Ormond's Disease - 26 Years of Experience at One Centre.

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.