|
1
|
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.
Collapse
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.
| |
Collapse
|
|
2
|
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.
Collapse
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.
| |
Collapse
|
|
3
|
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.
Collapse
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
| |
Collapse
|
|
4
|
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.
Collapse
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
| |
Collapse
|
|
5
|
Renganathan G, Thangarasu S, Pathak N, Kunam VK, Afzal Z. The Enigma of Retroperitoneal Fibrosis: Clinical Implications and Diagnostic and Therapeutic Challenges. Cureus 2024; 16:e74499. [PMID: 39726480 PMCID: PMC11670788 DOI: 10.7759/cureus.74499] [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: 08/08/2024] [Accepted: 11/26/2024] [Indexed: 12/28/2024] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare disease with a nonspecific presentation. RPF can be classified into Idiopathic, the most common, or secondary due to malignancy and various medications resulting in chronic inflammation and fibrosis in the retroperitoneum. The complications arise due to the compression of structures in the retroperitoneum. The most common presentations are constitutional symptoms, abdominal pain, and renal insufficiency due to ureteral obstruction. Venous thrombosis or claudication on presentation is rare. Diagnosis and treatment remain challenging due to the lack of standard diagnostic or treatment protocols. Our patient presented with symptoms of acute deep vein thrombosis (DVT) with varices on the abdomen and mild bilateral hydronephrosis. A CT scan revealed a retroperitoneal mass, which was confirmed to be RPF by biopsy. Relevant laboratory tests, including IgG4, were negative. High-dose corticosteroid therapy reduced the inflammatory markers and the size of the retroperitoneal mass.
Collapse
Affiliation(s)
- Gowri Renganathan
- Department of Internal Medicine, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, USA
| | - Sudhagar Thangarasu
- Department of Internal Medicine, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, USA
| | - Nibesh Pathak
- Internal Medicine, Tribhuvan University, Kathmandu, NPL
| | - Vamsi K Kunam
- Interventional Radiology, The Hospitals of Providence Transmountain, El Paso, USA
| | - Zeeshan Afzal
- Department of Internal Medicine/Rheumatology, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, USA
| |
Collapse
|
|
6
|
Krogh MB, Jespersen B, Gormsen LC, Budtz-Lilly J, Keller KK, Birn H. Inferior vena cava occlusion in patients with retroperitoneal fibrosis. Scand J Rheumatol 2024; 53:365-367. [PMID: 39105237 DOI: 10.1080/03009742.2024.2381919] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2024] [Accepted: 07/16/2024] [Indexed: 08/07/2024]
Affiliation(s)
- M B Krogh
- Department of Renal Medicine, Aarhus University Hospital, Aarhus, Denmark
| | - B Jespersen
- Department of Renal Medicine, Aarhus University Hospital, Aarhus, Denmark
| | - L C Gormsen
- Department of Nuclear Medicine and PET-Centre, Aarhus University Hospital, Aarhus, Denmark
| | - J Budtz-Lilly
- Department of Cardiothoracic and Vascular Surgery, Aarhus University Hospital, Aarhus, Denmark
| | - K K Keller
- Department of Rheumatology, Aarhus University Hospital, Aarhus, Denmark
- Department of Clinical Medicine, Aarhus University, Aarhus, Denmark
| | - H Birn
- Department of Renal Medicine, Aarhus University Hospital, Aarhus, Denmark
- Department of Biomedicine, Aarhus University, Aarhus, Denmark
| |
Collapse
|
|
7
|
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.
Collapse
Affiliation(s)
| | - Sara Rotunno
- Internal Medicine, Ospedale San Pietro Fatebenefratelli, Rome, ITA
| | | |
Collapse
|
|
8
|
Wang A, Peng L, Zhou J, Liu Z, Lu H, Peng Y, Li J, Fei Y, Zhang W. Pilot study of tocilizumab monotherapy for active chronic periaortitis. RMD Open 2023; 9:rmdopen-2023-003007. [PMID: 36977534 PMCID: PMC10069601 DOI: 10.1136/rmdopen-2023-003007] [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: 01/16/2023] [Accepted: 03/15/2023] [Indexed: 03/30/2023] Open
Abstract
OBJECTIVES To investigate the effectiveness and safety of TCZ (tocilizumab) monotherapy for chronic periaortitis (CP) patients at acute active stage. METHODS Twelve patients with definite or possible diagnosis of CP were enrolled and received intravenous infusions of TCZ (8 mg/kg) every 4 weeks for at least 3 months. Clinical features, laboratory and imaging findings were recorded at baseline and during the follow-up. The primary endpoint was the rate of partial and complete remission after 3 months TCZ monotherapy and the secondary endpoint was the frequency of treatment related adverse events. RESULTS Three patients (27.3%) achieved partial remission and seven patients (63.6%) obtained complete remission after 3 months TCZ treatment. The total remission rate achieved 90.9%. All patients reported improvement in clinical symptoms. Inflammatory markers such as erythrocyte sedimentation rate and C reactive protein decreased to normal levels after TCZ treatment. Nine patients (81.8%) showed remarkable shrinkage of perivascular mass greater than or equal to 50% on CT. CONCLUSION Our study showed that TCZ monotherapy contributed to remarkable clinical and laboratory improvement in CP patients and could be an alternative treatment option for CP.
Collapse
Affiliation(s)
- Anqi Wang
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| | - Linyi Peng
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| | - Jiaxin Zhou
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| | - Zheng Liu
- Department of Rheumatology, Beijing TongRen Hospital, Capital Medical University, Beijing, China
| | - Hui Lu
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| | - Yu Peng
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| | - Jieqiong Li
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| | - Yunyun Fei
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| | - Wen Zhang
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
- Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China
| |
Collapse
|
|
9
|
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.
Collapse
|
|
10
|
Adam Z, Čermák A, Petrášová H, Řehák Z, Koukalová R, Fojtík Z, Pour L, Boichuk I, Krejčí M, Král Z, Benda P. Successful therapy of retroperitoneal fibrosis due to IgG4-related disease with rituximab, cyclophosphamide and glucocorticoids followed by maintenance therapy wit ritutixmab. VNITRNI LEKARSTVI 2023; 69:4-15. [PMID: 37468330 DOI: 10.36290/vnl.2023.035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 07/21/2023]
Abstract
Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain, and constitutional symptoms such as malaise, fever, and anorexia and weight loss. Pain is frequently referred to the hip, to the groin and to the lateral regions of the leg, with nocturnal exacerbations, and typically does not modify with position. We report a case of 56 year-old male with recurrent lower back pain and lower abdominal pain. Contrast-enhanced computed tomography and was suggestive of retroperitoneal fibrosis and unilateral ureteral occlusion. Histologic examination with immunohistochemical staining for IgG4 demonstrate IgG4-related retroperitoneal fibrosis. Therapy was started with prednison 1 mg/kg, but the tolerance of this dose was poor. Therefore the therapy was switched to combination of rituximab 375 mg/ m2 on day 1, cyclophosphamide 300 mg/m2 mg infusion and dexamethasone 20 mg total dose infusion on day 1 and 15 in 28 days cycle. FDG-PET/CT control in fourth month showed residual accumulation of FDG in retroperitoneal fibrotic mass, and therefore the therapy was prolonged to 8 month. The subjective symptoms of this diseases disappeared in the 8th month. Then the maintenance therapy, administration of rituximab in 6 month interval, was started. The activity of this disease be further evaluated by FDG-PET/CT imagination. Glucocorticoids are considered the cornerstone of therapy. The use of other immunosuppressive agents, including cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil and biological agents such as rituximab, tocilizumab and infliximab and sirolimus have been reported as a valuable option mostly in case reports, cases series and small studies. This agents allowed to reduce cumulative dose of glucocorticoids and its adverse effects. Therefore in our patients we preferred combination of rituximab cyclophosphamide s dexamethasone with lover dose of prednisonem. This combination is preferable for patients who cannot tolerate glucocorticoids or who are likely to suffer from significant glucocorticoids -related toxicity.
Collapse
|
|
11
|
Adhikari R, Banga A, Koritala T, Dasari N, Pattan V. A Rare Co-association of Autoimmune Thyroiditis and Idiopathic Retroperitoneal Fibrosis. Cureus 2022; 14:e30980. [DOI: 10.7759/cureus.30980] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/01/2022] [Indexed: 11/07/2022] Open
|
|
12
|
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.
