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Dong ZY, Zhu HB. Idiopathic retroperitoneal fibrosis arising from peritoneal space: A case report and review of literature. World J Gastroenterol 2025; 31:105443. [DOI: 10.3748/wjg.v31.i18.105443] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2025] [Revised: 03/27/2025] [Accepted: 04/24/2025] [Indexed: 05/13/2025] Open
Abstract
BACKGROUND Retroperitoneal fibrosis is a rare fibro-inflammatory condition which can be classified into idiopathic (accounting for over 75%) and secondary types (due to malignancies, infections, medications, radiotherapy or other conditions). Idiopathic retroperitoneal fibrosis (IRPF) typically affects the abdominal aorta and iliac arteries along with the surrounding retroperitoneal area. This case review aims to summarize the imaging characteristics of IRPF arising from the peritoneal space.
CASE SUMMARY An abdominal mass was discovered in a 52-year-old man during a routine physical examination, he had not complained of abdominal pain, distension, nausea, vomiting, diarrhea, fever, and had no significant past medical or family history. Abdominal magnetic resonance imaging revealed a soft tissue mass with poorly defined margins surrounding the duodenum, exhibiting slight to moderate high signal intensity on both T1-weighted and T2-weighted images. Diffusion-weighted imaging with a β value of 800 mm²/second demonstrated slightly to moderate high signal intensity. Dynamic contrast enhanced images showed uneven enhancement on the arterial phase, with significant enhancement observed on the delayed phase. The mass infiltrated adjacent structures, including the head of the pancreas, the hepatic flexure of the colon, and part of the intestine, raising suspicion for malignant tumors such as sarcoma or lymphoma. However, surgery confirmed the diagnosis of IRPF. The patient underwent routine follow-up for one year, with no recurrence.
CONCLUSION IRPF is a rare condition that presents considerable diagnostic challenges when lesions arise from the peritoneal space. In cases where imaging findings are atypical, a further puncture biopsy may be necessary to confirm the diagnosis.
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Affiliation(s)
- Zhen-Yu Dong
- Department of Radiology, Qingdao Huangdao District People’s Hospital, Qingdao 266400, Shandong Province, China
| | - Hai-Bin Zhu
- Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Radiology, Peking University Cancer Hospital and Institute, Beijing 100142, China
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Ji Z, Sun Y, Zhang L, Kong X, Jiang L. Predictors of irreversible renal dysfunction in patients with idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 2025; 64:2855-2861. [PMID: 39460947 DOI: 10.1093/rheumatology/keae565] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2024] [Revised: 09/10/2024] [Accepted: 09/22/2024] [Indexed: 10/28/2024] Open
Abstract
OBJECTIVES Idiopathic retroperitoneal fibrosis (iRPF) can lead to irreversible kidney damage. This study aimed to investigate predictors of irreversible renal dysfunction in patients with iRPF. METHODS Eighty-three patients with newly diagnosed iRPF were enrolled between January 2010 and September 2022 at Zhongshan Hospital of Fudan University, including 60 in the training set and 23 in the validation set. They were regularly contacted or followed up via outpatient examinations by specialist doctors, who documented their condition and treatment progress. Predictors of irreversible renal dysfunction were identified using univariate and multivariate regression, logistic model, and receiver operating curve analyses. RESULTS In the training set, over a median follow-up of 29 months, 16.7% of patients had an estimated glomerular filtration rate (eGFR) of <60 ml/min/1.73 m2 at the last follow-up, and 25% had hydronephrosis or required prolonged double-J stents. A prognostic score was developed by assigning 1, 1, and 2 points for peripheral CD19+ B cells <9.3%, serum creatinine (sCr) ≥120 µmol/L, and no response at 6 months, respectively. A score of ≥2 for predicting irreversible renal dysfunction had sensitivity and specificity of 100% and 92%, respectively. In the validation set, 21.7% of patients suffered from irreversible renal dysfunction. The sensitivity and specificity for predicting irreversible renal dysfunction were 100% and 94.4%, respectively. CONCLUSIONS A prognostic score based on factors including CD19+ B cells <9.3% and sCr ≥120 µmol/L at baseline, and no response at 6 months, is suitable for predicting irreversible renal dysfunction in iRPF.
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Affiliation(s)
- Zongfei Ji
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Ying Sun
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Lijuan Zhang
- Department of Rheumatology, Xiamen Branch, Zhongshan Hospital, Fudan University, Xiamen, China
| | - Xiufang Kong
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Lindi Jiang
- Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
- Evidence-Based Medicine Center, Fudan University, Shanghai, China
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Průcha M, Zdráhal P, Kříž R, Šnajdrová A, Voska L. Ormond's Disease - 26 Years of Experience at One Centre. Prague Med Rep 2025; 126:3-8. [PMID: 40026157 DOI: 10.14712/23362936.2025.1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/04/2025] Open
Abstract
Ormond's disease is a systemic autoimmune disease with serious complications. We present our retrospective analysis of 83 patients diagnosed with and treated for idiopathic retroperitoneal fibrosis (Ormond's disease) in our department from 1997 to 2023. In this retrospective study, we analysed the diagnostic approaches, the clinical history and surgical and immunosuppressive therapies, and their subsequent effects on our patients. Patients with established disease activity were given immunosuppressive treatment, using corticosteroids alone or in combination with azathioprine, in patients with exacerbation of the disease mycophenolate mofetil. Three patients with Ormond's disease and systemic complications (IgG4-related disease) were treated with rituximab. In the entire cohort, 83 patients received immunosuppressive therapy; the next 5 patients did not receive this treatment because they did not present inflammatory activity from the disease. In these 83 patients, computed tomography showed that immunosuppressive treatment resulted in partial or complete regression of the inflammatory infiltrate. Out of the 83 patients, 10 patients experienced disease exacerbation 7 and 24 months after the immunosuppressive treatment was discontinued. The follow-up ranged from 24 months to 26 years.
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Affiliation(s)
- Miroslav Průcha
- Department of Clinical Biochemistry, Haematology and Immunology, Hospital Na Homolce, Prague, Czech Republic.
| | - Pavel Zdráhal
- Department of Vascular Surgery, Hospital Na Homolce, Prague, Czech Republic
| | - Radek Kříž
- Department of Radiology, Hospital Na Homolce, Prague, Czech Republic
| | - Alena Šnajdrová
- Department of Radiology, Hospital Na Homolce, Prague, Czech Republic
| | - Luděk Voska
- Department of Clinical and Experimental Pathology, IKEM, Prague, Czech Republic
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Liu H, Gao H, Zhao JX, Wong UK, Liu SB, Liu J, Zhang G, Wang KT, Wang Y, Zhao L, Ma XB, Lu YW, Zhang XW. Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients. Front Med (Lausanne) 2024; 11:1435870. [PMID: 39717179 PMCID: PMC11663637 DOI: 10.3389/fmed.2024.1435870] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2024] [Accepted: 11/15/2024] [Indexed: 12/25/2024] Open
Abstract
Objective Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes have not been well elucidated. Methods A total of 115 RPF-related hydronephrosis patients have been recruited from 9 clinical centers in China since March 2010. They were followed up until death or September 2021, whichever came first. Results The mean age at diagnosis was 58.83 ± 12.13 years, and 80 patients (69.57%) were men. The median disease duration was 3.00 (1.00, 9.00) months. Renal impairment was observed in 88.35% of the patients, and 49.57% showed bilateral ureteral involvement. Elevated ESR and CRP were presented in 80.28 and 62.02% of the patients, respectively. Overall, 28.21% (11/39) of the patients had increased IgG4 levels, and 41.38% (12/29) showed positive pathological IgG4 staining (IgG4+/IgG+ ≥ 40% or IgG4+ ≥ 10/HPF). Among them, three patients were diagnosed as IgG4RD. After 60.43 ± 34.53 months of follow-up, 36 patients had poor prognosis, which was associated with severe kidney impairment, bilateral hydronephrosis and inflammation status (elevated ESR and IgG) at diagnosis by case-control study. eGFR and creatinine were independent risk factors after adjusting for all other significant associations (p = 0.002 and p = 0.067, respectively). Glucocorticoid-based therapy could reduce the time of stenting, decrease the need for long-term ureteral stenting/percutaneous nephrostomy (PNS)/ureterolysis, increase the rate of mass shrinkage, and reduce the new requirement of hemodialysis compared to surgery-only strategy for RPF-related hydronephrosis patients in need of renal drainage, but did not reduce new-onset renal atrophy. Conclusion Severity of kidney dysfunction and inflammation status were related to the poor prognosis of hydronephrosis induced by RPF. More efficient interventions and strategies are needed to further improve outcomes.
