The concurrence of an enterocutaneous fistula and granulomatosis with polyangiitis: The role of immunosuppression as a bridge to definitive surgical treatment

Abstract

Granulomatosis with polyangiitis (GPA) is a systemic disease that consists of vasculitis and granulomatous inflammation, and that usually affects the respiratory tract, the ear, nose, and throat sphere, and the kidneys. GPA may also cause skin manifestations that include ulcerations, nodules, or papules. An enterocutaneous fistula (ECF) is an abnormal tract that connects the skin surface to the gastrointestinal system. We report the first case of an ECF as a concurrent clinical manifestation during a new-onset GPA in a 68-year-old male patient. The patient presented with an abdominal cutaneous wound with subcutaneous abscess that evolved into an ECF with spontaneous enteric drainage. He also complained of nasal crusting, epistaxis, and cough, with further investigation revealing bilateral pulmonary nodules. Transthoracic biopsy was performed and was suggestive of necrotizing vasculitis. A diagnosis of autoimmune vasculitis was highly suspected, and an immunosuppressive regimen of corticosteroid and intravenous cyclophosphamide was initiated. Significant improvement was noted in nasal manifestations, cough, and the output of the ECF. Definitive surgical management of the ECF was performed successfully. To the best of our knowledge, the presentation of a GPA with an ECF has not been previously reported and poses major challenges to medical and surgical treatment, as it constitutes a dilemma as to how to address an autoimmune process requiring immunosuppression in the context of an infectious condition. This presentation suggests that immunosuppression in these patients may still be considered. The concomitant presence of an ECF with abscess, an infectious process, and of an autoimmune disorder requiring immunosuppression is a major medical challenge. This case suggests that immunosuppression may still be considered in these patients to promote a better control of the concomitant ECF before definitive surgical therapy.