Collapse
|
|
13
|
Dondi F, Albano D, Giubbini R, Bertagna F. PET in idiopathic retroperitoneal fibrosis. Nucl Med Mol Imaging 2022. [DOI: 10.1016/b978-0-12-822960-6.00059-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
|
|
14
|
Łoń I, Wieliczko M, Lewandowski J, Małyszko J. Retroperitoneal fibrosis is still underdiagnosed entity with poor prognosis. Kidney Blood Press Res 2021; 47:151-162. [PMID: 34915518 DOI: 10.1159/000521423] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2021] [Accepted: 12/08/2021] [Indexed: 11/19/2022] Open
Abstract
BACKGROUND Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles abdominal organs including aorta and ureters. Data on the incidence of this disease are limited. SUMMARY The disease may be idiopathic or secondary to infections, malignancies, drugs or radiotherapy. Idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in aorta and surrounding tissues. Taking into account the dominant symptoms and clinical charac-teristics of patients with periaortitis, two subtypes of disease could be distinguished. Vascular subtype include patients with non-dilated aorta or with inflammatory abdominal aortic aneu-rysm, both with and without involvement of adjacent structures and with numerous risk factors for atherosclerosis. In renoureteral subtype obstructive uropathy manifesting with hydronephro-sis and acute kidney injury is predominant finding. Due to the variety of symptoms, diagnosis of RPF remains challenging, difficult and often delayed. A series of diagnostic tests should be performed, in order to confirm the diagnosis idiopathic RPF. Laboratory work-up include eval-uation of inflammatory indices and immunological studies. A biopsy and histopathological evaluation may be necessary to confirm diagnosis and differentiate the disease. Computed to-mography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) are the modalities of choice for the diagnosis and follow-up of this disease. Management of ureteral obstruction, hydronephrosis, and aortic aneurysms often requires surgical evaluation and treatment. The pharmacological treatment of RPF has been evaluated in a few randomized trials and is mainly based on observational studies. Steroid therapy remains the gold standard of treatment. Key messages: Nowadays multidisciplinary team approach with clinical and diagnos-tic experience in both primary and secondary RPF as well as two major subtypes should be offered. Centers specialized in rare diseases with collaboration with other units and referral sys-tem yield the best possible outcomes.
Collapse
Affiliation(s)
- Izabela Łoń
- Department of Hypertension, Angiology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland
| | - Monika Wieliczko
- Department of Nephrology, Dialysis and Internal Diseases, Medical University of Warsaw, Warsaw, Poland
| | - Jacek Lewandowski
- Department of Hypertension, Angiology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland
| | - Jolanta Małyszko
- Department of Nephrology, Dialysis and Internal Diseases, Medical University of Warsaw, Warsaw, Poland
| |
Collapse
|
|
15
|
Mizushima I, Kawano M. Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges. Int J Nephrol Renovasc Dis 2021; 14:279-289. [PMID: 34349543 PMCID: PMC8328390 DOI: 10.2147/ijnrd.s239160] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2021] [Accepted: 07/23/2021] [Indexed: 01/01/2023] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare disorder consisting of idiopathic and various secondary forms and characterized by chronic inflammatory infiltrates and marked fibrosis in the retroperitoneal space. In idiopathic RPF (IRPF), 35–60% of cases have been reported to be IgG4-related RPF, the retroperitoneal lesions of IgG4-related disease (IgG4-RD). IRPF can frequently lead to renal insufficiency mediated by urinary tract obstruction and hydronephrosis irrespective of being IgG4-related or not. Clinical pictures, laboratory and imaging findings, and location of the urinary tract obstruction are generally similar in IgG4-related and non-IgG4-related IRPF although multiple organ involvement and serum IgG4 elevation may be characteristic of the IgG4-related forms. Periaortic/periarterial lesions are the most frequent cause of renal insufficiency. Although the response to glucocorticoids is generally good, relapse does occur in a considerable proportion of patients, and may require an additional immunosuppressive agent and/or urological intervention in cases with multiple relapses or refractory obstructive uropathy. In general, the prognosis of patients with IRPF is good, but careful attention needs to be paid to chronic kidney disease as a major complication and rupture of the affected aorta/artery as a life-threatening one. Further studies are necessary to better understand the pathogenesis of the disease and to establish the optimal diagnostic and therapeutic strategies for it.
Collapse
Affiliation(s)
- Ichiro Mizushima
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Mitsuhiro Kawano
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| |
Collapse
|
|
16
|
Altshuler E. Atypical Presentation of Idiopathic Retroperitoneal Fibrosis Effectively Treated With Colchicine After Lymphoma Misdiagnosis. Cureus 2021; 13:e14756. [PMID: 34094726 PMCID: PMC8169009 DOI: 10.7759/cureus.14756] [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: 11/05/2022] Open
Abstract
Idiopathic retroperitoneal fibrosis (RPF) is a rare disease characterized by a fibro-inflammatory mass encasing the abdominal aorta. We report a case of a 43-year-old man with an unusual presentation of RPF who was initially misdiagnosed with lymphoma. Our patient presented with constipation and did not have common findings such as ureteral displacement or renal impairment. Our patient had a complicated disease course complicated by multiple treatment failures and pulmonary embolism. We discuss the patient's first 100 months of treatment, which included the use of prednisone, mycophenolate, tamoxifen, methotrexate, azathioprine, and, now, colchicine. Our case demonstrates that physicians should maintain an index of suspicion for RPF in patients with a homogenously attenuated mass encasing the anterior aorta. It also serves as one example in which RPF appeared to be responsive to colchicine.
Collapse
Affiliation(s)
- Ellery Altshuler
- Internal Medicine, University of Florida College of Medicine, Gainesville, USA
| |
Collapse
|
|
17
|
Characteristics of Adult Patients with Idiopathic Retroperitoneal Fibrosis and Assessment of Risk of Relapse at Diagnosis. J Clin Med 2021; 10:jcm10071380. [PMID: 33808093 PMCID: PMC8038021 DOI: 10.3390/jcm10071380] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2021] [Revised: 03/20/2021] [Accepted: 03/25/2021] [Indexed: 02/07/2023] Open
Abstract
OBJECTIVES To compare adult patients' characteristics suffering from idiopathic retroperitoneal fibrosis between "relapse-free" and relapsing patients at the diagnosis and identify factors associated with relapse at initial presentation. METHODS We conducted a retrospective multicentric study in four hospitals in Eastern France, from 1993 to 2020, of adult patients suffering from idiopathic retroperitoneal fibrosis. We analyzed clinical, biological, and radiological features at diagnosis and during a forty-month follow-up. RESULTS Of 47 patients suffering from retroperitoneal fibrosis, 21 patients had idiopathic retroperitoneal fibrosis. Among them, 13 experienced one or more relapses during follow-up. At diagnosis, clinical characteristics, relevant comorbidities, biological and radiological features were similar between groups. Smoking cessation seems associated with decreased relapse risk (p: 0.0624). A total of 8 patients developed chronic renal failure during follow-up. Ureteral infiltration at diagnosis was associated with evolution to chronic renal failure (p: 0.0091). CONCLUSION No clinical, biological, or radiological features could predict relapse at retroperitoneal fibrosis diagnosis, but smoking cessation may prevent relapse.
Collapse
|
|
18
|
Pacella JC, Niwattisaiwong S, Newman D. IgG4-Related Retroperitoneal Fibrosis: A Rare Association With Riedel's Thyroiditis. Cureus 2021; 13:e13997. [PMID: 33884241 PMCID: PMC8054938 DOI: 10.7759/cureus.13997] [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] [Indexed: 01/13/2023] Open
Abstract
Idiopathic retroperitoneal fibrosis is a rare fibro-inflammatory disease that can be associated with other IgG4-related diseases (IgG4-RDs). It is exceedingly uncommon to encounter this condition in a patient with Riedel's thyroiditis (RT), another disease in the IgG4-RD family. We present the case of a 53-year-old man with a history of RT who presented for severe localized lower abdominal and suprapubic pain due to obstructive uropathy from extensive retroperitoneal fibrosis. The biopsy of the mass demonstrated fibro-inflammatory tissue, and its immunohistochemistry was notable for IgG4-positive plasma cells. This case highlights the challenge associated with the diagnosis and management of this rare manifestation of IgG4-RD. In a patient with a history of any form of IgG4-RDs, providers should be vigilant for any signs or symptoms that suggest the development of fibrosis in other organs.
Collapse
Affiliation(s)
- Jonathan C Pacella
- Pediatrics, University of North Dakota School of Medicine and Health Sciences, Fargo, USA
| | | | - David Newman
- Endocrinology, Diabetes and Metabolism, Sanford Health, Fargo, USA
| |
Collapse
|
|
19
|
Wang K, Wang Z, Zeng Q, Zhu L, Gao J, Wang Z, Zhang S, Yang F, Shen D, Wang Y, Liu Y. Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis. PLoS One 2021; 16:e0245601. [PMID: 33600452 PMCID: PMC7891782 DOI: 10.1371/journal.pone.0245601] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2020] [Accepted: 01/04/2021] [Indexed: 12/23/2022] Open
Abstract
Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People’s Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.
