Original InvestigationPathogenesis and Treatment of Kidney DiseaseTreatment of Idiopathic Retroperitoneal Fibrosis With Colchicine and Steroids: A Case Series
Section snippets
Methods
All patients with a diagnosis of IRPF at discharge in the 14 years from October 1993 to August 2005 and treated by 1 of the authors were included. Pathological diagnosis was obtained in all cases. Six men and 1 woman were enrolled, and age varied from 35 to 83 years (median, 64 years). One patient had another fibrotic disease (Dupuytren disease), and no patient's history was consistent with the diagnosis of secondary or drug-related retroperitoneal fibrosis. Relevant information between the
Results
Comorbid conditions, symptoms, signs and laboratory determinations at the time of diagnosis of IRPF are listed in Table 1.
CT scans showed hydronephrosis (71%); calcified aortic atheromatosis (71%); dense tissue surrounding the abdominal aorta (86%), iliac arteries (100%), renal arteries (57%), and inferior cava vein (43%); and a partially thrombosed aortic aneurism (29%; Table 2).
IRFP was clinically suspected in 4 patients, and in 3 patients, the diagnosis became evident during a laparotomy
Discussion
IRPF, also called Ormond disease, has been known since 1905 after its first description by Albarran.3 Currently, IRPF is considered a variation of “chronic periaortitis,” an entity that includes abdominal aortic inflammatory aneurysm and perianeurysmal fibrosis.1, 24 The pathogenesis of the disease is unknown. Given the frequent association with advanced atherosclerosis, 1 theory suggested that a local inflammatory reaction may develop by means of B- and T-lymphocyte activation by antigens from
Acknowledgements
The authors thank Manuel Kukuljan, MD, PhD, and Leopoldo Ardiles, MD, for assistance in the revision of the manuscript.
Support: None.
Financial Disclosure: None.
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Extensive primary retroperitoneal fibrosis (Ormond's disease) with common bile duct and ureteral obstruction: A rare case report
2015, International Journal of Surgery Case ReportsCitation Excerpt :Retroperitoneal fibrosis (RPF) is a rare inflammatory fibrotic process causing compression of retroperitoneal structures particularly the ureters [1]. Idiopathic RPF (Ormond’s disease) may involve the perirenal tissue, mesentery and biliary system in extremely uncommon situations [2]. In this case report, we describe an uncommon presentation of retroperitoneal fibrosis presenting with a malignant-appearing common bile duct stricture.
Diagnostic Pathology: Soft Tissue Tumors
2015, Diagnostic Pathology: Soft Tissue TumorsRetroperitoneal Fibrosis
2013, Rheumatic Disease Clinics of North AmericaCitation Excerpt :Historically, treatment has focused on relieving the obstruction with percutaneous or cystoscopic assisted placement of ureteral stents followed by more definitive resolution of ureteric obstruction with open or laparoscopic ureterolysis, with or without omental wrapping. Over the past several years, case reports and small series have documented successful, nonsurgical management with the use of various immunosuppressive agents including prednisone, cyclosporine, methotrexate, azathioprine, cyclophosphamide, mycophenolate mofetil (MMF), infliximab, rituximab, colchicine, and the selective estrogen receptor antagonist tamoxifen.1–10 Management therefore has shifted from primarily a surgical approach to a therapy aimed at modulation of the immune system.
Aortitis and periaortitis in ankylosing spondylitis
2011, Joint Bone SpineCitation Excerpt :In RPF patients, prednisone was administered at the dose of 60 mg daily for 6 weeks or every other day for 2 months with a following slow tapering to 10 and 5 mg/daily, respectively [53,54]. Other molecules have been tried, usually with good results, as CS-sparing agents: tamoxifen [55,56], cyclophosphamide or azathioprine [57], mycophenolate mofetil [58,59], colchicine [60], and, in very few cases, methotrexate and cyclosporine. CP can frequently relapse, even several years after a treatment-induced remission.
Aortitis and periaortitis in ankylosing spondylitis
2011, Revue du Rhumatisme (Edition Francaise)Retroperitoneal Fibrosis Is Still an Underdiagnosed Entity with Poor Prognosis
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Originally published online as doi:10.1053/j.ajkd.2008.09.025 on January 5, 2009.