ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 245

Efficacy of Cyclophosphamide Therapy in Idiopathic Retroperitoneal Fibrosis in a Retrospective Monocentric Analysis

Bimba F. Hoyer1, Caroline Kumpert2, Marcus Makowski3, Birgit Rudolph4, U. Schneider5, Gerd R. Burmester6 and Falk Hiepe7, 1Charité University Medicine, Department of Medicine/Rheumatology and Clinical Immunology and German Rheumatism Research Centre Berlin (DRFZ), Berlin, Germany, 2department of Rheumatology and clinical Immunology, Charité University Medicine Berlin,, Berlin, Germany, 3Department for Radiology, Charité Universityhospital, Berlin, Germany, 4Department for pathology, Charité Universityhospital, Berlin, Germany, 5Rheumatology and Clinical Immunology, Charité, Rheumatology and Clinical Immunology, Berlin, Germany, 6Charité – University Medicine Berlin, Berlin, Germany, 7Charité – Universitätsmedizin, Berlin, Germany

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords:

Session Information

Date: Sunday, November 13, 2016

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.  Only few data is available on treatment approaches for IRF and even less differentiating between IgG4RD and non-IgG4-RD IRF

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.  IgG4 negative patients had a significant higher volume of the retroperitoneal mass than positive and unclear patients at the time of diagnosis. The volume of the retroperitoneal mass was significantly reduced under therapy only in the group of IgG4RD positive and unclear patients (P=0.003 and 0.0039). Renal function (creatinine) was impaired in all three groups initially and no significant improvement could be observed in any of the groups under therapy. Unfortunately, IgG4 serum levels were only available for very few patients therefore follow up was impossible.

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.  

Disclosure: B. F. Hoyer, None; C. Kumpert, None; M. Makowski, None; B. Rudolph, None; U. Schneider, None; G. R. Burmester, AbbVie, Bristol-Myers Squibb, MedImmune, Merck, Pfizer, Roche, and UCB, 2,AbbVie, Bristol-Myers Squibb, MedImmune, Merck, Pfizer, Roche, and UCB, 5,AbbVie, Bristol-Myers Squibb, Merck, Pfizer, Roche, and UCB, 8; F. Hiepe, None.

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 .

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