the mass grew, despite prednisone treatment, prompting a repeat biopsy that revealed well-differentiated liposarcoma. Liposarcoma should be considered in patients with retroperitoneal masses and elevated IgG4 levels. Surgical resection is the primary treatment, emphasizing the need for early identification and close monitoring. Confirming IGRD or ruling out other diagnoses through histopathologic analysis is vital.
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Affiliation(s)
| | - Ujjwal Soni
- University College of Medical Sciences, New Delhi, India
| | | | | | - Asif Zamir
- Doctors Hospital at Renaissance, Edinburg, TX
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25
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Luo H, Lou KC, Xie LY, Zeng F, Zou JR. Pharmacotherapy of urethral stricture. Asian J Androl 2024; 26:1-9. [PMID: 37738151 PMCID: PMC10846832 DOI: 10.4103/aja202341] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2023] [Accepted: 07/21/2023] [Indexed: 09/24/2023] Open
Abstract
Urethral stricture is characterized by the chronic formation of fibrous tissue, leading to the narrowing of the urethral lumen. Despite the availability of various endoscopic treatments, the recurrence of urethral strictures remains a common challenge. Postsurgery pharmacotherapy targeting tissue fibrosis is a promising option for reducing recurrence rates. Although drugs cannot replace surgery, they can be used as adjuvant therapies to improve outcomes. In this regard, many drugs have been proposed based on the mechanisms underlying the pathophysiology of urethral stricture. Ongoing studies have obtained substantial progress in treating urethral strictures, highlighting the potential for improved drug effectiveness through appropriate clinical delivery methods. Therefore, this review summarizes the latest researches on the mechanisms related to the pathophysiology of urethral stricture and the drugs to provide a theoretical basis and new insights for the effective use and future advancements in drug therapy for urethral stricture.
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Affiliation(s)
- Hui Luo
- The First Clinical College, Gannan Medical University, Ganzhou 341000, China
- Department of Urology, The First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, China
| | - Ke-Cheng Lou
- The First Clinical College, Gannan Medical University, Ganzhou 341000, China
- Department of Urology, The First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, China
| | - Ling-Yu Xie
- The First Clinical College, Gannan Medical University, Ganzhou 341000, China
- Department of Urology, The First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, China
| | - Fei Zeng
- The First Clinical College, Gannan Medical University, Ganzhou 341000, China
| | - Jun-Rong Zou
- Department of Urology, The First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, China
- Institute of Urology, The First Affiliated Hospital of Gannan Medical University, Ganzhou 341000, China
- Jiangxi Engineering Technology Research Center of Calculi Prevention, Ganzhou 341000, China
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26
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Benites-Yshpilco L, Barriales-Revilla L, Baltodano-Arellano R, Falcón-Quispe L, Cupe-Chacalcaje K, Cachicatari-Beltrán Á, Lévano-Pachas G. Multimodal imaging in Ig G4- related aortitis: case report. ARCHIVOS PERUANOS DE CARDIOLOGIA Y CIRUGIA CARDIOVASCULAR 2024; 5:50-53. [PMID: 38596609 PMCID: PMC10999319 DOI: 10.47487/apcyccv.v5i1.317] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 09/24/2023] [Accepted: 11/26/2023] [Indexed: 04/11/2024]
Abstract
We present the case of a 56-year-old patient with fever of unknown origin associated with chest and lumbar pain. Multimodality imaging revealed diffuse peri-aortitis in the thoracic aorta without involvement of the aortic valve, contributing substantially to the diagnosis of Ig G4-associated aortitis. Immunosuppressive therapy was started. Follow-up at five months with cardiac magnetic resonance imaging showed a reduction in the inflammatory process in the thoracic aorta.
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Affiliation(s)
- Lindsay Benites-Yshpilco
- Hospital Guillermo Almenara Irigoyen - EsSalud, Lima, Perú.Hospital Guillermo Almenara Irigoyen - EsSaludLimaPerú
| | - Lucia Barriales-Revilla
- Hospital Guillermo Almenara Irigoyen - EsSalud, Lima, Perú.Hospital Guillermo Almenara Irigoyen - EsSaludLimaPerú
| | - Roberto Baltodano-Arellano
- Facultad de Medicina, Universidad Nacional Mayor de San Marcos, Lima, PerúUniversidad Nacional Mayor de San MarcosFacultad de MedicinaUniversidad Nacional Mayor de San MarcosLimaPeru
- Cardiac imaging area of Cardiology Service, Hospital Guillermo Almenara Irigoyen - EsSalud, Lima, Perú.Cardiac imaging area of Cardiology ServiceHospital Guillermo Almenara Irigoyen - EsSaludLimaPerú
| | - Luis Falcón-Quispe
- Cardiac imaging area of Cardiology Service, Hospital Guillermo Almenara Irigoyen - EsSalud, Lima, Perú.Cardiac imaging area of Cardiology ServiceHospital Guillermo Almenara Irigoyen - EsSaludLimaPerú
| | - Kelly Cupe-Chacalcaje
- Cardiac imaging area of Cardiology Service, Hospital Guillermo Almenara Irigoyen - EsSalud, Lima, Perú.Cardiac imaging area of Cardiology ServiceHospital Guillermo Almenara Irigoyen - EsSaludLimaPerú
| | - Ángela Cachicatari-Beltrán
- Cardiac imaging area of Cardiology Service, Hospital Guillermo Almenara Irigoyen - EsSalud, Lima, Perú.Cardiac imaging area of Cardiology ServiceHospital Guillermo Almenara Irigoyen - EsSaludLimaPerú
| | - Gerald Lévano-Pachas
- Hospital Guillermo Almenara Irigoyen - EsSalud, Lima, Perú.Hospital Guillermo Almenara Irigoyen - EsSaludLimaPerú
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27
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Wiedbrauck D, Flemming P, Hollerbach S. Role of transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of retroperitoneal fibrosis (Ormond's disease). ZEITSCHRIFT FUR GASTROENTEROLOGIE 2023; 61:1623-1627. [PMID: 36972595 DOI: 10.1055/a-2003-9752] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 03/29/2023]
Abstract
BACKGROUND Retroperitoneal fibrosis (RPF), often referred to as Ormond's disease when it is of idiopathic origin, is a rare disease characterized by the presence of inflammatory infiltrates and periaortic masses in the retroperitoneum. For a definite diagnosis, a biopsy and subsequent pathological examination is required. Currently accepted methods for retroperitoneal biopsy include open, laparoscopic, or CT-guided approaches. However, transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of RPF has attracted only little attention in the literature. CASE REPORTS We report two male patient cases who presented with leukocytosis, elevated C-reactive protein, and a suspicious retroperitoneal mass of unknown origin on computed tomography. One patient also reported left lower quadrant pain, whereas the other patient suffered from back pain and weight loss. In both patients, idiopathic RPF was successfully diagnosed by using transduodenal EUS-FNA/FNB with 22- and 20-gauge aspiration needles. Histopathology revealed dense lymphocytic infiltrates and fibrosis. The procedures lasted approximately 25 and 20 minutes, respectively, and in both patients no serious adverse events occurred. Treatment included steroid therapy and administration of Azathioprine. CONCLUSION We demonstrate that using EUS-FNA/FNB to diagnose RPF is a feasible, fast, and safe method, which should always be considered as a first-line diagnostic modality. Hence, this case report emphasizes that gastrointestinal endoscopists are likely to play an important role in the setting of suspected RPF.