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Affiliation(s)
- Hongyan Liu
- Department of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Hui Gao
- Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China
| | - Jin-xia Zhao
- Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China
| | - Ut-kei Wong
- Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China
| | - Shi-bo Liu
- Department of Retroperitoneal Tumor Surgery, Peking University International Hospital, Beijing, China
| | - Jian Liu
- Department of Rheumatology and Immunology, Aerospace Center Hospital, Peking University Aerospace School of Clinical Medicine, Beijing, China
| | - Ge Zhang
- Department of Rheumatology and Immunology, Beijing Haidian Hospital, Beijing Haidian Section of Peking University Third Hospital, Beijing, China
| | - Kuan-ting Wang
- Department of Rheumatology and Immunology, Peking University Shougang Hospital, Beijing, China
| | - Yan Wang
- Department of Rheumatology and Immunology, The Fifth Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Lin Zhao
- Department of Endocrinology, Rheumatology, and Immunology, The Fourth Clinical College of Xinxiang Medical University, Xinxiang, China
| | - Xiang-bo Ma
- Department of Rheumatology and Immunology, Handan First Hospital, Handan, China
| | - Yue-wu Lu
- Department of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Xue-wu Zhang
- Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China
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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.
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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
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Xu J, Wang J, Chen H, Ye S, Guo H, Liu J, Xue H. Multiple Myeloma and Retroperitoneal Fibrosis: A Rare Association Report and Literature Review. Transplant Proc 2024; 56:1836-1840. [PMID: 39244448 DOI: 10.1016/j.transproceed.2024.08.037] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/22/2024] [Accepted: 08/24/2024] [Indexed: 09/09/2024]
Abstract
Multiple myeloma (MM) is a common hematological malignancy. Autologous hematopoietic stem cell transplantation (auto-HSCT) can significantly improve the prognosis of patients with MM, but a variety of complications may occur after transplantation. Retroperitoneal fibrosis (RPF) is a rare cause of obstructive nephropathy. Because there are no specific symptoms at the time of onset and the course of the disease is often insidious, special laboratory and instrumental examination methods are usually needed to confirm the diagnosis. This article describes the clinical case of a 50-year-old female patient diagnosed with multiple myeloma. She developed postoperative acute kidney injury (AKI) more than 20 days after transplantation and was subsequently diagnosed with retroperitoneal fibrosis. After multidisciplinary collaboration, early transurethral vesicoureteral stent placement was performed, the obstruction was relieved, and her renal function returned to normal. Reports of retroperitoneal fibrosis after multiple myeloma transplantation are relatively rare. This case report advances our understanding of these 2 diseases, and the correlation between MM and RPF warrants further exploration.
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Affiliation(s)
- Jianmei Xu
- Department of Hematology, Affiliated Hospital of Hebei University, Baoding, China
| | - Jing Wang
- Department of Hematology, Affiliated Hospital of Hebei University, Baoding, China
| | - Hao Chen
- Department of Electrocardiography, Affiliated Hospital of Hebei University, Baoding, China
| | - Shaojie Ye
- Department of Hematology, Affiliated Hospital of Hebei University, Baoding, China
| | - Huimei Guo
- Department of Hematology, Affiliated Hospital of Hebei University, Baoding, China
| | - Jia Liu
- Department of Hematology, Affiliated Hospital of Hebei University, Baoding, China
| | - Hua Xue
- Department of Hematology, Affiliated Hospital of Hebei University, Baoding, China.
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Kunow A, Freyer Martins Pereira J, Chenot JF. Extravertebral low back pain: a scoping review. BMC Musculoskelet Disord 2024; 25:363. [PMID: 38714994 PMCID: PMC11075250 DOI: 10.1186/s12891-024-07435-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2023] [Accepted: 04/11/2024] [Indexed: 05/12/2024] Open
Abstract
BACKGROUND Low back pain (LBP) is one of the most common reasons for consultation in general practice. Currently, LBP is categorised into specific and non-specific causes. However, extravertebral causes, such as abdominal aortic aneurysm or pancreatitis, are not being considered. METHODS A systematic literature search was performed across MEDLINE, Embase, and the Cochrane library, complemented by a handsearch. Studies conducted between 1 January 2001 and 31 December 2020, where LBP was the main symptom, were included. RESULTS The literature search identified 6040 studies, from which duplicates were removed, leaving 4105 studies for title and abstract screening. Subsequently, 265 publications were selected for inclusion, with an additional 197 publications identified through the handsearch. The majority of the studies were case reports and case series, predominantly originating from specialised care settings. A clear distinction between vertebral or rare causes of LBP was not always possible. A range of diseases were identified as potential extravertebral causes of LBP, encompassing gynaecological, urological, vascular, systemic, and gastrointestinal diseases. Notably, guidelines exhibited inconsistencies in addressing extravertebral causes. DISCUSSION Prior to this review, there has been no systematic investigation into extravertebral causes of LBP. Although these causes are rare, the absence of robust and reliable epidemiological data hinders a comprehensive understanding, as well as the lack of standardised protocols, which contributes to a lack of accurate description of indicative symptoms. While there are certain disease-specific characteristics, such as non-mechanical or cyclical LBP, and atypical accompanying symptoms like fever, abdominal pain, or leg swelling, that may suggest extravertebral causes, it is important to recognise that these features are not universally present in every patient. CONCLUSION The differential diagnosis of extravertebral LBP is extensive with relatively low prevalence rates dependent on the clinical setting. Clinicians should maintain a high index of suspicion for extravertebral aetiologies, especially in patients presenting with atypical accompanying symptoms.
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Affiliation(s)
- Anna Kunow
- Department of General Practice, University Medicine Greifswald, 17475, Fleischmannstraße, Greifswald, Germany.
| | | | - Jean-François Chenot
- Department of General Practice, University Medicine Greifswald, 17475, Fleischmannstraße, Greifswald, Germany
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Wiedbrauck D, Flemming P, Hollerbach S. Role of transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of retroperitoneal fibrosis (Ormond's disease). ZEITSCHRIFT FUR GASTROENTEROLOGIE 2023; 61:1623-1627. [PMID: 36972595 DOI: 10.1055/a-2003-9752] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 03/29/2023]
Abstract
BACKGROUND Retroperitoneal fibrosis (RPF), often referred to as Ormond's disease when it is of idiopathic origin, is a rare disease characterized by the presence of inflammatory infiltrates and periaortic masses in the retroperitoneum. For a definite diagnosis, a biopsy and subsequent pathological examination is required. Currently accepted methods for retroperitoneal biopsy include open, laparoscopic, or CT-guided approaches. However, transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of RPF has attracted only little attention in the literature. CASE REPORTS We report two male patient cases who presented with leukocytosis, elevated C-reactive protein, and a suspicious retroperitoneal mass of unknown origin on computed tomography. One patient also reported left lower quadrant pain, whereas the other patient suffered from back pain and weight loss. In both patients, idiopathic RPF was successfully diagnosed by using transduodenal EUS-FNA/FNB with 22- and 20-gauge aspiration needles. Histopathology revealed dense lymphocytic infiltrates and fibrosis. The procedures lasted approximately 25 and 20 minutes, respectively, and in both patients no serious adverse events occurred. Treatment included steroid therapy and administration of Azathioprine. CONCLUSION We demonstrate that using EUS-FNA/FNB to diagnose RPF is a feasible, fast, and safe method, which should always be considered as a first-line diagnostic modality. Hence, this case report emphasizes that gastrointestinal endoscopists are likely to play an important role in the setting of suspected RPF.
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Affiliation(s)
- Damian Wiedbrauck
- Department of Gastroenterology, Allgemeines Krankenhaus Celle, Celle, Germany
| | | | - Stephan Hollerbach
- Department of Gastroenterology, Allgemeines Krankenhaus Celle, Celle, Germany
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Batra VS, Singh M, Kathuria S, Jain S, Rasool S, Gupta M, Pandey H, Sharma A. Minimally invasive ureterolysis and intraperitonealization of ureter for idiopathic retroperitoneal fibrosis; single center analysis of an erratic disease. Urologia 2023; 90:535-541. [PMID: 35730727 DOI: 10.1177/03915603221104047] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/20/2023]
Abstract
INTRODUCTION Idiopathic retroperitoneal fibrosis is a known cause of obstructive uropathy. Ureterolysis is done when medical management fails or the presentation is at an advanced stage. Conventionally ureterolysis without omental wrap has been considered incomplete. Our Institute has experience of laparoscopic or robotic ureterolysis with intraperitonealization of the ureter alone and no other adjunctive procedure. This study retrospectively assesses the result of the procedure with patients presenting with varying severity of disease. METHODS From 2008, all patients who underwent laparoscopic or robotic ureterolysis were analyzed retrospectively for pre-operative management, operative findings, and post operative outcomes. RESULTS We operated and released nine renal units in seven patients. Two of the nine cases were performed robotically completely and the rest was performed by laparoscopic approach. Median follow up was 60 months. All patients documented resolution of symptoms. The mean post-operative creatinine at 1 year was significantly decreased to 1.47 ± 0.49 mg/dl in comparison to preoperative creatinine (p < 0.05). The postoperative mean ESR decreased significantly from a preoperative value of 58.2 ± 19.41 mm to 15.8 ± 17.23. The nuclear scan revealed unobstructed drainage and radiological imaging revealed resolution of hydronephrosis and fibrosis in all. The mean GFR on the nuclear scan after 3 and 12 months of surgery was 36.3 ± 4.33 and 40 ± 3.77, respectively. Thus, there was significant increase noted in GFR at 3 and 12 months in comparison to preoperative GFR (p < 0.05). CONCLUSION Laparoscopic/robotic ureterolysis with intraperitonealization alone is secure and durable procedure for idiopathic retroperitoneal fibrosis needing surgical release.
