Henoch Schonlein Purpura in Childhood: Epidemiological and Clinical Analysis of 150 Cases Over a 5-year Period and Review of Literature

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Objective

To examine epidemiological, clinical, and outcome in Italian children affected with Henoch Schönlein purpura (HSP).

Methods

Retrospective study of children discharged with a diagnosis of HSP from the Meyer Children’s Hospital, between 1998 and 2002. Epidemiological, clinical, laboratory data, treatment, and outcome were collected by reviewing medical charts. One year after data collection, the children’s parents were interviewed by telephone about the outcome.

Results

150 children entered the study: M:F = 1.8:1; mean age 6.1 ± 2.7 years. At onset, purpura was present in all cases, arthritis/arthralgias in 74%, abdominal involvement in 51%, scrotal edema in 13%, renal involvement in 54%, severe nephropathy in 7%, acute renal insufficiency in 2%, and intussusception in 0.6%. Purpura was the presenting symptom in 74%, arthritis in 15%, and abdominal pain in 12%. The most frequent laboratory abnormalities were high-erythrocyte sedimentation rate (ESR) (57%), hyper-IgA (37%), and proteinuria (42%). All patients recovered within 2 months. Recurrences, verified in 35%, were correlated with high ESR values and corticosteroid (CS) treatment, independently from other variables. After a mean 2.5-years follow-up, 2 patients had hematuria with normal renal function.

Conclusion

Epidemiological and clinical findings in our cohort are similar to those in the literature, even though the mean disease duration was shorter than previously reported. Relapses occurred significantly more frequently in children treated with CS. This finding supports the recommendation to limit the use of steroids to a carefully selected group of HSP children. The prognosis was excellent; although severe nephropathy was found in a small percentage of the children, at follow-up all had normal renal function. Thus, our study confirms the benignity of HSP in Italian children, especially regarding renal outcome.

Section snippets

Patients and Methods

This is a retrospective data study of all children with HSP hospitalized at the Anna Meyer Children’s Hospital, Florence, Italy, from January 1998 through December 2002; they were selected by a computerized search based on the discharging diagnosis of HSP.

Epidemiological Features

The main epidemiological data and etiological factors are shown in Table 1. Between January 1998 and December 2002, 150 white children, fulfilling the inclusion criteria described above in the “Patients and Methods” section, were selected. The patient population included 95 boys (63%) and 55 girls (37%). Males were affected more often than females (ratio M:F = 1.8:1). The patients ranged in age from 1 to 15 years (mean age 6.1 ± 2.7 years, median age 5.3 years). The age and gender distribution

Clinical Features

The main clinical manifestations of the 150 patients are summarized in Table 2.

Laboratory Findings

The main laboratory data of the acute phase are shown in Table 4. An increased ESR was detected in 85 (57%) patients. Leukocytosis was present in 31 (21%) patients, anemia in 21 (14%), and increased serum AST and/or ALT level in 5 (3%). Serum creatinine was measured in 145 patients and was normal in all but the 3 patients with renal insufficiency. Serum IgA level, evaluated in 104 patients, was higher than the normal range in 22 of 43 (51%) children less than 5 years old, and in 16 of 61 (26%)

Treatment

Twenty-two patients (15%) received nonsteroidal antiinflammatory drugs (NSAIDs), ie, flurbiprofen (5 mg/kg per day) or naproxen (15 mg/kg per day), to reduce severe joint pain. The duration of NSAID treatment ranged from 3 to 12 days (mean 6.1 ± 2.3 days).

A total of 19 children (13%) received corticosteroids, due to severe abdominal involvement in 16 and severe nephropathy in 3 cases. These patients were treated with oral or intravenous prednisone or prednisolone (1 to 2 mg/kg per day). The

Outcome

One year after data collection, the patients’ families were interviewed by telephone. The parents of 118 children (79%) provided information about the outcome of the disease. After at least 1-year follow-up (range 1 to 6 years, mean follow-up 2.5 years ± 0.8, median 2.1 years), 98 of 118 (83%) patients were found to have recovered within 2 weeks, and 20 (17%) patients recovered within 2 months after disease onset. Mean disease duration was 3 weeks. In 41 (35%) patients there had been a

Discussion

To our knowledge, this study provides 1 of the most extensive epidemiological reports published on HSP in children. In the present study, 150 children who presented with HSP over a period of 5 years were analyzed. HSP is the most common acute vasculitis affecting children, although it has also been reported in adults, especially in association with malignancies (23, 24, 25). Two cases of children with HSP and Wilm’s tumor have been published (26). The annual incidence of HSP ranges from 10 to

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