Cyclophosphamide is a highly effective and safe induction therapy in chronic periaortitis: a long-term follow-up of 35 patients with chronic periaortitis.

摘要

Chronic periaortitis is a fibro-inflammatory disease of unknown aetiology with histopathological and laboratory findings suggesting an IgG_4-related sclerosing disease in a subgroup of patients. Due to its inflammatory character and associated autoimmune phenomena, different immunosuppressive regimens have been suggested for treatment.In this study, we retrospectively examined the response to immunosuppressive treatment of 35 patients with chronic periaortitis applying the same study design as before. Imaging studies were performed and serum IgG_4 levels were measured both at the time of diagnosis and during follow-up. Retroperitoneal tissue was examined histologically in four patients. Emphasis has been placed on the evaluation of cyclophosphamide in the induction therapy and the hitherto unknown value of serum IgG_4 measurements in disease monitoring.