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Affiliation(s)
| | | | | | - Saurabh Jain
- Department of Urology, Sir Ganga Ram Hospital, Delhi, India
| | | | - Manu Gupta
- Department of Urology, Sir Ganga Ram Hospital, Delhi, India
| | | | - Ajay Sharma
- Department of Urology, Sir Ganga Ram Hospital, Delhi, India
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Vijayan J, Bora H, Ali A, K R D. Investigating the Link Between Low Back Ache, Abdominal Discomfort and Oliguria: A Unique Case of Histopathology-Proven Retroperitoneal Fibrosis. Cureus 2023; 15:e44220. [PMID: 37767266 PMCID: PMC10522402 DOI: 10.7759/cureus.44220] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/26/2023] [Indexed: 09/29/2023] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare condition characterized by systemic inflammation and the proliferation of fibroinflammatory tissues in the retroperitoneum. It may lead to the formation of a retroperitoneal mass and can encase the aorta, its branches and ureters. The pathogenesis of RPF is not fully known. We report a case of a 52-year-old male presented with low back ache, flank pain, swelling of legs, oliguria and features of obstructive uropathy, later diagnosed to be RPF. The mainstay of diagnosis includes blood workup, imaging and biopsy. The first line of treatment is corticosteroids. Surgical intervention is carried out when medical measures have failed or when contraindicated. Early diagnosis and prevention of complications is the key, and a high degree of suspicion is needed.
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Affiliation(s)
| | | | - Amir Ali
- Pharmacy, National Institute of Pharmaceutical Education and Research, Guwahati, IND
| | - Devika K R
- Radiodiagnosis, Fakhruddin Ali Ahmed Medical College and Hospital, Barpeta, IND
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Numata Y, Sasaki S, Magara K, Takasawa A, Sugawara T, Ohara N, Akutsu N, Hasegawa T, Osanai M, Nakase H. Generalized crystal-storing histiocytosis with noncirrhotic portal hypertension: an autopsy case report. Clin J Gastroenterol 2023; 16:450-456. [PMID: 37014540 DOI: 10.1007/s12328-023-01782-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/06/2022] [Accepted: 03/06/2023] [Indexed: 04/05/2023]
Abstract
Crystal-storing histiocytosis (CSH) is a rare disease associated with the accumulation of histiocytes containing crystalline matter within their cytoplasm. Herein, we present the case of a female patient who was diagnosed with Tolosa-Hunt syndrome at 45 years of age and idiopathic retroperitoneal fibrosis when she was 48 years. She developed portal hypertension (PH), but did not present with cirrhosis; as such, the cause of PH was not identified. Her PH gradually worsened when she was 54 years, and at the age of 60 years, she died from an acute subdural hematoma. Autopsy revealed retroperitoneal fibrosis with severe fibrosis extending around the hepatic veins and into the porta hepatis. Histologically, the retroperitoneal tissue showed a dense infiltrate of eosinophilic histiocytes with crystal structures in the cytoplasm, which was pathologically diagnosed as CSH. Nodular regenerative hyperplasia was observed in the liver parenchyma, whereas cirrhosis was not. In the present case, CSH caused fibrosis, which was believed to be the cause of PH. In addition, we considered that nodular regenerative hyperplasia caused by the altered hepatic blood flow due to treatment of gastric varices contributed to worsening PH. Hence, CSH should be considered as an underlying disease in noncirrhotic portal hypertension.
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Affiliation(s)
- Yasunao Numata
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
| | - Shigeru Sasaki
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan.
| | - Kazufumi Magara
- Department of Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Akira Takasawa
- Department of Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Taro Sugawara
- Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Naruki Ohara
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
| | - Noriyuki Akutsu
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
| | - Tadashi Hasegawa
- Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Makoto Osanai
- Department of Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Hiroshi Nakase
- Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome Chuo-Ku, Sapporo, 060-8543, Japan
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Gianfreda D, Superchi E, Peyronel F, Mazzariol M, Vaglio A. Chronic periaortitis: A clinical approach. Rev Med Interne 2023; 44:79-84. [PMID: 36697342 DOI: 10.1016/j.revmed.2022.11.009] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2022] [Revised: 11/13/2022] [Accepted: 11/30/2022] [Indexed: 01/25/2023]
Abstract
Chronic periaortitis (CP) is a rare disease characterised by the presence of a fibro-inflammatory tissue typically enveloping the abdominal aorta, the iliac arteries and, in some cases, the nearby structures, such as the ureters and the inferior vena cava. Imaging plays a key role in the diagnosis and follow-up: computed tomography and magnetic resonance imaging scans are used to define the extension of the pathological tissue, whereas fluorodeoxyglucose positron emission tomography is the gold standard to establish the degree of its metabolic activity. CP must be distinguished from secondary forms of periaortic infiltration, which include malignant, infectious, and drug-related aetiologies. This review focuses on the clinical aspects of CP and the differential diagnosis with secondary cases, and aims to provide the clinician with a guide through this challenging clinical approach.
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Affiliation(s)
- D Gianfreda
- Nephrology and Dialysis Unit, Santa Caterina Novella Hospital, Galatina, Italy
| | - E Superchi
- Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze, Italy
| | - F Peyronel
- Department of Experimental and Clinical Medicine, University of Fireze, Firenze, Italy; Nephrology and Dialysis Unit, Department of Pediatrics, Meyer Children's Hospital, Firenze, Italy
| | - M Mazzariol
- Department of Medical Sciences, University of Torino, Torino, Italy
| | - A Vaglio
- Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze, Italy; Nephrology and Dialysis Unit, Department of Pediatrics, Meyer Children's Hospital, Firenze, Italy.
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13
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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.
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14
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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
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15
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Si M, Zhang K, Li J, He H, Yao Y, Han J, Qiao J. Idiopathic retroperitoneal fibrosis with endometrial cancer: a case report and literature review. BMC Womens Health 2022; 22:399. [PMID: 36183056 PMCID: PMC9526921 DOI: 10.1186/s12905-022-01968-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2022] [Accepted: 09/11/2022] [Indexed: 11/09/2022] Open
Abstract
Background Retroperitoneal fibrosis is a rare disease characterized by chronic nonspecific inflammation, which leads to clinical compression manifestations of retroperitoneal organs especially ureter. Approximately 70 percent of retroperitoneal fibrosis cases are idiopathic which has no clear etiology. This study reported a rare case of a 48-year-old woman presented with idiopathic retroperitoneal fibrosis and endometrial cancer. Case presentation A 48-year-old woman presented with irregular vaginal bleeding without abdominal pain, bloating or discomfort. The patient was diagnosed iRPF after splenectomy 13 years ago. Then she took prednisone for 2 years and took tamoxifen for about 11 years. She stopped taking the medication from October 2019 to May 2020 and then started taking tamoxifen again until November 2020. Two weeks after she stopped taking tamoxifen, she presented with irregular vaginal bleeding. Gynecological ultrasound revealed a thick endometrium with uneven echo enhancement and blood flow signals. Then diagnostic curettage was performed with pathological examination showed endometroid carcinoma. Later, the patient was admitted to Peking University Third Hospital for surgery. Preoperative imaging examinations, including CT, MRI, and PET/CT, all showed pelvic enlarged lymph nodes and they were highly suspected to have lymph node metastasis. The patient underwent laparoscopic surgical staging and enlarged lymph nodes in the pelvic and aortic regions were removed. Finally, the pathology confirmed that endometrioid adenocarcinoma and fibrosis, but there was no tumor infiltration in these enlarged lymph nodes. The patient is now in good condition. Conclusion This case report stressed the difficulty to distinguish between lymph node metastasis and inflammatory hyperplasia by common imaging methods. Due to increased surgical difficulty among retroperitoneal patients, lymphadenectomy should be carefully evaluated to avoid additional surgical complications and over-treatment.
