Wegener’s granulomatosis and retroperitoneal fibrosis: a case report and review of the literature

Abstract

Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis (WG), is a rare systemic auto-inflammatory disease characterized by necrotizing granulomatous inflammation and antineutrophil cytoplasmic antibodies-associated small vessel vasculitis. Retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease of unclear etiology, characterized by a chronic non-specific inflammation of the retroperitoneum, which can entrap and obstruct retroperitoneal structures, notably abdominal aorta, iliac arteries and ureters. RPF is mostly idiopathic, but an association with vasculitis has been shown. However, only few cases of retroperitoneal fibrosis with ureterohydronephrosis secondary to GPA have been described in literature. The outcome seems to be better when compared to the idiopathic form, and immunosuppressive treatment demonstrated to be effective, with low need in surgery. We provide the case report of a 57-year-old patient, affected by GPA, referred to our Division for massive vein thrombosis and ureteral stenosis secondary to retroperitoneal fibrosis. We also provide a review of the literature currently available on this topic.