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Clinical update: Henoch-Schönlein purpura

2007; Elsevier BV; Volume: 369; Issue: 9566 Linguagem: Inglês

10.1016/s0140-6736(07)60474-7

1474-547X

Frank T. Saulsbury,

Renal Diseases and Glomerulopathies

Henoch-Schönlein purpura (HSP) is an acute small-vessel leucocytoclastic vasculitis. HSP is the most common vasculitis in children, with an incidence of about 10 cases per 100 000 a year. 1 Saulsbury FT Henoch-Schönlein purpura in children: report of 100 patients and review of the literature. Medicine. 1999; 78: 395-409 Crossref PubMed Scopus (475) Google Scholar , 2 Calvino MC Llorca J Garcia-Porrua C Fernandez-Inglesias JL Rodriguez-Ledo P Gonzalez-Gay MA Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine. 2001; 80: 279-290 Crossref PubMed Scopus (240) Google Scholar In most series, boys are affected more often than girls. Although it can occur at any age, HSP is overwhelmingly a disease of childhood. The mean age of patients is 6 years; 75% of patients are under 8 years of age and 90% are less than 10 years of age. 1 Saulsbury FT Henoch-Schönlein purpura in children: report of 100 patients and review of the literature. Medicine. 1999; 78: 395-409 Crossref PubMed Scopus (475) Google Scholar , 2 Calvino MC Llorca J Garcia-Porrua C Fernandez-Inglesias JL Rodriguez-Ledo P Gonzalez-Gay MA Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine. 2001; 80: 279-290 Crossref PubMed Scopus (240) Google Scholar , 3 Trapani S Micheli A Grisolia F et al. Henoch-Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005; 35: 143-153 Summary Full Text Full Text PDF PubMed Scopus (299) Google Scholar The clinical features of HSP may be atypical at the extremes of age. The severity tends to be milder in infants under 2 years of age and worse in adults. Most patients present from autumn to spring, and HSP often follows a respiratory infection. A wide variety of pathogens, drugs, and other environmental exposures have been associated with HSP. Of all pathogens linked to HSP, group A β-haemolytic streptococcus has been the most studied. Positive throat cultures have been reported in 10–30% of patients, and titres to anti-streptolysin O are raised in 20–50% of patients. 1 Saulsbury FT Henoch-Schönlein purpura in children: report of 100 patients and review of the literature. Medicine. 1999; 78: 395-409 Crossref PubMed Scopus (475) Google Scholar , 2 Calvino MC Llorca J Garcia-Porrua C Fernandez-Inglesias JL Rodriguez-Ledo P Gonzalez-Gay MA Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine. 2001; 80: 279-290 Crossref PubMed Scopus (240) Google Scholar , 3 Trapani S Micheli A Grisolia F et al. Henoch-Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005; 35: 143-153 Summary Full Text Full Text PDF PubMed Scopus (299) Google Scholar Thus a substantial minority of patients have concomitant or recent streptococcal infection, but most cases have no direct link to streptococcal infection.