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Affiliation(s)
- Damian Wiedbrauck
- Department of Gastroenterology, Allgemeines Krankenhaus Celle, Celle, Germany
| | | | - Stephan Hollerbach
- Department of Gastroenterology, Allgemeines Krankenhaus Celle, Celle, Germany
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28
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Petelytska L, Nikitina A, Tarasenko O, Chechotenko I, Mykhailov D, Kravchenko V, Iaremenko O. IgG4-related retroperitoneal fibrosis with acute kidney injury: a case report and literature review. Rheumatol Int 2023; 43:2141-2153. [PMID: 37542604 DOI: 10.1007/s00296-023-05402-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2023] [Accepted: 07/19/2023] [Indexed: 08/07/2023]
Abstract
IgG4-related retroperitoneal fibrosis is a rare cause of renal dysfunction that usually manifests as obstructive nephropathy (sometimes with extrarenal manifestations). Due to the non-specific symptoms at the onset of the disease and often latent course, special laboratory and instrumental examination methods are usually needed to establish a diagnosis. The article describes a clinical case of a relapse of IgG4-related retroperitoneal fibrosis in a 53-year-old patient, who developed bilateral ureterohydronephrosis with postrenal acute kidney injury after the withdrawal of glucocorticoid therapy. The patient underwent bilateral percutaneous nephrostomy and resumed glucocorticoids at a dose of 30 mg/day. Postobstructive diuresis was 22 L. Treatment resulted in a complete normalization of the creatinine level and transient hypokalemia, which was eliminated with potassium medications. At the final stage of the treatment, bilateral stenting of both ureters was performed with a tapering of glucocorticoids to 5 mg per day with CT control of the retroperitoneal space after 5 months. A clinical case demonstrates that an interruption of glucocorticoid treatment in patients with IgG4-related retroperitoneal fibrosis can lead to ureterohydronephrosis with the development of acute kidney injury. In such cases, stenting of the ureters could be considered an optimal therapeutic option.
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Affiliation(s)
- Liubov Petelytska
- Department of Internal Medicine #3, Bogomolets National Medical University, T. Shevchenko Boulevard, 13, Kiev, 01601, Ukraine.
| | - Adriana Nikitina
- Department of Internal Medicine #3, Bogomolets National Medical University, T. Shevchenko Boulevard, 13, Kiev, 01601, Ukraine
| | - Oksana Tarasenko
- The Therapy Department, Kyiv City Clinical Hospital #3, 26, P. Zaporozhtsia Street, Kiev, 02125, Ukraine
| | - Ihor Chechotenko
- Urology Department, Kyiv City Clinical Hospital #3, P. Zaporozhtsia Street, 26, Kiev, 02125, Ukraine
| | - Dmytro Mykhailov
- Urology Department, Kyiv City Clinical Hospital #3, P. Zaporozhtsia Street, 26, Kiev, 02125, Ukraine
| | - Vladyslav Kravchenko
- O. Bogomolets National Medical University, T. Shevchenko Boulevard, 13, Kiev, 01601, Ukraine
| | - Oleg Iaremenko
- Department of Internal Medicine #3, Bogomolets National Medical University, T. Shevchenko Boulevard, 13, Kiev, 01601, Ukraine
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29
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Nørregaard R, Mutsaers HAM, Frøkiær J, Kwon TH. Obstructive nephropathy and molecular pathophysiology of renal interstitial fibrosis. Physiol Rev 2023; 103:2827-2872. [PMID: 37440209 PMCID: PMC10642920 DOI: 10.1152/physrev.00027.2022] [Citation(s) in RCA: 33] [Impact Index Per Article: 16.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2022] [Revised: 07/05/2023] [Accepted: 07/09/2023] [Indexed: 07/14/2023] Open
Abstract
The kidneys play a key role in maintaining total body homeostasis. The complexity of this task is reflected in the unique architecture of the organ. Ureteral obstruction greatly affects renal physiology by altering hemodynamics, changing glomerular filtration and renal metabolism, and inducing architectural malformations of the kidney parenchyma, most importantly renal fibrosis. Persisting pathological changes lead to chronic kidney disease, which currently affects ∼10% of the global population and is one of the major causes of death worldwide. Studies on the consequences of ureteral obstruction date back to the 1800s. Even today, experimental unilateral ureteral obstruction (UUO) remains the standard model for tubulointerstitial fibrosis. However, the model has certain limitations when it comes to studying tubular injury and repair, as well as a limited potential for human translation. Nevertheless, ureteral obstruction has provided the scientific community with a wealth of knowledge on renal (patho)physiology. With the introduction of advanced omics techniques, the classical UUO model has remained relevant to this day and has been instrumental in understanding renal fibrosis at the molecular, genomic, and cellular levels. This review details key concepts and recent advances in the understanding of obstructive nephropathy, highlighting the pathophysiological hallmarks responsible for the functional and architectural changes induced by ureteral obstruction, with a special emphasis on renal fibrosis.
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Affiliation(s)
- Rikke Nørregaard
- Department of Clinical Medicine, Aarhus University, Aarhus, Denmark
- Department of Renal Medicine, Aarhus University Hospital, Aarhus, Denmark
| | | | - Jørgen Frøkiær
- Department of Clinical Medicine, Aarhus University, Aarhus, Denmark
| | - Tae-Hwan Kwon
- Department of Biochemistry and Cell Biology, School of Medicine, Kyungpook National University, Taegu, Korea
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30
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Vijayan J, Bora H, Ali A, K R D. Investigating the Link Between Low Back Ache, Abdominal Discomfort and Oliguria: A Unique Case of Histopathology-Proven Retroperitoneal Fibrosis. Cureus 2023; 15:e44220. [PMID: 37767266 PMCID: PMC10522402 DOI: 10.7759/cureus.44220] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/26/2023] [Indexed: 09/29/2023] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare condition characterized by systemic inflammation and the proliferation of fibroinflammatory tissues in the retroperitoneum. It may lead to the formation of a retroperitoneal mass and can encase the aorta, its branches and ureters. The pathogenesis of RPF is not fully known. We report a case of a 52-year-old male presented with low back ache, flank pain, swelling of legs, oliguria and features of obstructive uropathy, later diagnosed to be RPF. The mainstay of diagnosis includes blood workup, imaging and biopsy. The first line of treatment is corticosteroids. Surgical intervention is carried out when medical measures have failed or when contraindicated. Early diagnosis and prevention of complications is the key, and a high degree of suspicion is needed.
