Combination of Steroids and Azathioprine in the Treatment of Ormond’s Disease – A Single Centre Retrospective Analysis

Abstract

We present a retrospective analysis of patients treated in our Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, during 1997-2013 for Ormond's disease. We analyse the clinical history, diagnostic approaches, surgical, and immunosuppressive therapies and their subsequent effect on our patients. 28 patients treated for Ormond's disease were included. Patients with established disease activity (26 patients) were given immunosuppressive treatment, using corticosteroids in combination with azathioprine. Treatment response was evaluated using clinical symptomatology, inflammatory parameters and imaging methods. In the cohort as a whole, immunosuppressive therapy was applied in 26 patients; in two patients it was not used as no inflammatory activity was found with the disease. In all 26 patients, computed tomography showed that immunosuppressive treatment resulted in partial or complete regression of inflammatory infiltrate. Out of the total number of 26 patients, two patients experienced disease exacerbation 7 and 16 months after the immunosuppressive treatment was discontinued. The longest follow-up period was 16 years; the shortest one was 21 months. Idiopathic retroperitoneal fibrosis--Ormond's disease--is a disease with serious complications. Standard treatment involves a combination of surgery and immunosuppressive treatment. The combination of corticosteroids and azathioprine represents a potentially safe and useful method of treatment.

IgG4-related Diseases – A Rare Polycystic Form of Ormond’s Disease

“…The absence of inflammatory activity at the time of surgery is another interesting feature. It confirms our experience that there are patients in whom the disease is not clinically active (Průcha et al, 2016). For practical reasons, it is important to mention that the disease responds well to systemic corticosteroid therapy.…”

Retroperitoneal fibrosis – a report of five cases

“…E.g., promising results were presented by Marcolongo et al [ 14 ]: the combination of prednisone and AZA proved to be effective as first-line therapy and safer than the combination of prednisone and cyclophosphamide. In a retrospective analysis performed by Průcha et al [ 15 ], AZA with CS were applied in 26 patients with RPF: clinical and radiological improvement was observed in all cases; however, two patients experienced disease exacerbation after treatment discontinuation. AZA has also been successfully used for remission maintenance [ 16 ].…”

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

“…Other immunosuppressants reported as a valuable option for treatment of IRPF include methotrexate, 56 cyclophosphamide, 57 azathioprine, 58 and mycophenolate mofetil 59 although clinical evidence of these agents from controlled trial is lacking. In addition, refractory cases treated with biological agents such as rituximab 60 and tocilizumab 61 have been reported in case reports or case series, suggesting their efficacy.…”