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Affiliation(s)
- Manfei Si
- grid.411642.40000 0004 0605 3760Center for Reproductive Medicine, Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.419897.a0000 0004 0369 313XKey Laboratory of Assisted Reproduction (Peking University), Ministry of Education, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Beijing Key Laboratory of Reproductive Endocrinology and Assisted Reproductive Technology, Beijing, 100191 China
| | - Kun Zhang
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Jiaxin Li
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Huiying He
- grid.411642.40000 0004 0605 3760Department of Pathology, Peking University Third Hospital, Beijing, 100191 China
| | - Ying Yao
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Jinsong Han
- grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China
| | - Jie Qiao
- grid.411642.40000 0004 0605 3760Center for Reproductive Medicine, Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760National Clinical Research Center for Obstetrics and Gynecology, (Peking University Third Hospital), Beijing, 100191 China ,grid.419897.a0000 0004 0369 313XKey Laboratory of Assisted Reproduction (Peking University), Ministry of Education, Beijing, 100191 China ,grid.411642.40000 0004 0605 3760Beijing Key Laboratory of Reproductive Endocrinology and Assisted Reproductive Technology, Beijing, 100191 China
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16
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Serum IgG4 Concentration Is a Potential Predictive Biomarker in Glucocorticoid Treatment for Idiopathic Retroperitoneal Fibrosis. J Clin Med 2022; 11:jcm11123538. [PMID: 35743608 PMCID: PMC9224541 DOI: 10.3390/jcm11123538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2022] [Revised: 06/17/2022] [Accepted: 06/18/2022] [Indexed: 12/04/2022] Open
Abstract
Objectives: To evaluate the management and outcome of idiopathic retroperitoneal fibrosis (iRPF) in Japan, and to identify its clinical biomarker. Methods: We retrospectively analyzed 129 patients with iRPF treated between January 2008 and May 2018 at 12 university and related hospitals. Patients treated with glucocorticoid were analyzed to identify a predictive biomarker. These patients were classified into three groups according to overall effectiveness (no change: NC, complete response: CR, and partial response groups: PR), and each parameter was compared statistically. Results: Male–female ratio was 5:1, and median age at diagnosis was 69 (33–86) years. Smoking history was reported in 59.6% of the patients. As treatment, 95 patients received glucocorticoid therapy with an overall response rate of 84%. As a result, serum concentration of IgG4 was significantly decreased in NC group compared with the other two groups (56.6 mg/dL vs. 255 mg/dL, 206 mg/dL, p = 0.0059 and 0.0078). ROC analysis was performed between the nonresponder (NC) and responder groups (CR + PR) to identify the cut-off value of serum IgG4 as a predictive marker. As a result, AUC of 0.793 was confirmed. Conclusions: Pre-treatment serum IgG4 concentration may have potential as a predictive biomarker of steroid treatment.
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17
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Dattani R, Barwick TD, El Wardany G, Gibbons N, Mason JC, Morgan P, Pusey CD, Tam FWK, Tomlinson JAP. An international patient-centred study of retroperitoneal fibrosis. QJM 2022; 115:148-154. [PMID: 33377941 DOI: 10.1093/qjmed/hcaa327] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/22/2020] [Revised: 11/17/2020] [Indexed: 11/14/2022] Open
Abstract
BACKGROUND The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance. AIM To understand the physical and psychosocial impact that RPF has upon peoples' lives. DESIGN An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences. METHODS An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months. RESULTS A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically. CONCLUSION This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health.
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Affiliation(s)
- R Dattani
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
| | - T D Barwick
- Department of Surgery and Cancer, Imperial College London, London, UK
| | - G El Wardany
- Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK
| | - N Gibbons
- Department of Urology, Imperial College Healthcare NHS Trust, London, UK
| | - J C Mason
- Imperial College London, National Heart and Lung Institute London, London, UK
| | - P Morgan
- Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK
| | - C D Pusey
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
- Imperial College London, Centre for inflammatory Disease London, London, UK
| | - F W K Tam
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
- Imperial College London, Centre for inflammatory Disease London, London, UK
| | - J A P Tomlinson
- West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK
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18
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Adam Z, Dastych M, Čermák A, Doubková M, Skorkovská Š, Pour L, Řehák Z, Koukalová R, Adamová Z, Štork M, Krejčí M, Boichuk I, Král Z. Therapy of immunoglonuline IgG4 related disease (IgG4-RD). VNITRNI LEKARSTVI 2022; 68:15-22. [PMID: 36316207 DOI: 10.36290/vnl.2022.086] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/16/2023]
Abstract
Immunoglobulin IgG4 related disease (IgG4-RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the beginning of this century only. Evolving therapy is reviewed in this paper. Glucocorticoids are first choice drug but long administration of glucocorticoids is connected with many adverse effects. In case of combination glucocorticoids and immunosuppressive agents lower doses of glucocorticoids are needed, the response rate is higher and therapy is better tolerated. Rituximab is drug, that is possible use as monotherapy or in combination with glucocorticoids and immunosuppressive drugs. Only one study compared two immunosuporessive drugs, mycophenolate mofetil and cyclophosphamide. The response rated was similar but remissions were longer after glucocorticoids with cyclophosphamide then glucocorticoids with mycofenolat mofetil. No other comparative study of combination of various imunossupressive drugs with glucocorticoids was published. Rituximab has high number (90 %) of response rate in monotherapy, but can be used in combination with glucocorticoids and immunosuppressives. Rituximab is now preferred and recommended for maintenance therapy administered in 6-month interval. In case of advanced disease, we prefer therefore combination of rituximab, cyclofosphamide and dexamethasone for initial therapy followed by maintenance with rituximab in 6 months interval. There are two new drugs under investigation abatacept and dupilimab with promising results. Although we have very intensive therapies for good results of therapy early diagnosis before irreversible fibrotic changes in IgG4-RD involved organs is still needed.
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19
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Kotsis T, Christoforou P, Polydorou A. The contribution of oncovascular surgery in a young patient with idiopathic retroperitoneal fibrosis. J Surg Case Rep 2022; 2022:rjab589. [PMID: 35047174 PMCID: PMC8763601 DOI: 10.1093/jscr/rjab589] [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: 10/24/2021] [Accepted: 12/03/2021] [Indexed: 11/23/2022] Open
Abstract
Surgical resection remains the cornerstone for the treatment of oncological disease. When a critical arterial or venous structure is involved in a tumor mass, successful relief of symptoms and long-term oncological control are achieved through careful preoperative planning by an interdisciplinary team that necessarily includes a vascular surgeon. We describe the involvement of a vascular surgeon in the oncology of a 22-year-old woman, who is diagnosed with idiopathic retroperitoneal fibrosis.
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Affiliation(s)
- Thomas Kotsis
- Vascular Division, 2nd Clinic of Surgery, National & Kapodistrian University of Athens Medical School, “ARETAIEION” Hospital, Athens, Greece
| | - Panagitsa Christoforou
- Vascular Division, 2nd Clinic of Surgery, National & Kapodistrian University of Athens Medical School, “ARETAIEION” Hospital, Athens, Greece
| | - Andreas Polydorou
- 2nd Clinic of Surgery, National & Kapodistrian University of Athens Medical School, “ARETAIEION” Hospital, Athens, Greece
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20
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Keerthivasagam S, Roy Moulik N, Pandey A, Gala K, Patil V, Dhamne C, Chatterjee G, Patkar N, Narula G, Banavali S. Imatinib-induced retroperitoneal fibrosis in a child with chronic myeloid leukemia: a case report. AMERICAN JOURNAL OF BLOOD RESEARCH 2021; 11:600-604. [PMID: 35103114 PMCID: PMC8784644] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 06/15/2021] [Accepted: 11/17/2021] [Indexed: 06/14/2023]
Abstract
A 12 year old boy with chronic myeloid leukemia (CML) presenting with bilateral pitting pedal edema and abdominal distension after about 41 months of imatinib therapy and was diagnosed to have retroperitoneal fibrosis (RPF) based on imaging and biopsy findings. He was found to have bilateral hydroureteronephrosis needing double-J stenting to the more severely affected right ureter. Imatinib was briefly interrupted and restarted later due to rising transcript levels and unavailability of other alternatives at that time which was later substituted by dasatinib once generic versions became available. Child remains asymptomatic after 18 months of DJ stenting. RPF is a rare complication of imatinib this being the second case reported in the literature.
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Affiliation(s)
| | | | - Ankita Pandey
- Tata Memorial Centre, Homi Bhaba National Instituite Mumbai, India
| | - Kunal Gala
- Tata Memorial Centre, Homi Bhaba National Instituite Mumbai, India
| | - Vasundhara Patil
- Tata Memorial Centre, Homi Bhaba National Instituite Mumbai, India
| | - Chetan Dhamne
- Tata Memorial Centre, Homi Bhaba National Instituite Mumbai, India
| | | | - Nikhil Patkar
- Tata Memorial Centre, Homi Bhaba National Instituite Mumbai, India
| | - Gaurav Narula
- Tata Memorial Centre, Homi Bhaba National Instituite Mumbai, India
| | - Shripad Banavali
- Tata Memorial Centre, Homi Bhaba National Instituite Mumbai, India
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21
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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.
