RECURRENT HENOCH-SCHÖNLEIN PURPURA IN FAMILIAL MEDITERRANEAN FEVER
2008
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 16and clinical response to specific treatment. Different types of vasculitis have been reported in FMF.
Henoch-Schonlein 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 (crescentric
glomerulonephritis) and protracted
abdominal painin the second one.
Glomerulonephritiswas controlled by methyl-prednisolone pulse therapy plus oral corticosteroid and
azathioprine, but
abdominal painwas resistant to steroids and revealed completely by intravenous immunoglobuline (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 painof HSP.
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