EPIDEMIOLOGY, CLINICAL MANIFESTATIONS AND PROGNOSIS OF HENOCH - SCHOENLEIN PURPURA IN NW GREECE

A. Drougia, M. Tzoufi, E. Galanakis, M. Charisi. Z.L. Papadopoulou

Department of Child Health, University of loannina Medical School, loannina, Greece.

OBJECTIVE: Henoch-Schoenlein purpura (HSP) is a common cause of vasculitis in children. It is a multisystem disease with obscure etiopatho-genesis and uncertain clinical course. The aim of this study was to investigate the epidemiological and clinical characteristics as well as the long-term prognosis of HSP in children in North-Western Greece.

METHODS: A total of 94 children, 61 boys and 33 girls, presenting with HSP over a 19 - year period (1980-1998), were studied retrospectively.

RESULTS: The male to female ratio was 1.8 /1 and the mean age at onset was 5.5 years (range 1-14). The incidence was found to be 7.6/100.000 children / year and was significantly higher (ratio 3.8/1) among children from urban areas. There was a higher morbidity during the October-December period (47% of cases) as well as during the years 1987-1990 (39% of cases). Preceding upper respiratory tract infection was noted in 45% of the children. The main clinical features were: purpuric skin rash (in all patients), joint involvement (71%), gastrointestinal symptoms (55%) and renal involvement (21%). Unusual clinical manifestations, such as testicular swelling and neurological complications were found in a small percentage of cases (6%). Management with corticosteroids was given in 17 patients (18%): 15 with gastrointestinal symptoms and 2 with nephrotic features. Clinical recurrence was observed in 21 children (22%), 15 boys and 6 girls. 71% of the recurrences occurred during the first 3 months (range 15 days - 3 years) after the onset of the disease. A second recurrence was presented in three patients and a third in one. Boys as well as older children were more prone to recurrences. Ominous prognostic features for recurrences were the involvement of the gastrointestinal tract (p < 0.05) and the prolonged duration of the first attack (p < 0.01). After a mean follow-up period of 6.4 years, the ultimate outcome was excellent with complete recovery in all patients.

CONCLUSIONS: HSP in NW Greece affects more frequently preschool boys who live in urban areas. Seasonal and yearly clusterings were identified; this finding supports the hypothesis of an infectious origin of HSP. The gastrointestinal involvement and the prolonged duration of the first attack were risk factors for recurrences. The overall prognosis in this unselected population was excellent without any long term morbidity. This study indicates a more optimistic outcome in HSP as compared to the majority of published cases involving primarily a more selected group of patients.