Introduction:Sepsis is a well-recognised medical emergency requiring aggressive medical management, often in a critical care setting. Granulomatosis with Polyangiitis (GPA) (previously named Wegener's Granulomatosis) is a type of primary systemic antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) [2][3][4]. AAV can present with a variety of symptoms including those which mimic infective disease. Differentiation between these two diseases is extremely important because their respective treatments differ significantly and delays in commencing therapy can be fatal. Case Presentation: A 70 year old Caucasian gentleman presented with symptoms suggestive of severe sepsis who failed to respond to standard treatment. However an autoimmune screen revealed that he was in fact suffering from severe generalised GPA. Despite aggressive treatment

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