Collapse
Affiliation(s)
- Kunkun Wang
- Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, China
- Department of Rheumatology and Immunology, Tengzhou Central People’s Hospital, Tengzhou, China
| | - Zhenfan Wang
- Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, China
| | - Qiaozhu Zeng
- Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, China
| | - Lijuan Zhu
- Department of Rheumatology and Immunology, Zhengzhou Central Hospital Affiliated to Zhengzhou University, Zhengzhou, China
| | - Jingyuan Gao
- Department of Geriatrics, Affiliated Hospital of North China University of Technology, Tangshan, China
| | - Ziqiao Wang
- Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, China
| | - Shanshan Zhang
- Department of Ultrasound, Peking University People’s Hospital, Beijing, China
| | - Fei Yang
- Department of Pathology, Peking University People’s Hospital, Beijing, China
| | - Danhua Shen
- Department of Pathology, Peking University People’s Hospital, Beijing, China
| | - Yi Wang
- Department of Radiology, Peking University People’s Hospital, Beijing, China
| | - Yanying Liu
- Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, China
- * E-mail:
| |
Collapse
|
|
20
|
Into Clinical Practice: Diagnosis and Therapy of Retroperitoneal Fibrosis. Curr Rheumatol Rep 2021; 23:18. [PMID: 33569638 DOI: 10.1007/s11926-020-00966-9] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/15/2020] [Indexed: 02/07/2023]
Abstract
PURPOSE OF THE REVIEW We aim to review the most relevant diagnostic features and treatment options of retroperitoneal fibrosis, in order to provide a useful guide for clinical practice. RECENT FINDINGS The recent literature highlights the role of imaging studies such as computed tomography, magnetic resonance imaging and positron emission tomography as useful tools for the diagnosis of retroperitoneal fibrosis, with retroperitoneal biopsy being reserved to atypical cases. The treatment approach is mainly conservative and is based on the use of medical therapies plus urological interventions. Medical therapies essentially comprise glucocorticoids and immunosuppressants-either traditional or biological agents such as rituximab. Surgical ureterolysis is only left for refractory cases. Recent findings in retroperitoneal fibrosis highlight the possibility of a non-invasive diagnostic approach and a conservative treatment strategy.
Collapse
|
|
21
|
Azizi M, Zajjari Y, Rafik H, El Kabbaj D. Retroperitoneal fibrosis in the military hospital of Morocco. SAUDI JOURNAL OF KIDNEY DISEASES AND TRANSPLANTATION 2021; 31:169-175. [PMID: 32129210 DOI: 10.4103/1319-2442.279937] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare disease. It is characterized by the presence of fibro-inflammatory tissue involving retroperitoneal structures. The usual mode of presentation of this disease is with lumbar pain, kidney failure, and a biological inflammatory syndrome. The aim of our study is to describe the diagnostic, etiologic, therapeutic aspects and outcomes of RPF in a nephrology unit in Morocco. Twelve cases of RPF were included in our study. The mean age was 57 ± 10 years (32.70). Nine patients were male and three were female. Symptoms were highly variable, dominated by pain that was present in all patients. Venous compressive signs were described in four patients (33.3%), anuria in one patient (8.3%), and hematuria in two patients (16.6%). Laboratory examinations found an inflammatory syndrome in all patients and renal failure in nine patients (75%), with a mean serum creatinine at 35 mg/L ± 8.5. Diagnosis was suspected on the ultrasound data and confirmed by computed tomography or magnetic resonance imaging. RPF was idiopathic in nine patients (75%). It was secondary to aortic aneurysm in one patient (8.3%), Riedel's thyroiditis in one patient (8.3%), and drug induced in another patient (8.3%). All patients received surgical treatment along with corticosteroids. At six months, remission was achieved in nine patients, whereas three others had steroid resistance. These patients were treated by mycophenolate mofetil (MMF) at a dose of 2 g/day; two of them had intestinal intolerance to MMF and thus were treated by tamoxifen at a dose of 40 mg/day. At 24 months, they stabilized their renal function with incomplete regression of the fibrotic plate. No cases of recurrence were observed during the study period.
Collapse
Affiliation(s)
- Mounia Azizi
- Department of Nephrology-Dialysis, Military Hospital Mohammed V, Rabat, Morocco
| | - Yassir Zajjari
- Department of Nephrology-Dialysis, Military Hospital Mohammed V, Rabat, Morocco
| | - Hicham Rafik
- Department of Nephrology-Dialysis, Military Hospital Mohammed V, Rabat, Morocco
| | - Driss El Kabbaj
- Department of Nephrology-Dialysis, Military Hospital Mohammed V, Rabat, Morocco
| |
Collapse
|
|
22
|
Raglianti V, Rossi GM, Vaglio A. Idiopathic retroperitoneal fibrosis: an update for nephrologists. Nephrol Dial Transplant 2020; 36:1773-1781. [PMID: 33005943 DOI: 10.1093/ndt/gfaa083] [Citation(s) in RCA: 28] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2019] [Indexed: 12/17/2022] Open
Abstract
Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications of IRF, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. IRF may be isolated or develop in association with autoimmune diseases (e.g. Hashimoto's thyroiditis and psoriasis) and other fibro-inflammatory disorders (often within the spectrum of immunoglobulin G4-related disease), which suggests that it should be considered as a potentially systemic condition. IRF is an immune-mediated disease: genetic variants (e.g. human leukocyte antigen (HLA)-DRB1*03) and environmental agents (mainly exposure to asbestos and smoking) are strongly associated with an increased risk of developing the disease, while a complex network of chemokines (e.g. CXCL12 and C-C moti chemokine 11 (CCL11)) and cytokines [e.g. interleukin (IL)-6, IL-12 and IL-13] is likely to orchestrate the inflammatory response and simultaneously promote fibrosis. Glucocorticoids, alone or in combination with traditional immunosuppressants such as methotrexate and mycophenolate mofetil, are usually efficacious and promptly induce disease remission; however, up to 50% of patients relapse, thus requiring repeat immunosuppressive courses. Biologic drugs, namely rituximab, are being explored for the treatment of IRF. In addition to medical therapies, interventional procedures (mainly ureteral stenting) are required to relieve ureteral obstruction, whereas surgical ureterolysis is generally reserved to refractory cases. If appropriately treated, then the overall and renal prognosis of IRF are good, with <5% patients developing end-stage renal disease.
Collapse
Affiliation(s)
- Valentina Raglianti
- Deptartment of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze, Italy
| | - Giovanni M Rossi
- Nephrology Unit, Parma University Hospital, Parma, Italy.,Department of Medicine and Surgery, University of Parma, Parma, Italy
| | - Augusto Vaglio
- Deptartment of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze, Italy.,Nephrology and Dialysis Unit, Meyer Children's Hospital, Firenze, Italy
| |
Collapse
|
|
23
|
Roussel E, Callemeyn J, Van Moerkercke W. Standardized approach to idiopathic retroperitoneal fibrosis: a comprehensive review of the literature. Acta Clin Belg 2020; 75:239-244. [PMID: 31035909 DOI: 10.1080/17843286.2019.1609152] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND Idiopathic retroperitoneal fibrosis (iRPF) is a rare fibro-inflammatory disease, characterized by inflammation of the abdominal aorta and its surrounding structures. The exact pathophysiology remains unclear. Diagnosis is often troublesome due to the non-specific and highly variable clinical presentation. Standardized treatment protocols are lacking. OBJECTIVE This article presents a review on iRPF, addressing clinical and diagnostic modalities as well as its pathophysiology and the possible inclusion within the IgG4-related disease (IgG4-RD) spectrum. Finally, a diagnostic-therapeutic algorithm for a standardized approach to iRPF is proposed. METHODS The PubMed Internet database was searched. Articles were selected based on the relevance of abstract, article type and impact of the journal. RESULTS iRPF and IgG4-RD share a common autoimmune aetiology. Diagnostics are multimodal and based on imaging. Ruling out malignancy should be of primary concern. Complications are mostly of renal or vascular origin due to compression of retroperitoneal structures. Corticosteroids remain the first-line treatment regimen and are mostly successful, but evidence supporting alternative immunosuppressive and anti-inflammatory treatments is growing. Long-term therapy, as well as follow-up, is paramount in this chronic and often relapsing disease.
