Wegenerʼs Granulomatosis Presenting with Gastrointestinal Bleeding and Jejunal Ulcerations

Abstract

Purpose: Wegener's granulomatosis (WG) is a rare multisystem disease commonly characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, kidneys and necrotizing vasculitis affecting smalland medium-sized vessels. Although most organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We report a case of a 40 year old man with no significant medical history that developed epistaxis, arthralgias, and skin rash over the course of two months. At this time, he presented to an outside hospital where a chest radiograph showed a cavitary lung lesion as well as pulmonary nodules. Therefore, a CT guided biopsy of the lung nodules was performed and revealed normal lung parenchyma. Upon follow-up with his primary physician he was found to have a rising creatinine (1.7 mg/dL) and worsening complaints of arthralgias and skin rash and was subsequently admitted to our institution for workup. Physical examination displayed a purpuric diffusely erythematous skin rash present on the upper torso and lower extremities. Skin biopsy showed leukocytoclastic vasculitis. A fiber optic exam of the nasopharynx revealed mucosal ulcerations and a nasal septal perforation. Laboratory data showed an elevated creatinine (7.5 mg/dL), hematuria, proteinuria, elevated erythrocyte sedimentation rate (131 mm/hr), and highly positive c-ANCA (1:160). The patient was initiated on hemodialysis. A renal biopsy performed suggested crescentic glomerulonephritis. He was diagnosed with Wegener's granulomatosis and started on high dose corticosteroids, plasmapheresis, and IV cyclophosphamide. Three days after treatment was initiated the patient passed a large melanic stool and became unstable with hypotension and tachycardia. His hemoglobin dropped from 8.9 gm/dL to 5.7 gm/dL. Small bowel push enteroscopy was undertaken using a pediatric colonoscope with visualization of four distinct ulcers seen scattered throughout the proximal jejunum as well as a few small ulcers in the stomach. One site in the jejunum appeared to have a visible vessel within an area of ulceration and erythema. The bleeding vessel was treated with epinephrine injection. Biopsies were taken of the gastric ulcer edges and revealed benign antral mucosa with changes consistent with reactive gastropathy. However, typical histological findings in the gastrointestinal tract for WG are uncommon. The patient was continued on high dose corticosteroids and IV cyclophosphamide monthly and his symptoms improved with no further GI bleeding. The gastric and jejunal ulcerations are thought to be secondary to WG. This rare case illustrates that Wegener's granulomatosis can target the gastrointestinal tract.