Acute vasculitis with multiorgan involvement in a patient with familial Mediterranean fever

We report a rare case of a patient with long-standing familial Mediterranean fever who presented with sudden onset of dyspnea, abdominal pain, and cutaneous manifestations. Chest CT and histologic preparations disclosed pulmonary hemorrhage and signs of systemic vasculitis. Cyclophosphamide and steroid therapy were initiated, with marked improvement. Based on this and 1 other case, we propose that systemic vasculitis should be included as a clinical manifestation of FMF.