A man in his twenties with fever and severe abdominal pain below the right costal margin.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterised by chronic rhinosinusitis, asthma, and pronounced peripheral blood eosinophilia. The most commonly involved organ is the lung. However, EGPA can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems.

Case Presentation: A previously healthy 24-year-old man was admitted to the hospital with fever and abdominal pain. He was treated with antibiotics due to suspicion of cholangitis, but his general condition did not improve. He was then given corticosteroids 1 mg/kg x 1 for suspected hypereosinophilic syndrome because of peripheral blood eosinophilia. The corticosteroids improved his condition. After a few days, however, he developed headache, paresis and impaired consciousness. CT cerebral venography revealed haemorrhaging secondary to cerebral venous sinus thrombosis. The patient developed brain herniation and died. Autopsy revealed that he suffered from EGPA.

Interpretation: Our patient had an unusual presentation with fever and abdominal pain. After the onset of fever and general symptoms, his vasculitis took an aggressive course. He did not have asthma, sinusitis, or allergies. According to the literature, about 96-100 % of EGPA cases are associated with asthma. Because EGPA is a rare disease, which can have a very serious course, increased knowledge and awareness of the condition is important to achieve early diagnosis and optimal treatment.