A Rare Presentation of Gastrointestinal Hemorrhage in Patient with Granulomatosis with Polyangitis

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Introduction: Granulomatosis with polyangitis (GPA also known as Wegener's granulomatosis) is characterized by a necrotizing granulomatous vasculitis. It is clinically characterized by the triad of upper airway disease, lower respiratory tract disease and glomerulonephritis. Gastrointestinal (GI) manifestations of GPA are rare and can include ischemia, bowel infarction, perforations, and aneurysmal ruptures. GI hemorrhage is extremely rare presentation of GPA. Case report: A 58 year-old woman with pulmonary-renal syndrome due to GPA was treated with corticosteroids and cyclophosphamide. About 2 months later, patient was admitted with respiratory failure due to diffuse alveolar hemorrhage and renal failure. She was transferred to our hospital where treatment with pulse dose cortico-steroids and plasmapharesis was initiated. She developed lower GI bleeding for which colonoscopy and upper GI endoscopy with push enteroscopy was performed but failed to reveal a source. Tagged RBC scan showed active hemorrhage in the small intestine and mesenteric angiogram showed active bleeding from jejunal branch of superior mesenteric artery (SMA). Coil embolization of two branches of SMA was performed. Post embolization GI bleeding continued requiring frequent PRBC transfusions. She underwent exploratory laparotomy with small bowel resection and primary anastomosis. Histopathological examination of intestine showed granulomatous vasculitis of submucosal vessels, fibrinoid necrosis of vascular wall and scattered luminal thrombi along with multiple foci of mucosal ulceration and ischemia. These finding were consistent with GPA involving the small intestine. The patient was started on aggressive treatment with rituximab along with cortico-steroids and plasmapharesis. Despite intensive treatment with immunosuppression and supportive care, her clinical condition worsened and she eventually died. Discussion: GI involvement of GPA may vary from temporary abdominal pain to life-threatening hemorrhage and perforation. Often the diagnosis gets delayed because of the difficulty in discerning whether GI manifestations are due to vasculitis or high-dose immunosuppressive agents1. Histopathological examination confirmed this patient's diagnosis however it was too late in the course of her illness. Early diagnosis is often difficult but crucial to improve long-term morbidity and mortality.