scholarly journals Esophageal Involvement in Wegener’s Granulomatosis: A Case Report and Review of the Literature

2000 ◽  
Vol 14 (5) ◽  
pp. 449-451 ◽  
Author(s):  
Glen A Fallows ◽  
Sean F Hamilton ◽  
Douglas S Taylor ◽  
S Bharati Reddy

Wegener’s granulomatosis is characterized by a granulomatous arteritis involving the upper and lower respiratory tracts, progressive glomerulonephritis and systemic symptoms attributable to small vessel vasculitis. Although multisystemic manifestations are frequent, involvement of the gastrointestinal tract is uncommon. Cases have been reported of intestinal perforation, ulceration and hemorrhage. A patient whose initial presentation of Wegener’s granulomatosis was odynophagia secondary to esophageal vasculitis is described. Endoscopy revealed multiple punched out ulcerations in the esophagus, which resolved with standard therapy for systemic Wegener’s granulomatosis. There are only two previous reports of symptomatic esophageal vasculitis in patients with Wegener’s granulomatosis. These reports illustrate the need to consider odynophagia as a reflection of disease activity as opposed to complications of immunosuppressive therapy.

2019 ◽  
Vol 13 (2) ◽  
pp. 109-115
Author(s):  
Lia Salvati ◽  
Valeria Ludovici ◽  
Giuliana Properzi ◽  
Laura Natali ◽  
Angelo Viscido ◽  
...  

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.


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