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Affiliation(s)
| | | | - Amir Ali
- Pharmacy, National Institute of Pharmaceutical Education and Research, Guwahati, IND
| | - Devika K R
- Radiodiagnosis, Fakhruddin Ali Ahmed Medical College and Hospital, Barpeta, IND
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31
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Gao H, Liu S, Mai Y, Wang Y, Zhang X, Zheng S, Luo C, Pan C. Combined therapy of prednisone and mTOR inhibitor sirolimus for treating retroperitoneal fibrosis. Ann Rheum Dis 2023; 82:688-697. [PMID: 36720581 PMCID: PMC10176363 DOI: 10.1136/ard-2022-223736] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2022] [Accepted: 01/19/2023] [Indexed: 02/02/2023]
Abstract
OBJECTIVES Retroperitoneal fibrosis (RPF) is a rare autoimmune disease with fibrous tissue growth and inflammation in retroperitoneum. Its current treatments involve long-term uptake of glucocorticoids (e.g., prednisone) for controlling inflammation; however, side effects are common. We strived for an improved therapy for fibrosis remission while reducing side effects. METHODS We surveyed gene-disease-drug databases and discovered that mammalian target of rapamycin (mTOR) was a key signalling protein in RPF and the mTOR inhibitor compound sirolimus affected many RPF pathways. We designed a therapy combining a gradual reduction of prednisone with a long-term, stable dosage of sirolimus. We then implemented a single-arm clinical trial and assessed the effects in eight RPF patients at 0, 12 and 48 weeks of treatment by measuring fibrous tissue mass by CT, markers of inflammation and kidney functions by lab tests, immune cell profiles by flow cytometry and plasma inflammatory proteins by Olink proteomics. RESULTS With the combined therapy, fibrous tissue shrunk about by half, markers of acute inflammation reduced by 70% and most patients with abnormal kidney functions had them restored to normal range. Molecularly, fibrosis-related T cell subsets, including TH2, TH17 and circulating TFH cells, were reduced and tumour necrosis factor and related cytokines restored to healthy levels. No severe long-term side effects were observed. CONCLUSIONS Our combined therapy resulted in significant fibrosis remission and an overall regression of the immune system towards healthy states, while achieving good tolerance. We concluded that this new therapy had the potential to replace the steroid monotherapy for treating RPF.
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Affiliation(s)
- Hui Gao
- Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China
| | - Shibo Liu
- Department of Retroperitoneal Tumor Surgery, Peking University International Hospital, Beijing, China
| | - Yuanbang Mai
- Center for Intelligent Medicine Research, Greater Bay Area Institute of Precision Medicine (Guangzhou), Fudan University, Guangzhou, China
- School of Life Sciences, Fudan University, Shanghai, China
| | - Yuying Wang
- Department of Pharmacy, Peking University International Hospital, Beijing, China
| | - Xuewu Zhang
- Department of Rheumatology, Peking University People's Hospital, Beijing, China
| | - Shufen Zheng
- Center for Intelligent Medicine Research, Greater Bay Area Institute of Precision Medicine (Guangzhou), Fudan University, Guangzhou, China
- School of Life Sciences, Fudan University, Shanghai, China
| | - Chenghua Luo
- Department of Retroperitoneal Tumor Surgery, Peking University International Hospital, Beijing, China
| | - Cuiping Pan
- Center for Intelligent Medicine Research, Greater Bay Area Institute of Precision Medicine (Guangzhou), Fudan University, Guangzhou, China
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32
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Numata Y, Sasaki S, Magara K, Takasawa A, Sugawara T, Ohara N, Akutsu N, Hasegawa T, Osanai M, Nakase H. Generalized crystal-storing histiocytosis with noncirrhotic portal hypertension: an autopsy case report. Clin J Gastroenterol 2023; 16:450-456. [PMID: 37014540 DOI: 10.1007/s12328-023-01782-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/06/2022] [Accepted: 03/06/2023] [Indexed: 04/05/2023]
Abstract
Crystal-storing histiocytosis (CSH) is a rare disease associated with the accumulation of histiocytes containing crystalline matter within their cytoplasm. Herein, we present the case of a female patient who was diagnosed with Tolosa-Hunt syndrome at 45 years of age and idiopathic retroperitoneal fibrosis when she was 48 years. She developed portal hypertension (PH), but did not present with cirrhosis; as such, the cause of PH was not identified. Her PH gradually worsened when she was 54 years, and at the age of 60 years, she died from an acute subdural hematoma. Autopsy revealed retroperitoneal fibrosis with severe fibrosis extending around the hepatic veins and into the porta hepatis. Histologically, the retroperitoneal tissue showed a dense infiltrate of eosinophilic histiocytes with crystal structures in the cytoplasm, which was pathologically diagnosed as CSH. Nodular regenerative hyperplasia was observed in the liver parenchyma, whereas cirrhosis was not. In the present case, CSH caused fibrosis, which was believed to be the cause of PH. In addition, we considered that nodular regenerative hyperplasia caused by the altered hepatic blood flow due to treatment of gastric varices contributed to worsening PH. Hence, CSH should be considered as an underlying disease in noncirrhotic portal hypertension.
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Affiliation(s)
- Yasunao Numata
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
| | - Shigeru Sasaki
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan.
| | - Kazufumi Magara
- Department of Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Akira Takasawa
- Department of Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Taro Sugawara
- Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Naruki Ohara
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
| | - Noriyuki Akutsu
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
| | - Tadashi Hasegawa
- Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Makoto Osanai
- Department of Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Hiroshi Nakase
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
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33
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Korol PO, Lukashenko MS, Shcherbina OV, Murashko VO, Ivchuk VP. Роль позитронно-емісійної томографії при інфекційних та запальних захворюваннях (огляд літератури). UKRAINIAN JOURNAL OF MILITARY MEDICINE 2023; 4:104-115. [DOI: 10.46847/ujmm.2023.1(4)-104] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
Abstract
Мета. Дослідити клінічну роль методів гібридної променевої візуалізації, зокрема позитронно-емісійної томографії (ПЕТ) у діагностиці та лікуванні пацієнтів із запальними та інфекційними захворюваннями.
Матеріали та методи. Матеріалом для дослідження були наукові результати публікацій фахових наукових видань провідних країн світу наукової та клінічної діяльності за останні 10 років щодо клінічного значення методів гібридної променевої візуалізації у діагностиці та лікуванні пацієнтів із запальними та інфекційними захворюваннями. Методи дослідження передбачали застосування методів променевої візуалізації ПЕТ при інфекційних та запальних захворюваннях.
