[Henoch-Schönlein purpura].
Henoch-Schönlein purpura (HSP) is a systemic IgA vasculitis affecting small vessels. The main clinical features include cutaneous purpura, arthritis and gastrointestinal symptoms. Nephritis may occur, typically a glomerulonephritis with IgA mesangial deposits. Pulmonary, cardiac, genital and neurological symptoms have also been observed. Although the cause is unknown, it is clear that IgA plays a pivotal role in the immunopathogenesis of HSP. HSP usually affect children whereas it is rare in adults in whom the disease is often more serious with more frequent and severe nephritis. Short term outcome depends on the severity of the gastro-intestinal manifestations. The long term prognosis depends on the presence and severity of the nephritis. Studies with prolonged follow-up show that up to one third of patients may reach end stage renal failure. Only symptomatic treatment is recommended in case of self limited disease. Treatment of severe HSP nephritis is still debated but some studies, which need to be confirmed, have reported the beneficial effect of corticosteroids combined with immunosuppressive drugs.
Pillebout
Evangéline
E
Service de néphrologie et de transplantation, hôpital Saint-Louis, Paris. evangeline.pillebout@sls.aphp.fr
Niaudet
Patrick
P
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005922:Glomerulonephritis, IGA / Q000209:etiology / Q000473:pathology; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D007070:Immunoglobulin A / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D007223:Infant; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009393:Nephritis / Q000209:etiology / Q000473:pathology; D009404:Nephrotic Syndrome / Q000209:etiology; D011379:Prognosis; D012737:Sex Factors
D000305:Adrenal Cortex Hormones; D007070:Immunoglobulin A; D007166:Immunosuppressive Agents