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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
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22
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Wallwork R, Perugino CA, Fu X, Harkness T, Zhang Y, Choi HK, Stone JH, Wallace ZS. The association of smoking with immunoglobulin G4-related disease: a case-control study. Rheumatology (Oxford) 2021; 60:5310-5317. [PMID: 33751033 PMCID: PMC8783539 DOI: 10.1093/rheumatology/keab172] [Citation(s) in RCA: 23] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2020] [Accepted: 02/03/2021] [Indexed: 12/24/2022] Open
Abstract
OBJECTIVE To evaluate the association between cigarette smoking and the odds of IgG4-related disease (IgG4-RD). METHODS We performed a case-control study of patients with IgG4-RD compared in a 1:5 ratio with age-, race- and sex-matched controls. We included cases evaluated at the Massachusetts General Hospital, a hospital within the Mass General Brigham (MGB) System. Controls were identified from the MGB Biobank. Smoking status at the date of IgG4-RD diagnosis or corresponding index date was determined. Conditional logistic regression was used to estimate the association between cigarette smoking and the odds of having IgG4-RD. RESULTS There were 234 IgG4-RD cases and 1170 controls. The mean age (59 years), sex (62% male) and race (75% white) were well balanced. IgG4-RD cases were more likely to be current smokers compared with controls [25 (11%) vs 70 (6%); odds ratio (OR) 1.79 (95% CI 1.08, 2.95)]. This association was strongest among female cases [13 (14%) vs 19 (4%);, OR 3.79 (95% CI 1.71, 8.39)] and those with retroperitoneal fibrosis [RPF; 13 (28%) vs 13 (6%);, OR 6.93 (95% CI 2.78, 17.26)] or normal IgG4 concentrations [21 (21%) vs 21 (4%); OR 6.22 (95% CI 3.09, 12.49)]. When RPF cases were excluded, there was no longer an association between current smoking and the odds of having IgG4-RD [12 (6%) vs 57 (6%); OR 0.95 (95% CI 0.49, 1.86)]. CONCLUSION Being a current smoker is associated with greater odds of having IgG4-RD, especially among women and those with RPF or normal IgG4 concentrations. Current smoking is the first recognized modifiable risk factor for IgG4-RD.
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Affiliation(s)
- Rachel Wallwork
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
| | - Cory A Perugino
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
| | - Xiaoqing Fu
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
- Division of Rheumatology, Allergy, and Immunology, Clinical Epidemiology Program, Massachusetts General Hospital
- Harvard Medical School, Boston, MA, USA
| | - Tyler Harkness
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
| | - Yuqing Zhang
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
- Division of Rheumatology, Allergy, and Immunology, Clinical Epidemiology Program, Massachusetts General Hospital
- Harvard Medical School, Boston, MA, USA
| | - Hyon K Choi
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
- Division of Rheumatology, Allergy, and Immunology, Clinical Epidemiology Program, Massachusetts General Hospital
- Harvard Medical School, Boston, MA, USA
| | - John H Stone
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
- Division of Rheumatology, Allergy, and Immunology, Clinical Epidemiology Program, Massachusetts General Hospital
- Harvard Medical School, Boston, MA, USA
| | - Zachary S Wallace
- Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology
- Division of Rheumatology, Allergy, and Immunology, Clinical Epidemiology Program, Massachusetts General Hospital
- Harvard Medical School, Boston, MA, USA
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23
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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.
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Affiliation(s)
- Ichiro Mizushima
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
| | - Mitsuhiro Kawano
- Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan
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24
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Uddin MM, Varley R, Napier-Hemy RD. Our experience in laparoscopic ureterolysis and omental wrapping as a definitive surgical management of ureteric obstruction related to retroperitoneal fibrosis. JOURNAL OF CLINICAL UROLOGY 2021. [DOI: 10.1177/20514158211022215] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Introduction and Objectives: Retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory disease and a cause of ureteric obstruction that leads to obstructive uropathy. Although the mainstay of RPF treatment is medical, ureterolysis becomes a necessity if medical treatment fails or if patients are unable to tolerate side effects of conservative/medical management, including those of ureteric stents. We aimed to investigate our experience of laparoscopic ureterolysis (LU) and omental wrapping as a definitive surgical treatment for RPF. Patients and methods: We conducted a retrospective analysis of 17 patients who underwent LU between September 2009 and October 2018 in a single institution. Mode of presentation, investigation and interventions were reviewed. Outcome measures were stent or nephrostomy-free rates, post-ureterolysis change in estimated glomerular filtration rate (eGFR), operative time, estimated blood loss, length of hospital stay, complications and need for additional procedures. MAG3 renography was performed to assess ureteral patency. Results: Among 17 patients, a total of 23 LU procedures were performed in 20 operative sessions. Median (IQR) length of hospital stay was 4 (2–6) days. Median length of follow-up was 23.5 months (range 4–92 months), where 14 patients completed at least 15 months of follow-up. All patients were either stent ( n=9) or nephrostomy ( n=8) dependent before ureterolysis. Some 82.4% patients ( n=14) remain stent/nephrostomy free after LU. Renal function remains stable after ureterolysis with a median (IQR) change in eGFR at latest follow-up of: +7.5 (−8 to +23), ( P=0.74). Conclusions: LU is safe and effective at halting decline in renal dysfunction secondary to RPF. It has reliably avoided internal or external ureteric drainage in most patients. Consideration should be given to early referral to a specialised centre for ureterolysis in the context of multi-disciplinary management of benign RPF, to preservation of renal function and to render patients stent or nephrostomy free. Level of Evidence: 4 (case series).
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Affiliation(s)
- MM Uddin
- Department of Urology, Manchester Royal Infirmary, UK
| | - R Varley
- Department of Urology, Manchester Royal Infirmary, UK
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25
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Ali SK, Du K, Orare K. Idiopathic Retroperitoneal Fibrosis: An East African Diagnostic Challenge. Cureus 2021; 13:e15683. [PMID: 34277272 PMCID: PMC8281799 DOI: 10.7759/cureus.15683] [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] [Accepted: 06/15/2021] [Indexed: 11/24/2022] Open
Abstract
Retroperitoneal fibrosis (RPF) is a rare disease with variable etiology characterized by chronic inflammation and fibrosis of tissue surrounding the abdominal aorta and iliac arteries. The majority of cases of RPF remain idiopathic and mostly reported in men. Glucocorticoids remain key in the treatment of RPF. Little has been reported on RPF in sub-Saharan Africa.
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Affiliation(s)
- Sayed K Ali
- Medicine, Aga Khan University Hospital, Nairobi, KEN
| | - Katie Du
- Internal Medicine, Aga Khan University Hospital, Nairobi, KEN
| | - Kelvin Orare
- Medicine, Aga Khan University Hospital, Nairobi, KEN
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26
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Patra S, Padhi S, Padhan P, Kar M, Nayak P, Samal SC. Ergotamine Induced Retroperitoneal Fibrosis. Mediterr J Rheumatol 2021; 32:168-173. [PMID: 34447915 PMCID: PMC8369278 DOI: 10.31138/mjr.32.2.168] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2020] [Revised: 09/13/2020] [Accepted: 09/30/2020] [Indexed: 11/17/2022] Open
Abstract
Retroperitoneal fibrosis (RPF) is an uncommon disease characterised by the presence of fibroinflammatory reaction which starts around the infrarenal portion of the abdominal aorta in the retroperitoneum and frequently entrap the ureter causing obstructive uropathy. Approximately, two thirds of the cases are idiopathic, where aetiopathogenesis is not known. Ergotamine-induced RPF, although rare, is considered under secondary group. The fibrogenic process here is thought to be due to serotonergic activity. We report a case of RPF in a young female with obstructive uropathy who had history of long-term ergotamine intake for migraine. Histopathological evaluation revealed different evolving stages of necrotising vasculitis. In addition, the patient has responded to withdrawal of offending drug along with immunosuppressive therapy. We believe, apart from serotonergic activity, ergotamine can lead to RPF through a vasculitic process which has not been reported earlier.
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Affiliation(s)
- Susama Patra
- Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Somanath Padhi
- Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Prasanta Padhan
- Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneshwar, Odisha, India
| | - Madhabananda Kar
- Department of Surgical Oncology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Prasant Nayak
- Department of Urology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Subash Chandra Samal
- Department of Gastroenterology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
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27
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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.
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Affiliation(s)
- Ellery Altshuler
- Internal Medicine, University of Florida College of Medicine, Gainesville, USA
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28
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Capecchi R, Giannese D, Moriconi D, Bonadio AG, Pratesi F, Croia C, Egidi MF, Puxeddu I, Tavoni AG, Migliorini P. Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight. Front Med (Lausanne) 2021; 8:635706. [PMID: 33869249 PMCID: PMC8044528 DOI: 10.3389/fmed.2021.635706] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2020] [Accepted: 03/10/2021] [Indexed: 12/12/2022] Open
Abstract
IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.