Результати. Показана ефективність ПЕТ у випадках, коли інші звичайні дослідження є неможливими через високий ризик ускладнень. В роботі розглянуто корисність методу при діагностиці станів, які виникають як ускладнення після протезування: при ендокардитах протезованого клапану; інфекції імплантованих електронних пристроїв; протезованих суглобів та імплантантів для фіксації переломів. ПЕТ в таких випадках дозволяє провести диференційну діагностику між асептичним та інфекційним запаленням, особливо за наявності спричинених металевими елементами артефактів, які можуть обмежують дослідження за допомогою комп’ютерної томографії або магнітно-резонансної томографії.
Висновки. Позитронно-емісійна томографія у пацієнтів із запальними або інфекційними захворюваннями є ефективним допоміжним додатковим методом променевої візуалізації. ПЕТ знаходить своє ефективне застосування на різних етапах лікування, а також є корисним методом променевої візуалізації для моніторингу терапевтичної відповіді на лікування.
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Huynh KN, Kong MJ, Nguyen BD. Anatomic and Functional Imaging of Immunoglobulin G4-related Disease and Its Mimics. Radiographics 2023; 43:e220097. [PMID: 36821510 DOI: 10.1148/rg.220097] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/24/2023]
Abstract
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibrosclerosing disease with tumefactive lesions infiltrated by IgG4-positive plasma cells. Initially described as autoimmune pancreatitis, IgG4-RD is now recognized as a discrete entity and is found to affect virtually any organ in the body. Common extrapancreatic sites include the biliary tree, salivary glands, periorbital tissue, lungs, kidneys, lymph nodes, aorta, retroperitoneum, and thyroid gland. Diagnosis-which relies on histopathologic, serologic, and radiologic features-can be challenging with the disease underdiagnosed, as IgG4-RD often mimics malignancy, infectious processes, or other immune-mediated conditions. Patients may present with signs of compression of nearby structures due to mass effect or with organ failure when the disease is left untreated. The clinical course is complex, with single- or multiorgan involvement and metachronous or synchronous occurrence of lesions. IgG4-RD responds well to glucocorticoid therapy, disease-modifying antirheumatic drugs, and B-cell-depleting biologic agents; prompt diagnosis is important to avoid delay in treatment and unnecessary pharmacologic or surgical intervention. While imaging features may not be specific for IgG4-RD, functional whole-body imaging with fluorine 18-fluorodeoxyglucose PET/CT is a useful adjunct for localizing extrapancreatic sites for biopsy, monitoring therapeutic response, and demonstrating disease relapse. The authors describe the pancreatic and extrapancreatic sites of involvement in IgG4-RD, with imaging features and patterns to aid in distinguishing IgG4-RD from its mimics through a multimodality approach with emphasis on functional imaging evaluation. ©RSNA, 2023 Quiz questions in the supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article.
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Affiliation(s)
- Kenneth N Huynh
- From the Department of Radiological Sciences, University of California-Irvine, Orange, Calif (K.N.H.); and Department of Radiology, Mayo Clinic Scottsdale, 13400 E Shea Blvd, Scottsdale, AZ 85259 (M.J.K., B.D.N.)
| | - Min J Kong
- From the Department of Radiological Sciences, University of California-Irvine, Orange, Calif (K.N.H.); and Department of Radiology, Mayo Clinic Scottsdale, 13400 E Shea Blvd, Scottsdale, AZ 85259 (M.J.K., B.D.N.)
| | - Ba D Nguyen
- From the Department of Radiological Sciences, University of California-Irvine, Orange, Calif (K.N.H.); and Department of Radiology, Mayo Clinic Scottsdale, 13400 E Shea Blvd, Scottsdale, AZ 85259 (M.J.K., B.D.N.)
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Gianfreda D, Superchi E, Peyronel F, Mazzariol M, Vaglio A. Chronic periaortitis: A clinical approach. Rev Med Interne 2023; 44:79-84. [PMID: 36697342 DOI: 10.1016/j.revmed.2022.11.009] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2022] [Revised: 11/13/2022] [Accepted: 11/30/2022] [Indexed: 01/25/2023]
Abstract
Chronic periaortitis (CP) is a rare disease characterised by the presence of a fibro-inflammatory tissue typically enveloping the abdominal aorta, the iliac arteries and, in some cases, the nearby structures, such as the ureters and the inferior vena cava. Imaging plays a key role in the diagnosis and follow-up: computed tomography and magnetic resonance imaging scans are used to define the extension of the pathological tissue, whereas fluorodeoxyglucose positron emission tomography is the gold standard to establish the degree of its metabolic activity. CP must be distinguished from secondary forms of periaortic infiltration, which include malignant, infectious, and drug-related aetiologies. This review focuses on the clinical aspects of CP and the differential diagnosis with secondary cases, and aims to provide the clinician with a guide through this challenging clinical approach.
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Affiliation(s)
- D Gianfreda
- Nephrology and Dialysis Unit, Santa Caterina Novella Hospital, Galatina, Italy
| | - E Superchi
- Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze, Italy
| | - F Peyronel
- Department of Experimental and Clinical Medicine, University of Fireze, Firenze, Italy; Nephrology and Dialysis Unit, Department of Pediatrics, Meyer Children's Hospital, Firenze, Italy
| | - M Mazzariol
- Department of Medical Sciences, University of Torino, Torino, Italy
| | - A Vaglio
- Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze, Italy; Nephrology and Dialysis Unit, Department of Pediatrics, Meyer Children's Hospital, Firenze, Italy.
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Muacevic A, Adler JR, Bazigh K, Duhan C, Alhamdan N. Retroperitoneal Fibrosis: Still a Diagnostic Challenge. Cureus 2023; 15:e33998. [PMID: 36811048 PMCID: PMC9939012 DOI: 10.7759/cureus.33998] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/20/2023] [Indexed: 01/22/2023] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disorder usually involving the abdominal aorta and surrounding structures. It is divided into primary (idiopathic) and secondary RPF. Primary RPF can be immunoglobulin (Ig) G4-related disease or non-IgG4-related disease. Recently, there has been a rise in case reports regarding the topic, but awareness about the disease is still far from ideal. Hence, we present the case of a 49-year-old female who had repeated admissions for chronic abdominal pain attributed to chronic alcoholic pancreatitis. She had a medical history significant for psoriasis and surgical history significant for cholecystectomy. Her computed tomography (CT) scans on each admission for the last year showed some signs of RPF, but it was never considered the primary etiology of her chronic symptoms. We also obtained magnetic resonance imaging (MRI) which did not show any underlying malignancy but showed the progression of her RPF. She was started on a steroid regimen, which significantly improved her symptoms. She was diagnosed with idiopathic RPF due to unclear etiology, although her underlying risk factors, including psoriasis, past surgeries, and pancreatitis-associated inflammation, were considered predisposing factors. Idiopathic RPF accounts for more than two-thirds of total cases of RPF. Patients with autoimmune diseases can overlap with other autoimmune disorders. For non-malignant RPF, medical management with 1mg/kg/day steroids is deemed effective. Still, there is a lack of prospective trials and consensus for guidelines on treating RPF. The follow-up involves laboratory tests, including erythrocyte sedimentation rate, C-reactive protein, and CT or MRI in an outpatient setting to identify treatment response and relapse. There is a need for more streamlined guidelines to diagnose and manage this disease.