Collapse
Affiliation(s)
- Eduard Roussel
- Department of Urology, University Hospitals Leuven, Leuven, Belgium
| | - Jasper Callemeyn
- Department of Internal Medicine, Nephrology and Renal Transplantation Research Group, Leuven, Belgium
| | - Wouter Van Moerkercke
- Department of Gastroenterology, University Hospitals Leuven, Leuven, Belgium
- Department of Gastroenterology, AZ Groeninge, Department of Gastroenterology, Kortrijk, Belgium
| |
Collapse
|
|
24
|
Ruan GJ, Goyal G, Go RS. 43-Year-Old Man With Polyuria and Bone Pain. Mayo Clin Proc 2020; 95:e13-e18. [PMID: 32029098 DOI: 10.1016/j.mayocp.2019.07.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/01/2019] [Revised: 07/20/2019] [Accepted: 07/29/2019] [Indexed: 10/25/2022]
Affiliation(s)
- Gordon J Ruan
- Resident in Internal Medicine, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Gaurav Goyal
- Fellow in Hematology, Mayo Clinic School of Graduate Medical Education, Rochester, MN
| | - Ronald S Go
- Advisor to resident and fellow and Consultant in Hematology, Mayo Clinic, Rochester, MN.
| |
Collapse
|
|
25
|
Vashi B, Khosroshahi A. IgG4-Related Disease with Emphasis on Its Gastrointestinal Manifestation. Gastroenterol Clin North Am 2019; 48:291-305. [PMID: 31046976 DOI: 10.1016/j.gtc.2019.02.008] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
IgG4-related disease is an immune-mediated fibroinflammatory condition with a diverse spectrum of organ involvement, commonly in the pancreas and bile ducts among other organs such as salivary and lacrimal glands. Classic histopathologic findings are the gold standard for confirmation of diagnosis, although diagnosis remains challenging, as biomarkers to date are neither sufficient nor necessary. Glucocorticoids are the most effective initial treatment, generally having a dramatic response, although limited clinical evidence exists regarding effective maintenance therapy. This review summarizes key GI manifestations of this condition for the practicing gastroenterologist and addresses the pathology, disease mechanism, and current therapeutic recommendations.
Collapse
Affiliation(s)
- Bijal Vashi
- Department of Medicine, Emory University, 200 Whitehead Building, 615 Michael Street, Atlanta, GA 30322, USA
| | - Arezou Khosroshahi
- Department of Medicine, Emory University, 244 Whitehead Building, 615 Michael Street, Atlanta, GA 30322, USA.
| |
Collapse
|
|
26
|
Morin G, Mageau A, Benali K, Bertinchamp R, Piekarski E, Raimbourg Q, Alexandra JF, Goulenok T, van Gysel D, Papo T, Sacre K. Persistent FDG/PET CT uptake in idiopathic retroperitoneal fibrosis helps identifying patients at a higher risk for relapse. Eur J Intern Med 2019; 62:67-71. [PMID: 30711361 DOI: 10.1016/j.ejim.2019.01.019] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/26/2018] [Revised: 01/10/2019] [Accepted: 01/30/2019] [Indexed: 12/20/2022]
Abstract
BACKGROUND The aim of this study was to evaluate the prognostic value of persistent retroperitoneal fibrosis FDG uptake using FDG/PET CT in patients with idiopathic retroperitoneal fibrosis (IRF). METHODS In this monocentric retrospective cohort study, all patients admitted for IRF from January 2009 to December 2017 underwent a FDG/PET CT at diagnosis and during follow up. Metabolic activity of IRF was assessed by retroperitoneal fibrosis FDG uptake measured as maximal standardized uptake value (SUVmax). The primary outcome was IRF relapse rate during follow-up. RESULTS 23 consecutive patients (54.7 [36.9-89] years, 73.9% of men) diagnosed with IRF had FDG/PET CT imaging performed at diagnosis, 3.1 [1-8.7] months (i.e 1st evaluation) and 10.4 [4.9-17.5] months (i.e 2nd evaluation) after diagnosis. High FDG retroperitoneal fibrosis uptake was present in all patients at diagnosis (SUVmax 6.5 [3.8-11.9]) and persisted in 16 (69.6%; SUVmax 3.65 [2.1-5.4]) and 12 (52.2%; SUVmax 3.75 [2.7-7.8]) patients, at 1st and 2nd evaluation respectively. All but one patient had received steroids at IRF diagnosis and 21 (91.3%) were in complete remission at both 1st and 2nd evaluation. During a median follow-up period of 38.7 [3-106.9] months, 6 (26.1%) patients suffered IRF relapse that occurred 15.7 [9.2-42.8] months after diagnosis. Multivariate analysis showed that only persistent retroperitoneal fibrosis FDG uptake at 2nd evaluation was associated with IRF relapse (p = .046). CONCLUSIONS In IRF, persistent retroperitoneal fibrosis FDG uptake during follow up is associated with clinical outcome. FDG/PET CT may help to better stratify the risk of relapse and target therapy in IRF.
Collapse
Affiliation(s)
- Gabriel Morin
- Département de Médecine Interne, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Arthur Mageau
- Département de Médecine Interne, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Khadija Benali
- Département de Médecine Nucléaire, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Remi Bertinchamp
- Département de Médecine Interne, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Eve Piekarski
- Département de Médecine Nucléaire, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Quentin Raimbourg
- Département de Nephrologie, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Jean-Francois Alexandra
- Département de Médecine Interne, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Tiphaine Goulenok
- Département de Médecine Interne, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Damien van Gysel
- Département d'Information Médicale, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France
| | - Thomas Papo
- Département de Médecine Interne, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France; Département Hospitalo-Universitaire FIRE (Fibrosis, Inflammation and Remodelling in Renal and Respiratory Diseases), Paris, France; INSERM U1149, Paris, France
| | - Karim Sacre
- Département de Médecine Interne, Hôpital Bichat, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, Paris, France; Département Hospitalo-Universitaire FIRE (Fibrosis, Inflammation and Remodelling in Renal and Respiratory Diseases), Paris, France; INSERM U1149, Paris, France.
| |
Collapse
|
|
27
|
Acute Kidney Failure in a Young African American Male. Case Rep Nephrol 2019; 2019:2591560. [PMID: 30911422 PMCID: PMC6398077 DOI: 10.1155/2019/2591560] [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: 12/11/2018] [Accepted: 02/03/2019] [Indexed: 11/29/2022] Open
Abstract
Retroperitoneal fibrosis (RPF) is a condition characterized by chronic inflammatory and fibrotic changes in the retroperitoneum that can lead to serious complications including kidney failure, mesenteric and limb ischemia, and deep venous thrombosis among others. Affected individuals may present with nonspecific symptomology that would require a high clinical index of suspicion for prompt diagnosis. We herein discuss a case of a young African-American man with recurrent deep venous thrombosis who presents with a 4-week history of constant aching pain of abdomen and back and kidney failure. Initial noncontrast computed tomogram (CT) only revealed mild bilateral hydroureteronephrosis with inflammatory changes but without obvious mass or lymphadenopathy. At the insistence of the renal consulting team to rule out RPF, a CT-urogram was performed which revealed an infiltrative mass encasing the aorta, inferior vena cava, and common iliac vessels. Laparoscopic biopsy revealed dense fibroadipose tissue, lymphocytic aggregates, focal scattered IgG4-positive plasma cells, and fibrin deposition. Patient underwent bilateral nephrostomy placement and empirical corticosteroid therapy with resolution of kidney failure. Our case illustrates a classic presentation of RPF with relatively benign findings on noncontrast CT that could have been missed if clinicians did not keep a high index of suspicion for the condition.
Collapse
|
|
28
|
Sharma S, Paniagua-Morales JA, Cordoba GG, Then EO, Sharma S. Idiopathic Retroperitoneal Fibrosis Presenting as Low Back Pain. Cureus 2019; 11:e4075. [PMID: 31019853 PMCID: PMC6464980 DOI: 10.7759/cureus.4075] [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: 11/17/2022] Open
Abstract
Retroperitoneal fibrosis is a rare condition characterized by the presence of fibrous inflammatory tissue in the retroperitoneal structures, such as the infrarenal great vessels and ureters. We are reporting an atypical case of an alcoholic who presented with chronic back pain and abnormal liver function tests. Abdominal imaging revealed an incidental, ill-defined, abnormal soft tissue mass in the left pelvis and mild to moderate left hydroureteronephrosis. Interventional radiology (IR)-guided core biopsy reported cores of dense fibrous tissue with extensive lymphoplasmacytic infiltrates, consistent with idiopathic retroperitoneal fibrosis (IRF). The patient had a left ureteral stent placed and, subsequently, had robotic surgery for ureteral reimplantation.
Collapse
Affiliation(s)
- Shorabh Sharma
- Internal Medicine, St. Barnabas Hospital Health System, Bronx, USA
| | | | | | - Eric O Then
- Internal Medicine, St. Barnabas Hospital Health System, Bronx, USA
| | - Sapna Sharma
- Internal Medicine, Mahatma Gandhi Mission Institute of Health Sciences, Navi Mumbai, USA
| |
Collapse
|
|
29
|
Subramani AV, Lockwood GM, Jetton JG, Dhungana N, Sato TS. Pediatric idiopathic retroperitoneal fibrosis. Radiol Case Rep 2019; 14:459-462. [PMID: 30740191 PMCID: PMC6357545 DOI: 10.1016/j.radcr.2019.01.006] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2018] [Revised: 01/09/2019] [Accepted: 01/13/2019] [Indexed: 10/29/2022] Open
Abstract
Retroperitoneal fibrosis (RPF) is a very rare disease that is even more rare in the pediatric population. Even less common are idiopathic pediatric cases of retroperitoneal fibrosis, with a majority of reported pediatric retroperitoneal fibrosis cases being associated with secondary etiologies. We present an 11-year-old Caucasian female that was diagnosed with idiopathic retroperitoneal fibrosis using magnetic resonance imaging (MRI) to work-up severe bilateral hydronephrosis that was identified with retroperitoneal ultrasound. Given the uncommon nature of this serious condition, we present this case to illustrate the importance for physicians to include retroperitoneal fibrosis in the differential diagnosis of a pediatric patient presenting with obstructive urinary findings and understand the utility of using MRI to diagnosis and monitor this disease.
