A Surgical Presentation of Churg-Strauss Syndrome.

Abstract

Case presentation: Eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome (CSS) is a rare, autoimmune vasculitis usually affecting small and medium-sized blood vessels in its later phases. It is a diffuse, systemic, multisystem disease that is reported to present with gastrointestinal manifestations but very rarely as an acute abdomen secondary to eosinophilic peritonitis. A 28-year-old relatively healthy male with a pre-existing diagnosis of inactive pulmonary sarcoidosis presented to the emergency department with an acute abdomen. After an exploratory laparotomy, multi-specialty involvement, and extensive investigations to exclude other differentials, a diagnosis of EGPA was made. The patient was treated with systemic glucocorticoids initially, followed by a tapering course of steroids and anti-interleukin 5 monoclonal antibodies as maintenance upon remission. EGPA can manifest in a myriad of ways including an acute abdomen, and medical treatment is useful in managing this presentation. Surgeons should be aware of the atypical causes of acute abdomen and should routinely broaden their differential diagnosis to include medical pathologies.