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Pisciotta A, Di Tinco R, Bertani G, Orlandi G, Bertoni L, Pignatti E, Orciani M, Sena P, Bertacchini J, Salvarani C, Carnevale G. Human dental pulp stem cells (hDPSCs) promote the lipofibroblast transition in the early stage of a fibro-inflammatory process. Front Cell Dev Biol 2023; 11:1196023. [PMID: 37206922 PMCID: PMC10189147 DOI: 10.3389/fcell.2023.1196023] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/29/2023] [Accepted: 04/18/2023] [Indexed: 05/21/2023] Open
Abstract
Introduction: In autoimmune diseases, particularly in systemic sclerosis and chronic periaortitis, a strict correlation between chronic inflammation and fibrosis exists. Since the currently used drugs prove mostly effective in suppressing inflammation, a better comprehension of the molecular mechanisms exerted by cell types implicated in fibro-inflammation is needed to develop novel therapeutic strategies. Mesenchymal stromal/stem cells (MSCs) are being matter of deep investigation to unveil their role in the evolution of fibrogenetic process. Several findings pointed out the controversial implication of MSCs in these events, with reports lining at a beneficial effect exerted by external MSCs and others highlighting a direct contribution of resident MSCs in fibrosis progression. Human dental pulp stem cells (hDPSCs) have demonstrated to hold promise as potential therapeutic tools due to their immunomodulatory properties, which strongly support their contribution to tissue regeneration. Methods: Our present study evaluated hDPSCs response to a fibro-inflammatory microenvironment, mimicked in vitro by a transwell co-culture system with human dermal fibroblasts, at early and late culture passages, in presence of TGF-β1, a master promoter of fibrogenesis. Results and Discussion: We observed that hDPSCs, exposed to acute fibro-inflammatory stimuli, promote a myofibroblast-to-lipofibroblast transition, likely based on BMP2 dependent pathways. Conversely, when a chronic fibro-inflammatory microenvironment is generated, hDPSCs reduce their anti-fibrotic effect and acquire a pro-fibrotic phenotype. These data provide the basis for further investigations on the response of hDPSCs to varying fibro-inflammatory conditions.
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Affiliation(s)
- Alessandra Pisciotta
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
- *Correspondence: Alessandra Pisciotta,
| | - Rosanna Di Tinco
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Giulia Bertani
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Giulia Orlandi
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Laura Bertoni
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Elisa Pignatti
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Monia Orciani
- Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy
| | - Paola Sena
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Jessika Bertacchini
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
| | - Carlo Salvarani
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
- Unit of Rheumatology, Azienda Unità Sanitaria Locale-IRCCS, Reggio Emilia, Italy
| | - Gianluca Carnevale
- Department of Surgery, Medicine, Dentistry and Morphological Sciences with Interest in Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
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Yokota R, Sakamoto SI, Murata T, Hiromoto A, Yamaguchi T, Suzuki K, Kobayashi M, Kure S, Takeno M, Ishii Y. A Case of Contained Rupture of the Common Iliac Artery with Idiopathic Retroperitoneal Fibrosis: Efficacy of Surgical Treatment and Immunosuppressive Therapy at 2-Year Follow-Up. Ann Vasc Dis 2023; 16:95-99. [PMID: 37006861 PMCID: PMC10064296 DOI: 10.3400/avd.cr.22-00120] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2022] [Accepted: 01/13/2023] [Indexed: 03/04/2023] Open
Abstract
Rupture of inflammatory aortic aneurysm associated with retroperitoneal fibrosis (RF) is rare. We report a 62-year-old man with an inflammatory abdominal aortic aneurysm (IAAA) complicated with idiopathic RF, resulting in a contained rupture of the common iliac artery. The patient also presented with mild renal insufficiency due to urethral obstruction and left hydronephrosis. Surgical procedures including graft replacement and ureterolysis relieved the symptoms. Postoperative immunosuppressive treatment using corticosteroid and methotrexate successfully maintained clinical remission without signs of recurrence of RF and IAAA at the 2-year follow-up.
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Affiliation(s)
- Ryo Yokota
- Department of Cardiovascular Surgery, Nippon Medical School Musashikosugi Hospital
| | - Shun-Ichiro Sakamoto
- Department of Cardiovascular Surgery, Nippon Medical School Musashikosugi Hospital
| | - Tomohiro Murata
- Department of Cardiovascular Surgery, Nippon Medical School Musashikosugi Hospital
| | - Atsushi Hiromoto
- Department of Cardiovascular Surgery, Nippon Medical School Musashikosugi Hospital
| | - Takako Yamaguchi
- Department of Cardiovascular Surgery, Nippon Medical School Musashikosugi Hospital
| | - Kenji Suzuki
- Department of Cardiovascular Surgery, Nippon Medical School Musashikosugi Hospital
| | - Michiko Kobayashi
- Department of Integrated Diagnostic Pathology, Nippon Medical School Musashi-kosugi Hospital
| | - Shoko Kure
- Department of Integrated Diagnostic Pathology, Nippon Medical School Musashi-kosugi Hospital
| | - Mitsuhiro Takeno
- Department of Allergy and Rheumatology, Nippon Medical School Musashikosugi Hospital
| | - Yosuke Ishii
- Department of Cardiovascular Surgery, Nippon Medical School
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Liu L, Yan H, Wang Y, Xie Y, Jiang L, Zhao J, Gao C, Li X, Wang C. Decreased absolute number of peripheral regulatory T cells in patients with idiopathic retroperitoneal fibrosis. Front Immunol 2022; 13:1012513. [PMID: 36524110 PMCID: PMC9744965 DOI: 10.3389/fimmu.2022.1012513] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2022] [Accepted: 11/16/2022] [Indexed: 11/30/2022] Open
Abstract
Objective In order to determine whether the immune balance of T helper 17(Th17)/regulatory T(Treg) is related to the pathogenesis of idiopathic retroperitoneal fibrosis (IRPF), we analyzed the differences in peripheral blood lymphocytes, CD4+T cell subsets and cytokines between patients with IRPF and healthy people to clarify the CD4+T cell subsets, especially Treg cell subsets, and the role of cytokines in the pathogenesis of IRPF. Methods This study included 22 patients with IRPF, 36 patients with IgG4-related diseases (IgG4-RD) without retroperitoneal fibrosis (RPF), and 28 healthy controls. The absolute numbers and percentage of peripheral blood lymphocyte subsets and CD4+T cell subsets in each group were detected by flow cytometry, and the serum cytokine level was detected by flow cytometric bead array (CBA). Results Compared with the healthy group, the absolute value of B cells in peripheral blood of IRPF patients was significantly decreased, and T, natural killer (NK), CD4+ and CD8+ were not significantly abnormal. The absolute numbers of Th2 cells were lower than healthy group(p=0.043). In particular, the absolute numbers of Treg cells were significantly lower than healthy group(p<0.001), while the absolute numbers of Th17 cells increased(p=0.682). Th17/Treg was significantly higher than healthy group (p< 0.001). Cytokine analysis showed that the level of interleukin (IL)-4 in IRPF patients was higher than healthy group(p=0.011), IL-6, IL-10, IL-17, TNF-α and IFN-γ were significantly higher than healthy group (all p<0.001). Receiver operating characteristic (ROC) curves showed that IL-10 and TNF-α could distinguish bilateral ureteral dilatation in IRPF patients, with areas under the ROC curve (AUCs) of 0.813 (95% CI:0.607-1.000, p=0.026) and 0.950 (95% CI:0.856-1.000, p=0.001), respectively. IL-6 could distinguish bilateral ureteral obstruction, with an AUC of 0.861 (95% CI: 0.682-1.000, p=0.015). Conclusions Our study showed that IRPF patients had reduced Treg cells and indeed had Th17/Treg imbalance, which may be related to the pathogenesis of the disease. The levels of IL-6, IL-10 and TNF-α appear to be associated with the progression of IRPF.