Collapse
Affiliation(s)
- Ashwin V Subramani
- Roy J. and Lucille A. Carver College of Medicine, 01334C PFP, 200 Hawkins Drive, Iowa City, IA 52242, US
| | - Gina M Lockwood
- University of Iowa Hospitals and Clinics, Department of Urology, 3240 RCP, 200 Hawkins Drive, Iowa City, IA 52242, US
| | - Jennifer G Jetton
- University of Iowa Hospitals and Clinics, Department of Pediatrics, 2027 BT, 200 Hawkins Drive, Iowa City, IA 52242, US
| | - Neha Dhungana
- University of Iowa Hospitals and Clinics, Department of Pathology, 5223 RCP, 200 Hawkins Drive, Iowa City, IA 52242, US
| | - T Shawn Sato
- University of Iowa Hospitals and Clinics, Department of Radiology, 3889 JPP, 200 Hawkins Drive, Iowa City, IA 52242, US
| |
Collapse
|
|
30
|
Mzabi A, Kéchrid N, Alaya Z, Rezgui A, Ben Fredj F, Bouajina E, Laouani C. [Retroperitoneal fibrosis in adults: Diagnostic approach based on a retrospective multicenter study on 32 cases]. Prog Urol 2018; 29:76-85. [PMID: 30579757 DOI: 10.1016/j.purol.2018.10.006] [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: 05/17/2017] [Revised: 06/29/2018] [Accepted: 10/31/2018] [Indexed: 10/27/2022]
Abstract
INTRODUCTION Retroperitoneal fibrosis is a rare disease, typically with an insidious and various clinical course. The peak incidence is seen in patients 40 to 60 years of age and mostly in man. The characteristic finding in this disease is a periaortic fibrous mass that often surrounds the ureters. The diagnostic approach remains uncodified. We aimed to determine the different clinical, radiological and biological aspects of retroperitoneal fibrosis. PATIENTS AND METHODS Retrospective multicenter study of 32 retroperitoneal fibrosis cases hospitalized between 1999 and 2014 in the Internal Medicine Department and Urology Department in the university hospital center Sahloul Sousse. RESULTS There were 24 men and 8 women with a mean age of 58 years. The lumbar pain is the most common clinical signs (53.1%). An inflammatory syndrome and renal failure were the most common biological signs. The diagnosis was suspected on data from the abdominal ultrasound and confirmed by pelvic CT scan that showed a periaortic fibrous mass that often surrounds the ureters. Histological analysis of a surgical biopsy specimen was performed in only eight cases. CONCLUSION The most common mode of presentation of retroperitoneal fibrosis remains lumbar pain with renal failure and a high sedimentation rate. Although abdominal ultrasound may contribute to the general evaluation of patients with retroperitoneal fibrosis, CT-scanner is the preferred imaging method. The imaging capability of magnetic resonance and the TEP-scan may facilitate assessment of disease extent. LEVEL OF EVIDENCE 4.
Collapse
Affiliation(s)
- A Mzabi
- Service de médecine interne, hôpital Sahloul, Sousse, Tunisie
| | - N Kéchrid
- Service de médecine interne, hôpital Sahloul, Sousse, Tunisie
| | - Z Alaya
- Service de rhumatologie, hôpital Farhat-Hached, avenue Ibn El Jazzar, 4000 Sousse, Tunisie.
| | - A Rezgui
- Service de médecine interne, hôpital Sahloul, Sousse, Tunisie
| | - F Ben Fredj
- Service de médecine interne, hôpital Sahloul, Sousse, Tunisie
| | - E Bouajina
- Service de rhumatologie, hôpital Farhat-Hached, avenue Ibn El Jazzar, 4000 Sousse, Tunisie
| | - C Laouani
- Service de médecine interne, hôpital Sahloul, Sousse, Tunisie
| |
Collapse
|
|
31
|
|
|
32
|
Abstract
Retroperitoneal fibrosis (RPF) refers to a fibro-inflammatory lesion in the retroperitoneum, often anterolateral to the aorta. Most cases are due to IgG4-related disease (IgG4-RD) or are idiopathic. RPF can lead to severe morbidity. Treatment strategies remain poorly-defined. We evaluated the efficacy and safety of rituximab (RTX) for idiopathic or IgG4-related RPF.We retrospectively reviewed the records of patients who had RPF treated with RTX. Treatment response was determined by assessing changes in both clinical features, including symptoms and laboratory measurements, as well as in the radiographic dimensions of the lesion.Twenty-six patients with IgG4-related (n = 19) or idiopathic RPF (n = 7) were identified. Patients without histopathological evidence of IgG4-RD on either retroperitoneal biopsies or sampling of extra-retroperitoneal organs were considered to have idiopathic RPF. Of the 26 patients, 19 (73%) received RTX without additional glucocorticoids. All 19 patients who presented with pain reported symptomatic improvement following RTX. Among 25 patients with follow-up imaging, 22 (88%) had radiologic improvement. Among 10 patients with ureteral stents and/or percutaneous nephrostomy tubes, 4 (40%) underwent successful stent or tube removal. Responses to treatment were similar among those treated with RTX monotherapy and those treated with RTX and glucocorticoids. RTX was generally well tolerated, but 3 (12%) patients experienced severe infections.In this study, RTX for RPF led to resolution of symptoms in all patients and radiographic improvement in the majority. Prospective studies of RTX for RPF are indicated.
Collapse
Affiliation(s)
| | - Zachary Wallace
- Department of Medicine
- Division of Rheumatology, Allergy, and Immunology
| | - Cory Perugino
- Department of Medicine
- Division of Rheumatology, Allergy, and Immunology
| | - Amita Sharma
- Department of Radiology, Massachusetts General Hospital/Harvard Medical School, Boston, MA
| | - John H. Stone
- Department of Medicine
- Division of Rheumatology, Allergy, and Immunology
| |
Collapse
|
|
33
|
Zhao J, Li J, Zhang Z. Long-term outcomes and predictors of a large cohort of idiopathic retroperitoneal fibrosis patients: a retrospective study. Scand J Rheumatol 2018; 48:239-245. [PMID: 30270709 DOI: 10.1080/03009742.2018.1497700] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022]
Abstract
OBJECTIVES Idiopathic retroperitoneal fibrosis (iRPF) is an uncommon disease with inflammatory features. Most patients have good prognosis but some may relapse or even progress to chronic renal failure. The aims of the study were to investigate the prognosis of a large cohort of iRPF patients and explore the risk factors for poor outcomes. METHODS All patients with a definite diagnosis of iRPF in Peking University First Hospital between 1 January 2003 and 31 December 2016 were enrolled. Their clinical and laboratory data at diagnosis and subsequent follow-up visits were collected. The endpoint of follow-up was defined as disease relapse or the last follow-up. RESULTS In total, 155 iRPF patients (45 females, 110 males) with a mean ± sd age of 55.1 ± 12 years at diagnosis were enrolled. The median duration of follow-up was 45.3 (0.1-169.2) months. During the whole follow-up, 27/153 (17.6%) patients encountered at least one relapse. Higher baseline erythrocyte sedimentation rate (ESR) was associated with a higher risk of relapse [p = 0.021, odds ratio (OR) = 1.016, 95% confidence interval (CI) 1.002-1.029]. A longer course of glucocorticoid treatment was associated with a lower risk of relapse (p = 0.030, OR = 0.948, 95% CI 0.904-0.995). Patients with a permanent presence of hydronephrosis were more likely to develop renal atrophy (33/154 vs 19/51, χ2 = 5.069, p = 0.024). CONCLUSIONS Higher baseline ESR and a shorter course of glucocorticoid treatment seemed to be predictors of relapse in iRPF patients. Prompt release of hydronephrosis to prevent kidney atrophy is very important for preserving renal function and improving the prognosis.
