Recurrent Henoch-Schönlein purpura in familial mediterranean fever

Abstract

Familial Mediterranean fever (FMF) is a relatively rare disorder, characterized by recurrent self-limited attacks of fever and polyserositis. Diagnosis is made by clinical features, gene identification on chromosome 16 and clinical response to specific treatment. Different types of vasculitis have been reported in FMF. Henoch-Schönlein purpura (HSP) is one of them, usually with a benign clinical course. Repeated attacks of HSP have been rarely reported in FMF. This is the report of a 7-year-old girl who presented initially with recurrent fever and abdominal pain. After the primary diagnosis of FMF and appropriate treatment, she experienced two documented repeated attacks of HSP with severe renal involvement (crescentic glomerulonephritis) and protracted abdominal pain in the second one. Glomerulonephritis was controlled by methyl-prednisolone pulse therapy plus oral corticosteroid and azathioprine, but abdominal pain was resistant to steroids and revealed completely by intravenous immunoglobulin (IVIg) administration. In conclusion, it is suggested to consider the recurrence of HSP in cases with FMF to prevent irreversible renal complications. IVIg seems to be a good choice for the management of intractable abdominal pain of HSP. © 2008 Tehran University of Medical Sciences. All rights reserved