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Affiliation(s)
- Riccardo Capecchi
- Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - Domenico Giannese
- Nephrology, Dialysis and Transplantation Unit, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy
| | - Diego Moriconi
- Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.,Department of Surgical, Medical and Molecular Pathology and Critical Care Medicine, University of Pisa, Pisa, Italy
| | | | - Federico Pratesi
- Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - Cristina Croia
- Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - Maria F Egidi
- Nephrology, Dialysis and Transplantation Unit, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy
| | - Ilaria Puxeddu
- Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - Antonio G Tavoni
- Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
| | - Paola Migliorini
- Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
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29
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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.
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30
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Choi SJ, Oh JS, Hong S, Lee CK, Yoo B, Hong B, Kim YG. Treatment Response to Idiopathic Retroperitoneal Fibrosis-associated Hydronephrosis With a Focus on IgG4/IgG3 Serum Concentration Ratio. JOURNAL OF RHEUMATIC DISEASES 2021; 28:38-44. [PMID: 37476393 PMCID: PMC10324956 DOI: 10.4078/jrd.2021.28.1.38] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2020] [Revised: 10/15/2020] [Accepted: 10/28/2020] [Indexed: 07/22/2023]
Abstract
OBJECTIVE Hydronephrosis, a common complication of idiopathic retroperitoneal fibrosis (iRPF), may lead to poor renal outcomes unless resolved in a timely manner IgG4-related diseases characterized by elevated serum IgG4 levels are responsible for a few iRPF cases However, the underlying immunologic features of most iRPF cases have not been clearly defined, and these cases exhibit varied responses to medical treatment Thus, we investigated the predictive factors for hydronephrosis-associated outcomes among iRPF patients. METHODS We retrospectively included 18 iRPF patients with hydronephrosis in a tertiary referral hospital from 2012 to 2019 Hydronephrosis improvement was assessed on images taken 6 months after diagnosis Categorical variables were compared using chi-square or Fisher's exact test Continuous variables were compared using Mann-Whitney U-test. RESULTS On follow-up images, 8 patients (444%) showed an improvement in hydronephrosis Patients with improvement more frequently had reverse serum IgG4/IgG3 ratio (875% vs 30%, p=0025), abdominal aorta involvement (875% vs 30%, p=0025) and glucocorticoid treatment administration (875% vs 30%, p=0025) than those without improvement The proportion of elevated serum IgG4 level did not differ between the two groups Even in the 14 cases with normal serum IgG4 levels, reverse serum IgG4/IgG3 ratio was more frequently observed in patients with improvement than in those without improvement (833% vs 125%, p=0026). CONCLUSION The reverse serum IgG4/IgG3 ratio was associated with hydronephrosis improvement in iRPF patients, suggesting it to be a suitable serologic marker for predicting favourable responses to glucocorticoid treatment.
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Affiliation(s)
- Su Jin Choi
- Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Ji Seon Oh
- Department of Biomedical Informatics, Asan Medical Center, Seoul, Korea
| | - Seokchan Hong
- Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Chang-Keun Lee
- Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Bin Yoo
- Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Bumsik Hong
- Department of Urology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Yong-Gil Kim
- Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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31
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Abstract
Retroperitoneal fibrosis (RPF) is a rare condition characterized by fibroinflammatory tissue infiltrating and compressing retroperitoneal structures. While mostly idiopathic (idiopathic retroperitoneal fibrosis or IRF), RPF is frequently associated with certain drugs, infections, and malignancies. It is thought to be immune-mediated because of response to steroids and RPF is commonly seen with other autoimmune diseases, especially IgG4-related disease (IgG4-RD). IRF is also a part of the chronic aortitis syndromes and the presence of aortic aneurysms is another characteristic of this disease. A 63-year old woman presented with left-sided flank pain. Computed tomography (CT) scan showed left hydronephrosis from compression of the ureter by a retroperitoneal mass. A thoracoabdominal aneurysm was also noted. A [18F]- fluorodeoxyglucose positron emission tomography (FDG-PET) scan showed hypermetabolism in the mass, with no abnormally increased activity noted elsewhere. Within four months, the mass enlarged to involve the right ureter as well, leading to right hydronephrosis. She required bilateral ureteral stents and aneurysm repair. Biopsy of the mass showed dense fibrosis with a mononuclear cell infiltrate. The histology of the aneurysm specimen showed chronic periaortic inflammation. Laboratory investigations were significant for elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), with no evidence of monoclonal gammopathy. She was referred to the rheumatology clinic to receive steroid treatment for IRF. IRF commonly involves the ureters and is diagnosed on CT scans during a workup for obstructive uropathy. The treatment is high dose steroids, while in resistant cases, other immunosuppressants have been used. The presentation of a patient with IRF can commonly mimic that of urinary calculi and malignancy. While rare, IRF should not be forgotten when evaluating a patient for obstructive uropathy.
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Affiliation(s)
- Radhika Sheth
- Internal Medicine, Henry Ford Health System, Jackson, USA
| | - Devin Malik
- Hematology/Oncology, Henry Ford Health System, Jackson, USA
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32
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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.
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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
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33
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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.
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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
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34
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Le Joncour A, Saadoun D, Cacoub P. La fibrose rétropéritonéale idiopathique. Rev Med Interne 2020; 41:822-828. [PMID: 32727694 DOI: 10.1016/j.revmed.2020.06.013] [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: 03/25/2020] [Revised: 05/27/2020] [Accepted: 06/20/2020] [Indexed: 11/27/2022]
Abstract
Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibro-inflammatory tissue around the aorta entrapping the adjacent structures. RPF can be idiopathic or secondary to many disorders. The physiopathology is unknown but can be part of the spectrum of IgG4 related diseases. Imaging studies and inflammatory markers are essential for initial evaluation and follow-up. Biopsy is usually not recommended. The first line of treatment is corticosteroids associated or not with immunosuppressive drugs. In case of ureteral obstruction with renal failure, ureteral stent placement or nephrostomies are recommended. Initial response to treatment is usually good but relapses are frequent.
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Affiliation(s)
- A Le Joncour
- Department of Internal Medicine and Clinical Immunology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France; Sorbonne Université, UPMC Université Paris 06, UMR 7211, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005 Paris, France.
| | - D Saadoun
- Department of Internal Medicine and Clinical Immunology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France; Sorbonne Université, UPMC Université Paris 06, UMR 7211, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005 Paris, France
| | - P Cacoub
- Department of Internal Medicine and Clinical Immunology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France; Sorbonne Université, UPMC Université Paris 06, UMR 7211, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005 Paris, France
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35
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Moriconi D, Giannese D, Capecchi R, Cupisti A, Barsotti S, Morganti R, Orsitto E, Gaetano Tavoni A, Francesca Egidi M. Risk factors for relapse and long-term outcome of idiopathic retroperitoneal fibrosis. Clin Exp Nephrol 2019; 23:1147-1153. [PMID: 31230189 DOI: 10.1007/s10157-019-01759-w] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2019] [Accepted: 05/20/2019] [Indexed: 12/11/2022]
Abstract
BACKGROUND Retroperitoneal fibrosis (RF) is a rare disease of unclear etiology characterized by the presence of fibroinflammatory tissue in the retroperitoneal space, which can entrap and obstruct retroperitoneal structures, notably the ureters. The disease responds well to steroid therapy, but tends to recur even after years. The aim of our study was to evaluate the long-term renal outcome of patients affected by idiopathic retroperitoneal fibrosis looking for predictive risk factors for recurrence of the disease and progression to end-stage renal disease. METHODS Retrospective observational study of patients with idiopathic RF diagnosed from 2004 to 2017 and follow-up of at least 1 year after the end of first course therapy with steroid, with or without tamoxifen (TMX) and with urological procedures when applicable. RESULTS Forty-three patients were included in the study. The follow-up was 93 ± 52 months. All the patients obtained remission after therapy that was maintained until the last observation in 26 of them. In 17 patients, there was at least one recurrence. Risk factors associated with relapse were identified and resulted in smoking habit, onset with acute kidney injury (AKI), low back pain and antinuclear antibodies (ANA) positivity. Renal function remained fairly stable during the long-term follow-up. The renal end-point (doubling of serum creatinine or ESRD) occurred in 8% of the patients; however, eGFR in patients with relapse was similar to that of non-recurrent at the diagnoses, but it decreased over time more in the relapsing than in non-relapsing patients (p group = 0.20; p time = 0.001; p time × group interactions = 0.04). Based on these 4 predictor conditions, patients were divided into "low risk" (with 0-1 risk factor), and "high risk" (3-4 risk factors). The renal end-point occurred in 40% of high-risk patients, while none of the low-risk patients reached it (p = 0.02). CONCLUSIONS Smoking habit, AKI at diagnosis, ANA positivity and lumbar pain were associated with relapse of RF after initial remission due to steroid and/or TMX therapy; the combination of these conditions was also predictive of worse renal function outcome. Identification of risk factors for relapse can be useful not only to modulate the choice, the dosage of first-line treatment and the duration of maintenance therapy but also for preventing a progressive loss of kidney function, as well.
