Session Title: Miscellaneous Rheumatic and Inflammatory Diseases - Poster I
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Ormond’s disease (idiopathic retroperitoneal fibrosis ,IRF) is a rare disease. Main clinical symptom is compression of the ureters leading to renal failure. A majority of patients is believed to have IgG4-related disease causing the symptoms. Until today no therapy is approved for the treatment of IRF. In our center cyclophosphamide bolus therapy over 6 months is used in treating these patients.
Methods: Patient data of the last 15 years from our center were evaluated concerning, clinical, serological and radiological response to cyclophosphamide therapy. Patients were differentiated according to the diagnosis of IgG4RD in available biopsies.
Results: In a total of 20 patients 7 patients could be diagnosed with having IgG4RD according to the biopsy, 4 were negative and 1 biopsy remained unclear. For 8 patients no biopsy was available.
Conclusion: In about 50% of the patients a significant reduction of the retroperitoneal mass in IRF could be observed under treatment with cyclophopshamide bolus therapy, without improvement of renal function in this time frame. IgG4RD negative patients might profit less from the therapy with cyclophosphamide. Prospective studies in larger cohorts and with a longer follow-up are need to get a more accurate idea about the efficacy in IRF.
To cite this abstract in AMA style:Hoyer BF, Kumpert C, Makowski M, Rudolph B, Schneider U, Burmester GR, Hiepe F. Efficacy of Cyclophosphamide Therapy in Idiopathic Retroperitoneal Fibrosis in a Retrospective Monocentric Analysis [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/efficacy-of-cyclophosphamide-therapy-in-idiopathic-retroperitoneal-fibrosis-in-a-retrospective-monocentric-analysis/. Accessed May 21, 2019.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-of-cyclophosphamide-therapy-in-idiopathic-retroperitoneal-fibrosis-in-a-retrospective-monocentric-analysis/