690 THE INCREASED FREQUENCY OF HENOCH SHOENLEIN PURPURA IN FAMILIAL MEDITERRANEAN FEVER

Abstract

Familial Mediterranean fever (FMF) is characterized by periodic attacks of fever and serositis, particularly peritonitis and synovitis. The disease is transmitted by an autosomal recessive gene and primarily affects Sephardic Jews, Arabs, Turks and Armenians. Duing the period 1970-1979 we observed seven children with FMF who developed Henoch-Shoenlein purpura (HSP). During this period 0.16% of 2140 pediatric admissions per year were due to HSP. From a pediatric FMF population of 70 patients an average of 23 (33%) were admitted annually and one (4.3%) had HSP each year. Pertinent clinical features at the time of HSP - age range 3-13 years (mean 7 years) males: females 5:2. All patients had non thrombocytopenic purpura, arthritis or arthlagia and fever. Five patients (70%) had diffuse abdominal pain and two boys had testicular pain and swelling. Four patients (56%) had hematuria and one proteinuria, only. The erythrocyte sedimentation rate ranged from 50-150 mm/hr (mean 88) and fibrinogen 208-660 mg/dl (mean 373 mg/dl). The duration of hospitalization ranged from 13-150 days (mean 51 days). The association of FMF and HSP, two diseases of unknown etiology may provide new avenues of study in these diseases.

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