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Affiliation(s)
- Diego Moriconi
- Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy.
| | | | - Riccardo Capecchi
- Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy
| | - Adamasco Cupisti
- Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy
| | - Simone Barsotti
- Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy
| | | | - Eugenio Orsitto
- Radiology Unit, Emergency Department, AOUP Pisa, Pisa, Italy
| | - Antonio Gaetano Tavoni
- Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126, Pisa, Italy
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36
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CT-Guided Percutaneous Needle Biopsy in Patients with Suspected Retroperitoneal Fibrosis: A Retrospective Cohort Study. Cardiovasc Intervent Radiol 2019; 42:1434-1440. [PMID: 31292673 DOI: 10.1007/s00270-019-02266-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2019] [Accepted: 06/08/2019] [Indexed: 10/26/2022]
Abstract
PURPOSE The outcome of CT-guided biopsy in patients with suspected retroperitoneal fibrosis (RF), regarded as technically challenging, remains unclear. This study aimed to evaluate the results of CT-guided biopsy in patients with lesions considered in the differential diagnosis of RF and compare them with results from patients with other retroperitoneal lesions. MATERIALS AND METHODS Patients who underwent CT-guided biopsy of retroperitoneal lesions between January 2010 and September 2018 were retrospectively reviewed. The study cohort with retroperitoneal lesions surrounding the infra-abdominal aorta, iliac vessels, and/or ureters was divided into two groups: Group F included patients with lesions for which RF was considered in the differential diagnosis, and Group C comprised patients with a retroperitoneal mass or lymphadenopathy. Lesion size and depth, and biopsy details including technical success, position, procedure time, diagnostic yield, and complications between the two groups were compared. RESULTS Group F included 27 patients (mean age 68.5 years ± 11.6, 17 male) and Group C 30 patients (mean age 65.0 years ± 11.8, 16 male). The short axis was significantly smaller in Group F than in Group C (19 mm vs 26 mm, P = 0.041), and procedure time was significantly longer in Group F than in Group C (31.5 min ± 13.2 vs 20.3 min ± 8.4, P = 0.001). Technical success rate (93% vs 100%), accuracy (93% vs 93%), and complications (3.7% vs 10%) between Groups F and C were not significantly different. CONCLUSIONS CT-guided biopsy of patients with suspected RF is considered safe and effective.
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37
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Raffiotta F, da Silva Escoli R, Quaglini S, Rognoni C, Sacchi L, Binda V, Messa P, Moroni G. Idiopathic Retroperitoneal Fibrosis: Long-term Risk and Predictors of Relapse. Am J Kidney Dis 2019; 74:742-750. [PMID: 31204195 DOI: 10.1053/j.ajkd.2019.04.020] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2018] [Accepted: 04/15/2019] [Indexed: 11/11/2022]
Abstract
RATIONALE & OBJECTIVE Idiopathic retroperitoneal fibrosis (IRF) is a rare disorder of unknown cause. Medical therapy can induce remission, but disease relapses are common. This study sought to characterize long-term outcomes of IRF and the factors associated with disease recurrences. STUDY DESIGN Retrospective cohort study. SETTING & PARTICIPANTS Retrospective analysis of 50 patients with IRF prospectively followed up for 8.9 (IQR, 4.7-12.7) years at a tertiary-care referral center. EXPOSURES Demographic, clinical, treatment, and laboratory parameters, including measures of autoimmunity. OUTCOME Disease relapse. ANALYTICAL APPROACH Proportional hazards analysis for the subdistribution of competing risks. RESULTS 49 patients received medical treatment and 35 underwent interventional procedures. All patients experienced a clinical response (defined as regression of disease-related symptoms and hydronephrosis, and decrease in the maximal transverse diameter of the retroperitoneal mass on computed tomography of >50%), 44 of whom responded within 1 year. The remaining 6 responded over a median of 2.95 years after starting therapy. 40 patients were alive at last observation, 1 receiving maintenance dialysis and 15 with estimated glomerular filtration rate < 60mL/min/1.73m2. Patient survival at 5, 10, and 15 years was 95%, 84%, and 68%, respectively. 19 (38%) patients had at least 1 relapse (occurring a median of 5.19 years after starting therapy), defined as an increase in serum creatinine level of at least 30% or recurrence/development of hydronephrosis and ≥20% increase in the maximal transverse diameter of the retroperitoneal mass on computed tomography. Cumulative incidences of relapse at 5, 10, and 15 years were 21%, 41%, and 48%, respectively. Baseline antinuclear antibody positivity and male sex were associated with relapse (subdistribution hazard ratios [sHRs] of 5.35 [95% CI, 2.15-13.27] and 4.94 [95% CI, 1.32-18.57], respectively), while higher corticosteroid therapy dosage at 1 year (sHR for relapse per 1-mg/d greater dosage, 0.91 [95% CI, 0.84-0.98]) and treatment with prednisone alone or with tamoxifen (sHR for relapse of 0.25 [95% CI, 0.07-0.85] vs other therapies) were associated with lower rate of relapse. LIMITATIONS Small sample size and variable approaches to therapy. CONCLUSIONS IRF relapses were common and were experienced more frequently by male patients. Corticosteroids alone or with tamoxifen were associated with a lower rate of relapse. The strong association of antinuclear antibody positivity with relapse supports the hypothesis of an autoimmune pathogenesis of IRF.
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Affiliation(s)
- Francesca Raffiotta
- Nephrology Unit, Fondazione Ca'Grande Ospedale Maggiore Policlinico, Milano, Italy
| | | | - Silvana Quaglini
- Department of Electrical, Computer and Biomedical Engineering, University of Pavia, Pavia, Italy
| | - Carla Rognoni
- Centre for Research on Health and Social Care Management (CERGAS), SDA Bocconi School of Management, Bocconi University, Milano, Italy
| | - Lucia Sacchi
- Department of Electrical, Computer and Biomedical Engineering, University of Pavia, Pavia, Italy
| | - Valentina Binda
- Nephrology Unit, Fondazione Ca'Grande Ospedale Maggiore Policlinico, Milano, Italy
| | - Piergiorgio Messa
- Nephrology Unit, Fondazione Ca'Grande Ospedale Maggiore Policlinico, Milano, Italy
| | - Gabriella Moroni
- Nephrology Unit, Fondazione Ca'Grande Ospedale Maggiore Policlinico, Milano, Italy.
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Choi YK, Yang JH, Ahn SY, Ko GJ, Oh SW, Kim MG, Cho WY, Jo SK. Retroperitoneal fibrosis in the era of immunoglobulin G4-related disease. Kidney Res Clin Pract 2019; 38:42-48. [PMID: 30754935 PMCID: PMC6481977 DOI: 10.23876/j.krcp.18.0052] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/05/2018] [Revised: 09/10/2018] [Accepted: 10/24/2018] [Indexed: 12/16/2022] Open
Abstract
Background Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue in the periaortic or periiliac retroperitoneum, where it frequently encases ureters. There is emerging evidence that a subset of this disease is part of a spectrum of multisystemic autoimmune diseases collectively referred to as “immunoglobulin G4 (IgG4)-related disease”. Methods We retrospectively analyzed 27 idiopathic RPF patients and identified a subset as IgG4-related RPF, which we categorized according to recently published comprehensive diagnostic criteria. We compared clinical and laboratory characteristics and response to treatment between the two groups. Results Of 27 total patients, 16 (59.3%) were diagnosed as having IgG4-related RPF, and these were predominantly male. They were also significantly older and more likely to have other organ involvement, hydronephrosis, and postrenal acute kidney injury (AKI) compared to those with idiopathic RPF. However, there was no difference in response rate to systemic steroid treatment. Conclusion IgG4-related RPF accounts for a substantial portion of RPF cases previously identified as “idiopathic RPF” in Korea. Clinical and laboratory characteristics of IgG4-related RPF are similar to those of idiopathic RPF except for a striking male predominance, older age, and higher incidence of postrenal AKI in IgG4-related RPF. More comprehensive, prospective studies are needed to clearly distinguish IgG4-related RPF from idiopathic RPF based on clinical manifestation and to further assess treatment response and long-term prognosis.
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Affiliation(s)
- Yoon Kyung Choi
- Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
| | - Ji Hyun Yang
- Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
| | - Shin Young Ahn
- Department of Internal Medicine, Korea University Guro Hospital, Seoul, Korea
| | - Gang Jee Ko
- Department of Internal Medicine, Korea University Guro Hospital, Seoul, Korea
| | - Se Won Oh
- Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
| | - Myung Gyu Kim
- Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
| | - Won Yong Cho
- Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
| | - Sang Kyung Jo
- Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
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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.
