A REPORTE OF 194 CASES WITH HENOCH SCHOENLEIN PURPURA IN IRANIAN PEDIATRICS

Henoch Schoenlein Purpura (HSP) is a vasculitis of small vessels (capillaries, arteries and veins) with unknown etiology. In this report, we studied this disease in 194 children with HSP who admitted in the Pediatric Medical Center between (March 1986 – to Feb 2004). Patients with mild to moderate severity form of the disease, the majority of cases, who are normally managed on out-patient basis were excluded from this study. The patients characteristics, clinical state, para-clinical findings and the type of treatment intervention were recorded. The results showed that the minimum age at onset of the disease was 2 years and maximum was 14 years with mean age of 7.6 with (SD ± 2.982). Male to female ratio of 1.81:1 (M:F 64: 36 %), with maximal seasonal occurrence, similar to European countries, was in Autumn and Spring. The mean duration between our visits and their last attack of disease was 48 months. History of infectious diseases, as possible predisposing factor, prior to HSP, mostly URI, was positive in 61.4 % of cases. While infections due to -hemolytic type A infections was 26 % which was lower than reported among European population. In addition, there was a history of prior drug consumption in 69.7 % of patients. The common signs and symptoms were: skin manifestation in 95%, artheralgia and arthritis in 91%, gasterointestinal involvement in 74.4 %, renal involvement in 47.7 %, of all patients. The latter findings was relatively higher to European estimates. Para-clinical data: Anemia was detected in 38 % of cases, increase of ESR in 64.9 %, positive CRP in 64 % Culture of pharynx was positive for Streptococcus  hemolytic Group A in 20 % of ASOT more than 625 in 14%, serum IgA was abnormal in 24.2 % and elevated BUN in 9.7 % and serum creatinine in 6 % of patients. Complications occurred in 47.7 % and renal problems in 30 % of cases. Haematuria was found in 23.7 %, proteinuria in 18% of cases. Gastrointestinal manifestation such as pain occult blood in stool was found in 74.7 % and severe gastrointestinal and hemorrhage in one case. Approximately 38 % of 194 patients were managed conservatively, 22 % were treated with NSAIDs, 53.6% with oral prednisolone, 12.3 % cytotoxic drugs and in 5% of cases were managed with intermittent administration of pulses of methylprednisolone and cyclophosphamide. Since in this study we did not include mild and moderate forms of HSP, who are the majority of cases and are managed out-patiently, therefore, in order to gain more accurate picture of this disease in our community the results of this study suggest children attending both private and emergency departments to be included in future studies.