Small bowel ischemia, perforation, and enterocutaneous fistula as gastrointestinal manifestations of eosinophilic granulomatosis with polyangiitis: A case report

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg–Strauss syndrome, is a rare small- to medium-vessel vasculitis characterized by asthma, eosinophilia, sinonasal disease, and variable systemic involvement. Gastrointestinal involvement is less common but clinically important, as it may indicate severe disease and can lead to complications such as bowel ischemia, perforation and fistula formation. Case Report: We report the case of a 46-year-old gentleman with a background of asthma and chronic sinusitis requiring functional endoscopic sinus surgery on two occasions, who initially presented with worsening shortness of breath, wheeze, productive cough, and lethargy. Initial investigations for pulmonary embolism were negative, and he was treated for an acute asthma exacerbation. During admission, he was found to have marked eosinophilia, raised inflammatory markers and positive antineutrophil cytoplasmic antibody (ANCA), supporting a diagnosis of EGPA. He improved following corticosteroid therapy and was discharged. One month later, after interruption of prednisolone therapy, he represented with severe abdominal pain and melaena. Computed tomography (CT) imaging demonstrated ischemic small bowel with portal and mesenteric venous gas. Laparotomy revealed diffuse dusky small bowel, most severe in the proximal jejunum, but no bowel resection was performed due to intraoperative improvement. He was managed in intensive care with high-dose corticosteroids, antibiotics, anticoagulation, nutritional support, and multidisciplinary input. Ongoing disease activity required cyclophosphamide, subsequently changed to rituximab. His course was complicated by bowel perforation, wound dehiscence, and enterocutaneous fistula, which were managed conservatively with bowel rest, total parenteral nutrition, antibiotics, intravenous immunoglobulin, and ongoing immunosuppression. Conclusion: This case highlights the importance of considering EGPA in patients with difficult-to-control asthma, sinonasal disease, and eosinophilia. Gastrointestinal involvement may be life-threatening and should prompt urgent multidisciplinary assessment and escalation of immunosuppressive therapy.