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Affiliation(s)
- Lu Liu
- Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, China,Shanxi Key Laboratory of Immunomicroecology, Taiyuan, Shanxi, China
| | - Huanhuan Yan
- Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, China,Shanxi Key Laboratory of Immunomicroecology, Taiyuan, Shanxi, China
| | - Yanyan Wang
- Department of Rheumatology, Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, Shanxi, China
| | - Yuhuan Xie
- Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, China,Shanxi Key Laboratory of Immunomicroecology, Taiyuan, Shanxi, China
| | - Lei Jiang
- Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, China,Shanxi Key Laboratory of Immunomicroecology, Taiyuan, Shanxi, China
| | - Jinfang Zhao
- Department of Medical Statistics, Shanxi Medical University, Taiyuan, Shanxi, China
| | - Chong Gao
- Pathology, Joint Program in Transfusion Medicine, Brigham and Women’s Hospital/Children’s Hospital, Harvard Medical School, Boston, MA, United States
| | - Xiaofeng Li
- Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, China,Shanxi Key Laboratory of Immunomicroecology, Taiyuan, Shanxi, China
| | - Caihong Wang
- Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, China,Shanxi Key Laboratory of Immunomicroecology, Taiyuan, Shanxi, China,*Correspondence: Caihong Wang,
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Muacevic A, Adler JR, Song T, Gan L, Sun W. A Case of Follicular Lymphoma Mimicking Idiopathic Retroperitoneal Fibrosis. Cureus 2022; 14:e32031. [PMID: 36600821 PMCID: PMC9800343 DOI: 10.7759/cureus.32031] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/29/2022] [Indexed: 12/05/2022] Open
Abstract
Retroperitoneal fibrosis (RF) is a rare disease, which can be primary (idiopathic) or secondary. We present the case of a 56-year-old patient with symptomatic RF, in whom, after ineffective treatment with glucocorticoids, immunosuppressants, and non-steroidal anti-inflammatory drugs for one year and a progressive clinical course, a follicular lymphoma in the retroperitoneal space and several lymphoma nodes was identified. We also include a literature review on differential diagnosis through image inspection and case reports of lymphoma mimicking RF.
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Si M, Zhang K, Li J, He H, Yao Y, Han J, Qiao J. Idiopathic retroperitoneal fibrosis with endometrial cancer: a case report and literature review. BMC Womens Health 2022; 22:399. [PMID: 36183056 PMCID: PMC9526921 DOI: 10.1186/s12905-022-01968-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2022] [Accepted: 09/11/2022] [Indexed: 11/09/2022] Open
Abstract
Background Retroperitoneal fibrosis is a rare disease characterized by chronic nonspecific inflammation, which leads to clinical compression manifestations of retroperitoneal organs especially ureter. Approximately 70 percent of retroperitoneal fibrosis cases are idiopathic which has no clear etiology. This study reported a rare case of a 48-year-old woman presented with idiopathic retroperitoneal fibrosis and endometrial cancer. Case presentation A 48-year-old woman presented with irregular vaginal bleeding without abdominal pain, bloating or discomfort. The patient was diagnosed iRPF after splenectomy 13 years ago. Then she took prednisone for 2 years and took tamoxifen for about 11 years. She stopped taking the medication from October 2019 to May 2020 and then started taking tamoxifen again until November 2020. Two weeks after she stopped taking tamoxifen, she presented with irregular vaginal bleeding. Gynecological ultrasound revealed a thick endometrium with uneven echo enhancement and blood flow signals. Then diagnostic curettage was performed with pathological examination showed endometroid carcinoma. Later, the patient was admitted to Peking University Third Hospital for surgery. Preoperative imaging examinations, including CT, MRI, and PET/CT, all showed pelvic enlarged lymph nodes and they were highly suspected to have lymph node metastasis. The patient underwent laparoscopic surgical staging and enlarged lymph nodes in the pelvic and aortic regions were removed. Finally, the pathology confirmed that endometrioid adenocarcinoma and fibrosis, but there was no tumor infiltration in these enlarged lymph nodes. The patient is now in good condition. Conclusion This case report stressed the difficulty to distinguish between lymph node metastasis and inflammatory hyperplasia by common imaging methods. Due to increased surgical difficulty among retroperitoneal patients, lymphadenectomy should be carefully evaluated to avoid additional surgical complications and over-treatment.
