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Henoch-Schönlein purpura
1Department of Pediatrics, Louisiana State University Health Sciences Center and Children's Hospital, 1542 Tulane Avenue, T8-1, New Orleans, LA 70112, USA. a61543@pol.net
Current Rheumatology Reports
|May 12, 2004
Summary
Henoch-Schönlein purpura (HSP) is an immunoglobulin A-mediated vasculitis affecting multiple organs, most common in children. Steroids may treat severe HSP symptoms, but long-term outcomes depend on kidney and neurological involvement.
Area of Science:
- Immunology
- Pediatrics
- Rheumatology
Background:
- Henoch-Schönlein purpura (HSP) is a common childhood vasculitis characterized by immunoglobulin A deposition.
- It typically affects the skin, joints, gastrointestinal tract, and kidneys, often presenting with a distinctive rash on the lower extremities and buttocks.
- Diagnosis in children is usually clinical, but skin biopsy may be necessary in atypical cases, including adults and very young children.
Purpose of the Study:
- To summarize the clinical presentation, diagnostic considerations, treatment modalities, and prognosis of Henoch-Schönlein purpura.
- To highlight the role of corticosteroids in managing severe HSP manifestations.
- To underscore the importance of monitoring for renal and neurological involvement due to potential long-term morbidity.
Main Methods:
- Review of existing literature on Henoch-Schönlein purpura, including retrospective studies and clinical experience.
- Analysis of diagnostic criteria and the utility of skin biopsy in different age groups.
- Evaluation of treatment strategies for various HSP involvements and their supporting evidence.
Main Results:
- HSP commonly affects children aged 3-10 years with a characteristic rash distribution, making biopsy often unnecessary in this group.
- Skin biopsy is frequently required for diagnosis in adults and very young children due to atypical rash presentation.
- Steroids are supported by retrospective data for severe gastrointestinal, renal, central nervous system, and testicular involvement.
Conclusions:
- While HSP generally has a self-limited course and excellent prognosis, severe renal and neurological complications can lead to significant long-term morbidity.
- Recurrences are common, occurring within weeks to several years after the initial episode.
- Aggressive management with steroids, and potentially immunosuppressive agents or plasmapheresis for severe cases, is crucial for improving outcomes.