Collapse
Affiliation(s)
- J Zhao
- a Department of Rheumatology and Clinical Immunology , Peking University First Hospital , Beijing , China
| | - J Li
- a Department of Rheumatology and Clinical Immunology , Peking University First Hospital , Beijing , China
| | - Z Zhang
- a Department of Rheumatology and Clinical Immunology , Peking University First Hospital , Beijing , China
| |
Collapse
|
|
34
|
Fibrose rétropéritonéale idiopathique : étude descriptive rétrospective de 19 patients et évaluation du risque de rechute de la maladie. Rev Med Interne 2018. [DOI: 10.1016/j.revmed.2018.03.315] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
|
|
35
|
Simopoulou T, Klimopoulos S, Sampaziotis D, Tzortziotis A, Bogdanos D, Sakkas LI. Inferior vena cava thrombosis as the initial presentation of IgG4-related retroperitoneal fibrosis: Case report and literature review. JOURNAL OF SCLERODERMA AND RELATED DISORDERS 2018; 3:NP1-NP6. [DOI: 10.1177/2397198318764793] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2017] [Accepted: 02/21/2018] [Indexed: 12/24/2022]
Abstract
IgG4-related disease is an immune-mediated deposition of fibro-inflammatory tissue with IgG4-positive plasma cells and dense fibrosis in a single or multiple organs. It often raises concern for malignancy requiring biopsy for diagnosis. Presentation may vary according to organ involved. IgG4-related retroperitoneal fibrosis accounts for two-thirds of the previously considered idiopathic retroperitoneal fibrosis cases. In IgG4-related retroperitoneal fibrosis, computed tomography scan or magnetic resonance imaging shows periaortic soft tissue that extends from below the kidneys to iliac arteries and entrap ureters causing hydronephrosis and renal failure. We present a rare case of IgG4-related retroperitoneal fibrosis presenting with leg swelling and pain due to inferior vena cava compression and thrombosis, and we review current concepts on disease pathogenesis, diagnosis and treatment.
Collapse
Affiliation(s)
- Theodora Simopoulou
- Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece
| | | | | | - Apostolos Tzortziotis
- Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece
| | - Dimitrios Bogdanos
- Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece
| | - Lazaros I Sakkas
- Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece
| |
Collapse
|
|
36
|
Liao S, Wang Y, Li K, Zhu J, Zhang J, Huang F. Idiopathic retroperitoneal fibrosis: a cross-sectional study of 142 Chinese patients. Scand J Rheumatol 2017; 47:198-205. [PMID: 29219671 DOI: 10.1080/03009742.2017.1363280] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Affiliation(s)
- S Liao
- Department of Rheumatology, PLA General Hospital, Beijing, PR China
| | - Y Wang
- Department of Rheumatology, PLA General Hospital, Beijing, PR China
| | - K Li
- Department of Rheumatology, PLA General Hospital, Beijing, PR China
| | - J Zhu
- Department of Rheumatology, PLA General Hospital, Beijing, PR China
| | - J Zhang
- Department of Rheumatology, PLA General Hospital, Beijing, PR China
| | - F Huang
- Department of Rheumatology, PLA General Hospital, Beijing, PR China
| |
Collapse
|
|
37
|
Zhang W, Xue F, Wang C, Shao L. Clinical features and prognostic factors of ten patients with renal failure caused by IgG4-related retroperitoneal fibrosis. Oncotarget 2017; 9:2858-2865. [PMID: 29416818 PMCID: PMC5788686 DOI: 10.18632/oncotarget.23103] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2017] [Accepted: 11/15/2017] [Indexed: 02/06/2023] Open
Abstract
IgG4-related retroperitoneal fibrosis (IgG4-RPF) is a newly recognized entity which often mimics cancer. We aimed to investigate the clinical features and the causes of renal failure, as well as to explore the factors affecting the prognosis of renal function by analysis of the clinical data of patients with IgG4-RPF. We reviewed clinical features of 10 patients with renal failure caused by IgG4-RPF, which was confirmed by pathology review and clinic-pathologic correlations. All patients were male, and the mean age at onset was 64.2 ± 10.0 years. Five patients were revealed with acute renal failure (ARF), while the other five ones with ARF on chronic kidney disease (CKD) (A on C) at diagnosis. Initial favorable responses obtained in 90% of the patients who underwent steroid therapy. The serum creatinine (SCr) level returned to normal in six patients including five with ARF and one with A on C, while those of the rest four patients with A on C restored to baseline levels (GFR remained below 60 ml/min/1.73 m2 however) after therapy. These four unrecovered patients had a history of CKD, a longer period of persistently elevated SCr, a thinner total renal parenchyma thickness, and continuous elevated serum IgG4 levels after steroid therapy, compared with those recovered patients (P < 0.05). We concluded that recovery and long-term prognosis of the disease were primarily associated with timely diagnosis and proper treatment.
Collapse
Affiliation(s)
- Wei Zhang
- Department of Nephrology, The Affiliated Hospital of Qingdao University, Qingdao 266003, China
| | - Feng Xue
- Department of Anesthesiology, The Affiliated Hospital of Qingdao University, Qingdao 266003, China
| | - Cui Wang
- Department of Nephrology, The Affiliated Hospital of Qingdao University, Qingdao 266003, China
| | - Leping Shao
- Department of Nephrology, The Affiliated Hospital of Qingdao University, Qingdao 266003, China
| |
Collapse
|
|
38
|
Sanchez-Alvarez C, Bowman AW, Menke DM, Wang B. IgG4 Isolated Retroperitoneal Fibrosis and Aneurysmal Periaortitis. Am J Med 2017; 130:e521-e524. [PMID: 28882661 DOI: 10.1016/j.amjmed.2017.08.017] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2017] [Revised: 08/16/2017] [Accepted: 08/23/2017] [Indexed: 12/30/2022]
Affiliation(s)
| | | | - David M Menke
- Department of Pathology, Mayo Clinic, Jacksonville, Fla
| | - Benjamin Wang
- Department of Rheumatology, Mayo Clinic, Jacksonville, Fla
| |
Collapse
|
|
39
|
Fernando A, Pattison J, Horsfield C, D'Cruz D, Cook G, O'Brien T. [ 18F]-Fluorodeoxyglucose Positron Emission Tomography in the Diagnosis, Treatment Stratification, and Monitoring of Patients with Retroperitoneal Fibrosis: A Prospective Clinical Study. Eur Urol 2017; 71:926-933. [PMID: 27876167 DOI: 10.1016/j.eururo.2016.10.046] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2016] [Accepted: 10/26/2016] [Indexed: 11/28/2022]
Abstract
BACKGROUND The ability to distinguish malignant from benign retroperitoneal fibrosis (RPF) and to select patients who are likely to respond to steroid treatment using a noninvasive test would be a major step forward in the management of patients with RPF. OBJECTIVE To prospectively evaluate the potential of [18F]-fluorodeoxyglucose positron emission tomography (FDG-PET) to improve clinical decision-making and management of RPF. DESIGN, SETTING, AND PARTICIPANTS A total of 122 RPF patients were assessed and managed by a multidisciplinary RPF service between January 2012 and December 2015. Of these, 78 patients underwent 101 FDG-PET scans, as well as computed tomography and blood tests. Management was based on the findings from these investigations. Median follow-up was 16 mo. RESULTS AND LIMITATIONS Of the 24 patients with negative [18F]-FDG-PET, none (0%) had malignancy on biopsy (negative predictive value 100%). [18F]-FDG-PET identified malignancy in 4/4 patients (100%) before biopsy. All four patients had highly avid PET (maximum standardised uptake value ≥4) with atypical avidity distribution. [18F]-FDG-PET revealed avidity in 19/38 patients (50%) with normal inflammatory markers and no avidity in 10/63 patients (16%) with raised marker levels. Patients with highly avid PET were significantly more likely to respond to steroids compared to those with low avidity (9/11 [82%] vs 3/24 [12%]; p<0.01) or negative PET (9/11 [82%] vs 0/14 [0%]; p<0.01). Limitations include the small number of patients and the predominance of tertiary referrals, which may represent patients with particularly problematic RPF. CONCLUSIONS This study has established a promising role for [18F]-FDG-PET in optimising and individualising the treatment of RPF. PATIENT SUMMARY This study shows that [18F]-fluorodeoxyglucose positron emission tomography scans could reduce the need for biopsy in patients with retroperitoneal fibrosis (RPF). This technique can distinguish cancer from noncancerous RPF, and may be better than blood tests in assessing and monitoring RPF. It also appears to predict a patient's response to steroids, which should allow more individualised treatment.