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Soriano Rios A, Paredes H, Hernández-Calleros J, Uscanga-Domínguez L, Peláez-Luna M. Retroperitoneal fibrosis. Steroid treatment response seems to depend on its association to IgG4 related disease. Med Hypotheses 2019; 122:120-123. [PMID: 30593393 DOI: 10.1016/j.mehy.2018.11.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2018] [Revised: 10/06/2018] [Accepted: 11/14/2018] [Indexed: 02/04/2023]
Abstract
Retroperitoneal fibrosis (RF) is part of a rare fibrosclerotic disorder. Oral steroids are the initial treatment. Steroid combination with other immunosupressants is used in refractory cases. Steroids refractoriness has been observed in chronic cases. Some cases of RF represent a manifestation of the IgG4 related disease (IgG4-RD) that is associated to a dramatic response to steroid therapy. It is uncertain if RF́s treatment response differs according to its association with IgG4-RD. We hypothesize that RF́s treatment response to steroids depends on the association with IgG4-RD, thus, we collected and compared clinical data from 10 RF cases; 6 male, mean age 50.6 (±16.15 SD) years. Mean FU was 28 (±25.7 SD) months. According to IgG4 levels, patients were categorized as idiopathic RF (IRF n = 5) or RF-IgG4-RD (n = 5). Therapy response was categorized as complete, partial, stable disease, recurrence or non-response. Nine cases received initial therapy with prednisone; complete response was achieved in 4 RF-IgG4 RD. The remaining 5 cases (1 RF-IgG4RD and 4 IRF) underwent a 2nd line therapy; 4 prednisone + tamoxifen and 1 prednisone + azathioprine. Prednisone + tamoxifen combination achieved complete response in 1 case (RF-IgG4RD), partial response in 1 IRF; in 1 IRF case, disease remained stable and 1 did not respond. The prednisone + azathioprine treatment achieved complete response. At follow-up all patients remained stable and no recurrence was registered. These observations suggest and support the hypothesis that response to steroid monotherapy depends on the association of RF with IgG4, suggesting that IRF cases might benefit from initial combination therapies instead of steroid monotherapy.
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Affiliation(s)
- Andrea Soriano Rios
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico
| | - Humberto Paredes
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico
| | - Jorge Hernández-Calleros
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico
| | - Luis Uscanga-Domínguez
- Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico
| | - Mario Peláez-Luna
- Research Division-School of Medicine Universidad Nacional Autónoma de Méxcio, Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico.
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Htet Z, Karim M. Retroperitoneal fibrosis: a rare disease for frontline clinicians. J R Coll Physicians Edinb 2019; 49:125-127. [DOI: 10.4997/jrcpe.2019.208] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
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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.
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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
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Adnan S, Bouraoui A, Mehta S, Banerjee S, Jain S, Dasgupta B. Retroperitoneal fibrosis; a single-centre case experience with literature review. Rheumatol Adv Pract 2018; 3:rky050. [PMID: 31431986 PMCID: PMC6649902 DOI: 10.1093/rap/rky050] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2018] [Revised: 11/28/2018] [Indexed: 12/24/2022] Open
Abstract
Objective We present 13 patients with retroperitoneal fibrosis, focusing on clinical features, radiological characteristics, treatments and their outcomes. Methods Retrospective review of the medical records was performed of all retroperitoneal fibrosis patients diagnosed and treated in our department between 2012 and 2017. Results Twelve patients were male, with a median age of 64 years. Eleven patients presented with abdominal pain or back pain or both. Aetiologies varied from idiopathic to malignancy and vasculitis. Twelve patients had PET scans. These showed 18F-fluorodeoxyglucose-avid retroperitoneal soft tissue around the abdominal aorta in the vast majority, with five scans also demonstrating localized or generalized uptake by the aorta. In all cases except one, glucocorticoids were applied as the first-line therapy. Further immunosuppressive therapy was required in 10 cases. Conclusion Our patients were male and older in age compared with the existing literature. PET scans were very helpful in diagnosis of retroperitoneal fibrosis. Rituximab was found to be an effective treatment in six of our patients.
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Affiliation(s)
| | | | - Sampi Mehta
- Urology Department, Southend University Hospital, Westcliff-on-sea, Essex
| | - Siwalik Banerjee
- Rheumatology Department, University Hospital Coventry and Warwickshire, Coventry
| | - Shaifali Jain
- Radiology Department, Southend University Hospital, Westcliff-on-sea, Essex, UK
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Sadasivan G, Arul R, Prabaharan A. An Unusual Presentation of Prostate Carcinoma. Indian J Med Paediatr Oncol 2018. [DOI: 10.4103/ijmpo.ijmpo_37_17] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
AbstractProstate carcinoma is the second most common cancer among men worldwide. Although prostate carcinoma is common, its presentation resembling retroperitoneal fibrosis is uncommon. We report a patient with prostate carcinoma mimicking retroperitoneal fibrosis. An elderly male presenting in a volume overload state with features of obstructive uropathy was diagnosed as a case of prostate carcinoma. Magnetic resonance imaging was suggestive of retroperitoneal fibrosis. The presentation of prostate carcinoma as retroperitoneal fibrosis is rare.
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Affiliation(s)
- Greeshma Sadasivan
- Departments of General Medicine, Coimbatore Medical College Hospital, Coimbatore, Tamil Nadu, India
| | - R Arul
- Departments of Nephrology, Coimbatore Medical College Hospital, Coimbatore, Tamil Nadu, India
| | - A Prabaharan
- Departments of Nephrology, Coimbatore Medical College Hospital, Coimbatore, Tamil Nadu, India
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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.
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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
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Cohan RH, Shampain KL, Francis IR, Davenport MS, Stuart Wolf J, Marder W, Swartz RD. Imaging appearance of fibrosing diseases of the retroperitoneum: can a definitive diagnosis be made? Abdom Radiol (NY) 2018; 43:1204-1214. [PMID: 28849414 DOI: 10.1007/s00261-017-1282-5] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
PURPOSE To assess the frequency with which previously reported characteristic findings of retroperitoneal fibrosis (RPF) (a circumferential or almost circumferential peri-aortic mass centered at L4, which does not displace the abdominal aorta or proximal common iliac arteries) are present in patients with RPF, in patients with other fibrosing diseases, and in cancer patients referred to a subspecialty clinic with a suspected diagnosis of RPF, in order to determine whether diagnostic percutaneous biopsy can be avoided in some patients. METHODS This HIPAA-compliant Institutional Review Board-approved retrospective study assessed clinical and CT and MR imaging abnormalities on imaging studies in 92 patients referred to a subspecialty clinic with suspected RPF over a 14-year period. Two reviewers, in consensus, determined the frequency of different CT and MRI findings in three groups of patients (Group 1: those with an eventual diagnosis of RPF, Group 2: those with a fibrosing disease associated with vascular or urologic abnormalities, and Group 3: those with cancer). Assessed imaging features included the presence of retroperitoneal masses, whether masses were single or multiple, whether such masses were circumferential or nearly circumferential, whether they displaced the aorta away from the spine (with the degree of such displacement measured), and whether there were abnormalities outside of the peri-aortic region of the retroperitoneum. The frequency with which findings previously reported as characteristic of RPF were present was determined for each of the three groups. Imaging results were correlated with the final diagnoses. RESULTS Of 68 subjects eventually diagnosed with retroperitoneal fibrosis (RPF) (Group 1), 47 had peri-aortic retroperitoneal masses, 18 of which displaced the aorta anteriorly away from the spine. Of 12 subjects with fibrosing abnormalities related to vascular or urologic disease (Group 2), six had retroperitoneal masses, none of which displaced the aorta away from the spine. Of 12 subjects with malignancies (Group 3), six had peri-aortic retroperitoneal masses only two of whom had aortic displacement. Only 34 of 68 Group 1 subjects had peri-aortic masses characteristic of RPF, compared with six Group 2 subjects and one Group 3 subject. Subjects with characteristic retroperitoneal masses were significantly more likely to have benign disease than cancer (p = 0.009). CONCLUSION Many patients with RPF do not have characteristic imaging findings. Contrary to prior publications, absence of aortic displacement is not seen in all patients with RPF and is seen in some cancer patients. Nonetheless, when infiltrative peri-aortic retroperitoneal soft tissue that does not displace the aorta is encountered on CT or MRI, RPF can be diagnosed with a high degree of confidence, obviating the need for biopsy.
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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.
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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
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Adimonye A, Parwaiz I, Cotton A. Unilateral renal forniceal rupture - A rare presentation of retroperitoneal fibrosis. Urol Case Rep 2018; 17:36-38. [PMID: 29321975 PMCID: PMC5752213 DOI: 10.1016/j.eucr.2017.11.014] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2017] [Revised: 10/18/2017] [Accepted: 11/17/2017] [Indexed: 11/29/2022] Open
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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.
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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
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