Henoch-Schönlein purpura.
Henoch-Schönlein purpura is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis. It is characterized by a triad of palpable purpura (without thrombocytopenia), abdominal pain, and arthritis. Most patients have an antecedent upper respiratory illness. More than 90 percent of Henoch-Schönlein purpura cases occur in children younger than 10 years; however, adults with this condition are more likely to experience complications than children. All patients with Henoch-Schönlein purpura develop a purpuric rash, 75 percent develop arthritis, 60 to 65 percent develop abdominal pain, and 40 to 50 percent develop renal disease. Because Henoch-Schönlein purpura spontaneously resolves in 94 percent of children and 89 percent of adults, supportive treatment is the primary intervention. Oral prednisone at 1 to 2 mg per kg daily for two weeks has been used to treat abdominal and joint symptoms. A meta-analysis found that corticosteroid use in children reduced the mean time to resolution of abdominal pain and decreased the odds of developing persistent renal disease. Early aggressive therapy with high-dose steroids plus immunosuppressants is recommended for patients with severe renal involvement. Long-term prognosis depends on the severity of renal involvement. End-stage renal disease occurs in 1 to 5 percent of patients.
Reamy
Brian V
BV
Department of Family Medicine at Uniformed Services University of Health Sciences in Bethesda, MD 20814, USA. breamy@usuhs.mil
Williams
Pamela M
PM
Lindsay
Tammy J
TJ
D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007674:Kidney Diseases / Q000209:etiology / Q000517:prevention & control*; D011241:Prednisone / Q000008:administration & dosage*; D011379:Prognosis; D055815:Young Adult
D000894:Anti-Inflammatory Agents, Non-Steroidal; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011241:Prednisone