Collapse
Affiliation(s)
- Archie Fernando
- The Urology Centre, Guy's and St Thomas' Hospital Foundation Trust, London, UK.
| | - James Pattison
- Department of Nephrology, Guy's and St Thomas' Hospital Foundation Trust, London, UK
| | - Catherine Horsfield
- Department of Histopathology, Guy's and St Thomas' Hospital Foundation Trust, London, UK
| | - David D'Cruz
- Department of Rheumatology and Immunology, Guy's and St Thomas' Hospital Foundation Trust, London, UK
| | - Gary Cook
- King's College London & Guy's & St Thomas' PET Centre, Division of Imaging Sciences and Biomedical Engineering, King's College London and Guy's & St Thomas' Hospital Foundation Trust, London, UK
| | - Tim O'Brien
- The Urology Centre, Guy's and St Thomas' Hospital Foundation Trust, London, UK
| |
Collapse
|
|
40
|
Zhang S, Chen M, Li CM, Song GD, Liu Y. Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography. Chin Med J (Engl) 2017; 130:691-697. [PMID: 28303852 PMCID: PMC5358419 DOI: 10.4103/0366-6999.201606] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
Background: Retroperitoneal fibrosis (RPF) and lymphoma presenting as retroperitoneal mass may closely resemble each other and misdiagnosis may occur. This study investigated the differential imaging features of RPF and lymphoma which presented as a retroperitoneal soft tissue using multidetector-row computed tomography (MDCT). Methods: The 42 consecutive patients were included in this retrospective review, including 19 RPF patients (45.2%; including 13 males and 6 females; mean age: 56.7 ± 6.2 years) and 23 patients with lymphoma (54.8%; including 14 males and 9 females; mean age: 57.4 ± 12.3 years). An array of qualitative computed tomography (CT) features of lesions in 42 consecutive patients with newly diagnosed untreated RPF and lymphoma were retrospectively analyzed. The quantitative size of the lesion at the para-aortic region and attenuation in the precontrast, arterial, and portal phases were calculated in regions of interest and compared between the patients with newly diagnosed untreated RPF and with lymphoma. Receiver operating characteristic curve analysis was used to assess the potential diagnostic value of each quantitative parameter. Inter-reader concordance was also calculated. Results: Mean ages between patients with RPF and lymphoma were not significantly different (56.7 ± 6.2 years vs. 57.4 ± 12.3 years P = 0.595). Compared to those in patients with lymphoma, homogeneous enhancement (65.2% vs. 94.7%, P = 0.027) and pelvic extension (52.2% vs. 89.5%, P = 0.017) were significantly more common while the involvement of additional nodes (78.3% vs. 5.3%, P < 0.001), suprarenal extension (60.9% vs. 15.8%, P = 0.004), and aortic displacement (43.5% vs. 5.3%, P = 0.006) were significantly less common in patients with RPF. Lesion size at the para-aorta was significantly greater in patients with lymphoma, compared with RPF patients (3.9 ± 1.2 cm vs. 1.8 ± 0.6 cm; P < 0.001). The attenuation values in three phases were not significantly different between patients with RPF and lymphoma. Inter-reader concordance for subjective features ranged from very good to excellent (range: 85.7–100.0%). Conclusions: This study showed that MDCT can help differentiate between untreated RPF and lymphoma on the basis of qualitative CT features and lesion sizes. Differentiating RPF from lymphoma on the basis of attenuation values in the precontrast, arterial, and portal phases was difficult to accomplish.
Collapse
Affiliation(s)
- Shuai Zhang
- Department of Radiology, Beijing Hospital, National Center of Gerontology, Beijing 100730; Graduate School, Peking Union Medical College, Beijing 100005, China
| | - Min Chen
- Department of Radiology, Beijing Hospital, National Center of Gerontology, Beijing 100730; Graduate School, Peking Union Medical College, Beijing 100005, China
| | - Chun-Mei Li
- Department of Radiology, Beijing Hospital, National Center of Gerontology, Beijing 100730, China
| | - Guo-Dong Song
- Department of Radiology, Beijing Hospital, National Center of Gerontology, Beijing 100730; Graduate School, Peking Union Medical College, Beijing 100005, China
| | - Ying Liu
- Department of Radiology, Beijing Hospital, National Center of Gerontology, Beijing 100730; Graduate School, Peking University Health Science Center, Peking University, Beijing 100083, China
| |
Collapse
|
|
41
|
Krishnan N, Perazella MA. The Role of PET Scanning in the Evaluation of Patients With Kidney Disease. Adv Chronic Kidney Dis 2017; 24:154-161. [PMID: 28501078 DOI: 10.1053/j.ackd.2017.01.002] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
Patients with underlying kidney disease are often required to undergo imaging for a variety of purposes including diagnosis and prognosis. A test that is being increasingly used with for this group of patients is the positron emission test (PET) scan. In addition, combining the nuclear medicine technique (PET) with computed tomography scan allows additional imaging advantages over either alone. These imaging modalities are commonly used for a number of extrarenal indications (ie, cancer, coronary artery disease, central nervous system disease, infectious diseases, and others). They have also been used for diagnosis of acute tubulointerstitial nephritis, evaluation and management of retroperitoneal fibrosis, identifying infection within kidney and liver cysts, and distinguishing complex kidney cysts from kidney cancer in patients with underlying CKD. We will review PET scan utility in patients with kidney disease.
Collapse
|
|
42
|
Rossi GM, Rocco R, Accorsi Buttini E, Marvisi C, Vaglio A. Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. Intern Emerg Med 2017; 12:287-299. [PMID: 28070877 DOI: 10.1007/s11739-016-1599-z] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/18/2016] [Accepted: 12/27/2016] [Indexed: 02/07/2023]
Abstract
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.
Collapse
Affiliation(s)
| | - Rossana Rocco
- Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy
| | | | - Chiara Marvisi
- Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy
| | - Augusto Vaglio
- Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.
| |
Collapse
|
|
43
|
[Management of ureteral obstruction : Value of percutaneous nephrostomy and ureteral stents]. Urologe A 2016; 55:1497-1510. [PMID: 27787581 DOI: 10.1007/s00120-016-0253-8] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
Ureteral obstruction represents a heterogeneous disease pattern and is treated by ureteral stenting or percutaneous nephrostomy (PCN) depending on the necessity. The benefits of urinary diversion with ureteral stenting or PCN in malignant ureteral obstruction (MUO) for patient survival are only moderate. No differences have been found between ureteral stenting and PCN in MUO with regard to median patient survival and complication rates. In cases of MUO there is currently no evidence that urinary diversion improves the quality of life. Alternative concepts of ureteral stenting, such as tandem ureteral stents, metallic ureteral stents or metal mesh ureteral stents have not yet shown clear benefits. In benign ureteral obstruction, prospective randomized studies have demonstrated comparable quality of life after PCN or ureteral stenting. The method of choice for urinary diversion is influenced by the recommendations, personal experience of the clinician and the availability of the method.
Collapse
|
|
44
|
Laroche AS, Bell RZ, Bezzaoucha S, Földes E, Lamarche C, Vallée M. Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management. Res Rep Urol 2016; 8:175-179. [PMID: 27822461 PMCID: PMC5087765 DOI: 10.2147/rru.s115448] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023] Open
Abstract
INTRODUCTION Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs. This study describes the clinical characteristics, diagnostic methods, and treatments and their effects on renal function. METHODS We conducted a retrospective analysis of patients diagnosed with RPF at Maisonneuve-Rosemont Hospital. RESULTS We identified 17 patients with RPF between 1998 and 2013. Eight patients were females (47%), and the mean age was 62±18 years. Eleven patients were idiopathic. Back pain was the most common symptom. All diagnoses were made based on the finding of a retroperitoneal mass on the computed tomography scan. Three patients had histological diagnosis of RPF and seven patients had unspecific changes on their biopsy. Twelve patients needed double-J stents, three patients had a temporary percutaneous nephrostomy, two patients had to have a nephrectomy for refractory ureteral obstruction, and one patient required hemodialysis. Ten patients with idiopathic RPF received medical treatment. In the treated group, only two patients had complete remission of the disease and five patients had improvement of their lesions. There were no deteriorations and only one relapse. Seven patients did not receive any treatment; two of them achieved complete remission, one of them deteriorated, and two of them had no changes. CONCLUSION Most of our cases of RPF were idiopathic. Almost all treated patients received prednisone and seemed to respond, at least partially. There was a lot of heterogeneity in patient management, which makes it difficult to compare treatment effects. However, treated patients seemed to have more favorable outcomes than those who were not.
Collapse
Affiliation(s)
| | | | | | - Eva Földes
- Section of Internal Medicine, Department of Medicine, Maisonneuve-Rosemont Hospital, University of Montreal, Montreal, QC, Canada
| | | | | |
Collapse
|
|
45
|
Ito T, Okamoto R, Taniguchi M, Tanabe M, Komatsubara H, Yamada N, Ito M. Hypertensive emergency preceding the progression of periaortitis and retroperitoneal fibrosis: case report and review of the literature. Blood Press 2016; 25:327-30. [PMID: 27112324 DOI: 10.3109/07434618.2016.1168968] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Hypertensive emergency is an important entity which should be managed adequately. A 65-year-old woman presented with blindness and elevated blood pressure of 254/131 mmHg. She was diagnosed with hypertensive emergency on physical examination, and brain magnetic resonance imaging showed posterior reversible encephalopathy syndrome (PRES) with bilateral thalamic haemorrhage. After controlling her blood pressure with intravenous antihypertensive agents, periaortitis and retroperitoneal fibrosis (RPF) were manifested by left hydronephrosis and creatinine elevation. She was diagnosed with periaortitis/RPF. Her blood pressure was well controllable, and PRES improved after treatment with prednisolone. Periaortitis/RPF should not be overlooked when hypertensive emergency suddenly occurs in patients with no history of hypertension.