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Affiliation(s)
- Manfei Si
- grid.411642.40000 0004 0605 3760Center for Reproductive Medicine, Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.419897.a0000 0004 0369 313XKey Laboratory of Assisted Reproduction (Peking University), Ministry of Education, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Beijing Key Laboratory of Reproductive Endocrinology and Assisted Reproductive Technology, Beijing, 100191 China
| | - Kun Zhang
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Jiaxin Li
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Huiying He
- grid.411642.40000 0004 0605 3760Department of Pathology, Peking University Third Hospital, Beijing, 100191 China
| | - Ying Yao
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Jinsong Han
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Jie Qiao
- grid.411642.40000 0004 0605 3760Center for Reproductive Medicine, Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.419897.a0000 0004 0369 313XKey Laboratory of Assisted Reproduction (Peking University), Ministry of Education, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Beijing Key Laboratory of Reproductive Endocrinology and Assisted Reproductive Technology, Beijing, 100191 China
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Raval DM, Rathod VM, Dave M, Patel NS, Dobariya R. Idiopathic Retroperitoneal Fibrosis Presented As Urinary Tract Obstruction. Cureus 2022; 14:e29582. [PMID: 36320943 PMCID: PMC9597267 DOI: 10.7759/cureus.29582] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/25/2022] [Indexed: 11/23/2022] Open
Abstract
Retroperitoneal fibrosis (RPF) or Ormond’s disease is a very uncommon fibro-inflammatory disease, under the umbrella of systemic autoimmune diseases. The majority of cases are idiopathic, known as idiopathic RPF (IRPF); however, diseases secondary to other causes are also seen in clinical practice. The commonest presenting features are seen due to the effects of fibrous tissue around iliac vessels, aorta and ureters, where compression of ureters is the major and most common complication. Computed tomography (CT) scans and magnetic resonance imaging (MRI) are the modalities of choice for the diagnosis. The primary management involves medical therapy with corticosteroids and reserving surgical options for ureteric obstruction and related complications. We present a case of a 65-year-old man who presented with bilateral pedal oedema, facial puffiness, decreased appetite, decreased urine output, and breathlessness with dry cough, tachypnoea, hypoxia and crepitation in both lung fields on examination. The blood investigations were suggestive of acute kidney injury (AKI); whereas radio imaging diagnosed him as a case of bilateral hydroureteronephrosis with RPF. The patient was treated for AKI in the case of IRPF. Once the patient stabilized, a low-dose systemic steroid was started for IRPF, and subsequently, the patient underwent stent placement surgery for ureteric obstruction. RPF, being a rare disease, is difficult to diagnose. However, CT and MRI scanning can easily reveal fibrous tissue surrounding the aorta and ureters. Medical management with glucocorticoids is the backbone drug for the disease, keeping surgery as a reserved option for ureteric obstruction and its complications.
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Sabanis N, Achilleas B, Eleni P. Immunoglobulin G4-related disease with large-size vessel involvement is more diverse than originally thought. J Vasc Surg Cases Innov Tech 2022; 8:872. [PMID: 36568955 PMCID: PMC9768225 DOI: 10.1016/j.jvscit.2022.07.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022] Open
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Sultan S, Acharya Y, Hezima M, Ramjohn J, Miresse D, Chua Vi Long K, Soliman O, Hynes N. Management of retroperitoneal fibrosis with endovascular aneurysm repair in patients refractory to medical management. Front Surg 2022; 9:946675. [PMID: 35928031 PMCID: PMC9345513 DOI: 10.3389/fsurg.2022.946675] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2022] [Accepted: 06/29/2022] [Indexed: 11/19/2022] Open
Abstract
Background Early diagnosis and treatment of under-recognized retroperitoneal fibrosis (RPF) are essential before reaching the poorly responsive fibrotic stage. Although most patients respond to medical therapy, relapses and unresponsiveness are common. However, open surgery in medically resistant patients is associated with major adverse clinical events. Methods This is a single-centre longitudinal study of optimal medical therapy (OMT) vs. endovascular aneurysm repair (EVAR) in patients presenting with RPF to our tertiary referral vascular centre. Out of 22,349 aortic referrals, we performed 1,555 aortic interventions over twenty years. Amongst them, 1,006 were EVAR, TEVAR and BEVAR. Seventeen patients (1.09%) had documented peri-aortic RPF. Results Out of the 17 RPF patients, 11 received OMT only, while 6 underwent EVAR after the failure of OMT. 82% (n = 14) were male, and the median follow-up was 62.7 months (IQR: 28.2–106). Nine (52%) had immunoglobulin G4-related disease (4 OMT vs. 5 EVAR). EVAR patients had 100% technical success without perioperative mortality. Furthermore, all the EVAR patients were symptom-free following the intervention. Pre-operative aortic RPF index (maximum peri-aortic soft tissue diameter/maximum aortic diameter) was higher in the EVAR than in OMT. However, there was a significant decrement in the aortic RPF index following EVAR (P = 0.04). Conclusion We believe that when optimal medical therapy fails in RPF, EVAR provides a promising outcome. Further studies are recommended to establish the role of endovascular repair.
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Affiliation(s)
- Sherif Sultan
- Western Vascular Institute, Department of Vascular and Endovascular Surgery, University Hospital Galway, National University of Ireland, Galway, Ireland
- Galway Clinic, Royal College of Surgeons in Ireland and National University of Ireland, Galway affiliated Hospital, Doughiska, Ireland
- CÚRAM-CORRIB-Vascular Group, National University of Ireland, Galway, Ireland
| | - Yogesh Acharya
- Western Vascular Institute, Department of Vascular and Endovascular Surgery, University Hospital Galway, National University of Ireland, Galway, Ireland
- Galway Clinic, Royal College of Surgeons in Ireland and National University of Ireland, Galway affiliated Hospital, Doughiska, Ireland
| | - Mohieldin Hezima
- Western Vascular Institute, Department of Vascular and Endovascular Surgery, University Hospital Galway, National University of Ireland, Galway, Ireland
| | - Joshua Ramjohn
- Western Vascular Institute, Department of Vascular and Endovascular Surgery, University Hospital Galway, National University of Ireland, Galway, Ireland
| | - David Miresse
- Western Vascular Institute, Department of Vascular and Endovascular Surgery, University Hospital Galway, National University of Ireland, Galway, Ireland
| | - Keegan Chua Vi Long
- Western Vascular Institute, Department of Vascular and Endovascular Surgery, University Hospital Galway, National University of Ireland, Galway, Ireland
| | - Osama Soliman
- CÚRAM-CORRIB-Vascular Group, National University of Ireland, Galway, Ireland
| | - Niamh Hynes
- CÚRAM-CORRIB-Vascular Group, National University of Ireland, Galway, Ireland
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Serum IgG4 Concentration Is a Potential Predictive Biomarker in Glucocorticoid Treatment for Idiopathic Retroperitoneal Fibrosis. J Clin Med 2022; 11:jcm11123538. [PMID: 35743608 PMCID: PMC9224541 DOI: 10.3390/jcm11123538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2022] [Revised: 06/17/2022] [Accepted: 06/18/2022] [Indexed: 12/04/2022] Open
Abstract
Objectives: To evaluate the management and outcome of idiopathic retroperitoneal fibrosis (iRPF) in Japan, and to identify its clinical biomarker. Methods: We retrospectively analyzed 129 patients with iRPF treated between January 2008 and May 2018 at 12 university and related hospitals. Patients treated with glucocorticoid were analyzed to identify a predictive biomarker. These patients were classified into three groups according to overall effectiveness (no change: NC, complete response: CR, and partial response groups: PR), and each parameter was compared statistically. Results: Male–female ratio was 5:1, and median age at diagnosis was 69 (33–86) years. Smoking history was reported in 59.6% of the patients. As treatment, 95 patients received glucocorticoid therapy with an overall response rate of 84%. As a result, serum concentration of IgG4 was significantly decreased in NC group compared with the other two groups (56.6 mg/dL vs. 255 mg/dL, 206 mg/dL, p = 0.0059 and 0.0078). ROC analysis was performed between the nonresponder (NC) and responder groups (CR + PR) to identify the cut-off value of serum IgG4 as a predictive marker. As a result, AUC of 0.793 was confirmed. Conclusions: Pre-treatment serum IgG4 concentration may have potential as a predictive biomarker of steroid treatment.
