A rare case of granulomatosis with polyangiitis with involvement of the gastrointestinal system

Abstract

We report a rare case of a 37-year-old man with granulomatosis with polyangiitis (GPA) with gastrointestinal system (GIS) involvement who needed 526 units of blood and blood product transfusions and was followed up in the intensive care unit (ICU). GIS involvement due to GPA is a rare condition that increases morbidity and mortality of patients. Patients may require ultramassive blood product transfusions. Thus, patients with GPA can be admitted to ICUs because of massive hemorrhage due to multisystem involvement, and survival is achievable with meticulous care through a multidisciplinary approach.

Figure 1

(a) Abdomen CT: Pre-contrast phase, (b) Abdomen CT: Arterial phase, (c) Abdomen CT: Portal phase, (d) Abdomen CT: Late phase, In the pre-contrast phase, the contrast agent is not visible in the intestinal lumen. In the other phases duodenum 3–4. Appears to begin to escape into the intestinal lumen, consistent with active bleeding from the continental level, (e) Thorax computer tomography (CT): Cavitary lesion in the lung.

Figure 2

(a and b) In bowel resection spesimen, necrotising leukocytoclastic vasculitis with fibrinoid necrosis and fibrin thrombi noted in submucosal vessels. In addition, ischemic secondary changes in overlying mucosa were detected. (H&E, ×40, ×100), (c) In skin biopsy, inflammatory infiltrate which made up neutrophil and lymphocytes with fibrinoid necrosis was observed in wall of dermal vessels. These feature was in keeping with acute small (leukocytoclastic) vasculitis. (H&E, ×40).