Collapse
Affiliation(s)
- Takayasu Ito
- a Department of Cardiology and Nephrology , Mie University Graduate School of Medicine , Mie , Japan
| | - Ryuji Okamoto
- a Department of Cardiology and Nephrology , Mie University Graduate School of Medicine , Mie , Japan
| | - Masaya Taniguchi
- a Department of Cardiology and Nephrology , Mie University Graduate School of Medicine , Mie , Japan
| | - Masaki Tanabe
- a Department of Cardiology and Nephrology , Mie University Graduate School of Medicine , Mie , Japan
| | - Haruna Komatsubara
- a Department of Cardiology and Nephrology , Mie University Graduate School of Medicine , Mie , Japan
| | - Norikazu Yamada
- a Department of Cardiology and Nephrology , Mie University Graduate School of Medicine , Mie , Japan
| | - Masaaki Ito
- a Department of Cardiology and Nephrology , Mie University Graduate School of Medicine , Mie , Japan
| |
Collapse
|
|
46
|
Maritati F, Iannuzzella F, Pavia MP, Pasquali S, Vaglio A. Kidney involvement in medium- and large-vessel vasculitis. J Nephrol 2016; 29:495-505. [PMID: 27098921 DOI: 10.1007/s40620-016-0303-8] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2016] [Accepted: 03/29/2016] [Indexed: 12/12/2022]
Abstract
Medium- and large-vessel vasculitides (MVV and LVV, respectively) comprise a heterogeneous group of disorders whose common denominator is the inflammatory involvement of vessels of medium and large size. This disease spectrum includes giant-cell arteritis and Takayasu's arteritis, which typically affect the aorta and its main branches, and Kawasaki's disease and polyarteritis nodosa, which involve medium-sized arteries. Chronic periaortitis, characterized by a perivascular fibro-inflammatory reaction affecting the abdominal aorta and the periaortic tissue, frequently has a systemic distribution, involving other segments of the aorta and its major branches, and could thus be included in this group. Unlike small-vessel vasculitides, MVV and LVV do not cause glomerulonephritis, although glomerular immune-mediated lesions and tubulo-interstitial nephritis occur with varying frequency. However, MVV and LVV can often involve the renal artery and its branches, causing a wide array of lesions that range from renal artery stenosis to intra-renal vasculitis causing renal ischaemia/infarction, microaneurysms and haemorrhage. This review focuses on renal involvement in MVV and LVV and underlines why renal abnormalities in these syndromes should not be overlooked.
Collapse
Affiliation(s)
| | | | | | - Sonia Pasquali
- Nephrology Unit, Arcispedale S. Maria Nuova, Reggio Emilia, Italy
| | | |
Collapse
|
|
47
|
Hsu L, Li H, Pucheril D, Hansen M, Littleton R, Peabody J, Sammon J. Use of percutaneous nephrostomy and ureteral stenting in management of ureteral obstruction. World J Nephrol 2016; 5:172-181. [PMID: 26981442 PMCID: PMC4777789 DOI: 10.5527/wjn.v5.i2.172] [Citation(s) in RCA: 49] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2015] [Revised: 12/25/2015] [Accepted: 01/22/2016] [Indexed: 02/06/2023] Open
Abstract
The management options for ureteral obstruction are diverse, including retrograde ureteral stent insertion or antegrade nephrostomy placement, with or without eventual antegrade stent insertion. There is currently no consensus on the ideal treatment or treatment pathway for ureteral obstruction owing, in part, to the varied etiologies of obstruction and diversity of institutional practices. Additionally, different clinicians such as internists, urologists, oncologists and radiologists are often involved in the care of patients with ureteral obstruction and may have differing opinions concerning the best management strategy. The purpose of this manuscript was to review available literature that compares percutaneous nephrostomy placement vs ureteral stenting in the management of ureteral obstruction from both benign and malignant etiologies.
Collapse
|
|
48
|
Abstract
Idiopathic retroperitoneal fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory disorders involving other organs, is considered part of the spectrum of IgG4-related disease, and often arises in patients with other autoimmune conditions. Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the B cell-depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases.
Collapse
|
|
49
|
Alvarez Argote J, Bauer FA, Posteraro AF, Dasanu CA. Retroperitoneal fibrosis due to B-cell non-Hodgkin lymphoma: Responding to rituximab! J Oncol Pharm Pract 2016; 22:179-85. [PMID: 25013186 DOI: 10.1177/1078155214543279] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Retroperitoneal fibrosis is a rare disease manifesting as chronic soft tissue fibrosis in the retroperitoneum, with potential anatomic and/or functional compromise of adjacent organs. It can be primary (idiopathic) or secondary to other conditions such as cancers, autoimmune disorders, or drugs. We report herein a 66-year-old patient with symptomatic retroperitoneal fibrosis leading to bilateral hydronephrosis and renal failure, in whom, after a complex diagnostic work-up and protracted clinical course, a B-cell non-Hodgkin lymphoma in the retroperitoneal space and several vertebral bodies was identified. The patient was treated with radiation therapy and weekly rituximab infusions, with resolution of hydronephrosis and lower back pain. We include a thorough literature review on etiopathogenesis, diagnosis, therapy, and prognosis of retroperitoneal fibrosis. A meticulous search for malignancy is necessary in this rare condition that, if positive, may have significant therapeutic and prognostic implications.
Collapse
Affiliation(s)
| | - Frank A Bauer
- Department of Pathology, St. Francis Hospital and Medical Center, Hartford, USA
| | - Anthony F Posteraro
- Department of Radiology, St. Francis Hospital and Medical Center, Hartford, USA
| | - Constantin A Dasanu
- Department of Hematology-Oncology, St. Francis Hospital and Medical Center, Hartford, USA
| |
Collapse
|
|
50
|
van der Bilt FE, Hendriksz TR, van der Meijden WAG, Brilman LG, van Bommel EFH. Outcome in patients with idiopathic retroperitoneal fibrosis treated with corticosteroid or tamoxifen monotherapy. Clin Kidney J 2016; 9:184-91. [PMID: 26985367 PMCID: PMC4792623 DOI: 10.1093/ckj/sfv148] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/26/2015] [Accepted: 12/02/2015] [Indexed: 01/20/2023] Open
Abstract
Background Although corticosteroids (CS) are used primarily in idiopathic retroperitoneal fibrosis (iRPF), tamoxifen (TMX) may be a suitable alternative. We compared outcome with CS or TMX monotherapy for first presentation in a large group of patients with iRPF disease. Methods Of all patients with iRPF disease who were seen at our tertiary care referral centre from February 1999 to December 2011, 118 patients were eligible for this retrospective study. Treatment success was defined as the composite of (i) amelioration of symptoms, (ii) computed tomography (CT)-documented mass regression and, if applicable, (iii) definitive removal of ureteral stent or nephrostomy tube. Recurrence was defined as recurrence of signs and symptoms and/or CT-documented mass increase after initial treatment success with primary treatment. Results Presenting signs and symptoms did not differ between patients treated with CS (n = 50) or TMX (n = 68). Time to amelioration of symptoms after treatment initiation was shorter in CS-treated patients [CS, 2.0 (0.8–3.8) weeks versus TMX, 4.0 (2.0–6.0) weeks; P < 0.01]. Short-term percentual decrease in acute-phase reactant levels (P < 0.001 for both erythrocyte sedimentation rate and C-reactive protein) and serum creatinine level (P < 0.01) following treatment initiation was greater in CS-treated patients compared with that in TMX-treated patients. Mass regression at first follow-up CT scan was observed more frequently in CS-treated patients (CS, 84.0% versus TMX, 68.3%; P = 0.05) with no difference in time interval from treatment initiation to first follow-up CT between groups [CS, 5 (2–7) months versus TMX, 4 (4–5) months; P = 0.34]. Definite treatment success was non-significantly higher in CS-treated patients (CS, 72.7% versus TMX, 58.3%; P = 0.15). In patients with initial treatment success with primary treatment, recurrence rate was lower in TMX-treated patients (CS, 62.5% versus TMX, 21.4%; P < 0.01). Conclusions CS are superior to TMX in treating iRPF disease. However, in patients with initial treatment success with primary treatment, recurrence rate was lower in TMX-treated patients.
Collapse
Affiliation(s)
- Floor E van der Bilt
- Department of Nephrology , Albert Schweitzer Hospital , Dordrecht , The Netherlands
| | - Tadek R Hendriksz
- Department of Radiology , Albert Schweitzer Hospital , Dordrecht , The Netherlands
| | | | - Lisette G Brilman
- Department of Nephrology , Albert Schweitzer Hospital , Dordrecht , The Netherlands
| | - Eric F H van Bommel
- Department of Nephrology , Albert Schweitzer Hospital , Dordrecht , The Netherlands
| |
Collapse
|