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Ishihama Y, Fukumoto K, Watanabe R, Nakatani S, Tsuda A, Otoshi T, Yamada K, Yamada S, Negoro N, Emoto M, Hashimoto M. Retroperitoneal fibrosis requiring prompt nephrostomy in a case with immunoglobulin A vasculitis. Scand J Rheumatol 2022; 51:419-421. [PMID: 35658823 DOI: 10.1080/03009742.2022.2047312] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Affiliation(s)
- Y Ishihama
- Department of Clinical Immunology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - K Fukumoto
- Department of Clinical Immunology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - R Watanabe
- Department of Clinical Immunology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - S Nakatani
- Department of Metabolism, Endocrinology, and Molecular Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - A Tsuda
- Department of Metabolism, Endocrinology, and Molecular Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - T Otoshi
- Department of Urology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - K Yamada
- Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - S Yamada
- Department of Clinical Immunology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - N Negoro
- Department of Clinical Immunology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - M Emoto
- Department of Metabolism, Endocrinology, and Molecular Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - M Hashimoto
- Department of Clinical Immunology, Osaka City University Graduate School of Medicine, Osaka, Japan
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Wang KK, El Khoury R, Joob A, Jacobs CE, White JV, Schwartz LB. Thoracobifemoral bypass for infrarenal aortic occlusion caused by retroperitoneal fibrosis. J Vasc Surg Cases Innov Tech 2022; 8:132-135. [PMID: 35330901 PMCID: PMC8938248 DOI: 10.1016/j.jvscit.2022.01.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2021] [Accepted: 01/18/2022] [Indexed: 11/25/2022] Open
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Abramicheva PA, Plotnikov EY. Hormonal Regulation of Renal Fibrosis. Life (Basel) 2022; 12:737. [PMID: 35629404 PMCID: PMC9143586 DOI: 10.3390/life12050737] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2022] [Revised: 05/11/2022] [Accepted: 05/13/2022] [Indexed: 11/16/2022] Open
Abstract
Fibrosis is a severe complication of many acute and chronic kidney pathologies. According to current concepts, an imbalance in the synthesis and degradation of the extracellular matrix by fibroblasts is considered the key cause of the induction and progression of fibrosis. Nevertheless, inflammation associated with the damage of tissue cells is among the factors promoting this pathological process. Most of the mechanisms accompanying fibrosis development are controlled by various hormones, which makes humoral regulation an attractive target for therapeutic intervention. In this vein, it is particularly interesting that the kidney is the source of many hormones, while other hormones regulate renal functions. The normal kidney physiology and pathogenesis of many kidney diseases are sex-dependent and thus modulated by sex hormones. Therefore, when choosing therapy, it is necessary to focus on the sex-associated characteristics of kidney functioning. In this review, we considered renal fibrosis from the point of view of vasoactive and reproductive hormone imbalance. The hormonal therapy possibilities for the treatment or prevention of kidney fibrosis are also discussed.
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Affiliation(s)
- Polina A. Abramicheva
- Belozersky Institute of Physico-Chemical Biology, Lomonosov Moscow State University, 119991 Moscow, Russia;
| | - Egor Y. Plotnikov
- Belozersky Institute of Physico-Chemical Biology, Lomonosov Moscow State University, 119991 Moscow, Russia;
- Kulakov National Medical Research Center of Obstetrics, Gynecology and Perinatology, 117997 Moscow, Russia
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Sfogliarini C, Pepe G, Dolce A, Della Torre S, Cesta MC, Allegretti M, Locati M, Vegeto E. Tamoxifen Twists Again: On and Off-Targets in Macrophages and Infections. Front Pharmacol 2022; 13:879020. [PMID: 35431927 PMCID: PMC9006819 DOI: 10.3389/fphar.2022.879020] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2022] [Accepted: 03/08/2022] [Indexed: 11/25/2022] Open
Abstract
Beyond the wide use of tamoxifen in breast cancer chemotherapy due to its estrogen receptor antagonist activity, this drug is being assayed in repurposing strategies against a number of microbial infections. We conducted a literature search on the evidence related with tamoxifen activity in macrophages, since these immune cells participate as a first line-defense against pathogen invasion. Consistent data indicate the existence of estrogen receptor-independent targets of tamoxifen in macrophages that include lipid mediators and signaling pathways, such as NRF2 and caspase-1, which allow these cells to undergo phenotypic adaptation and potentiate the inflammatory response, without the induction of cell death. Thus, these lines of evidence suggest that the widespread antimicrobial activity of this drug can be ascribed, at least in part, to the potentiation of the host innate immunity. This widens our understanding of the pharmacological activity of tamoxifen with relevant therapeutic implications for infections and other clinical indications that may benefit from the immunomodulatory effects of this drug.
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Affiliation(s)
- Chiara Sfogliarini
- Department of Pharmaceutical Sciences, University of Milan, Milan, Italy
| | - Giovanna Pepe
- Department of Pharmaceutical Sciences, University of Milan, Milan, Italy
| | - Arianna Dolce
- Department of Pharmaceutical Sciences, University of Milan, Milan, Italy
| | - Sara Della Torre
- Department of Pharmaceutical Sciences, University of Milan, Milan, Italy
| | | | | | - Massimo Locati
- IRCCS Humanitas Research Hospital, Rozzano, Italy.,Department of Medical Biotechnologies and Translational Medicine, University of Milan, Milan, Italy
| | - Elisabetta Vegeto
- Department of Pharmaceutical Sciences, University of Milan, Milan, Italy
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Dattani R, Barwick TD, El Wardany G, Gibbons N, Mason JC, Morgan P, Pusey CD, Tam FWK, Tomlinson JAP. An international patient-centred study of retroperitoneal fibrosis. QJM 2022; 115:148-154. [PMID: 33377941 DOI: 10.1093/qjmed/hcaa327] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/22/2020] [Revised: 11/17/2020] [Indexed: 11/14/2022] Open
Abstract
BACKGROUND The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance. AIM To understand the physical and psychosocial impact that RPF has upon peoples' lives. DESIGN An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences. METHODS An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months. RESULTS A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically. CONCLUSION This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health.
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Affiliation(s)
- R Dattani
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
| | - T D Barwick
- Department of Surgery and Cancer, Imperial College London, London, UK
| | - G El Wardany
- Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK
| | - N Gibbons
- Department of Urology, Imperial College Healthcare NHS Trust, London, UK
| | - J C Mason
- Imperial College London, National Heart and Lung Institute London, London, UK
| | - P Morgan
- Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK
| | - C D Pusey
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
- Imperial College London, Centre for inflammatory Disease London, London, UK
| | - F W K Tam
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
- Imperial College London, Centre for inflammatory Disease London, London, UK
| | - J A P Tomlinson
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
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