title: 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.
authors.affiliation: Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, Prague, Czech Republic. miroslav.prucha@homolka.cz.
authors.forename: Miroslav
authors.initials: M
authors.lastname: Průcha
authors.affiliation: Department of Urology, Third Faculty of Medicine, Charles University in Prague and University Hospital Královské Vinohrady, Prague, Czech Republic.
authors.forename: Ivan
authors.initials: I
authors.lastname: Kolombo
authors.affiliation: Department of Vascular Surgery, Na Homolce Hospital, Prague, Czech Republic.
authors.forename: Petr
authors.initials: P
authors.lastname: Štádler
mesh_terms: D001379:Azathioprine / Q000627:therapeutic use*; D018153:Czech Republic; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation / Q000188:drug therapy / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D062072:Patient Acuity; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D012187:Retroperitoneal Space / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D001379:Azathioprine
keywords: Hydronephrosis; Immunosuppression; Ormond’s disease; Periaortitis; Surgical treatment