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15 years with a peak incidence at 4 to 6 years of age. In pediatric cases, a
male predominance has been noted with male-to-female ratio as high as
1.8:1. Presentation during adolescence or adulthood portends a worse
prognosis. The disease is more prevalent during fall, winter, and early
spring. The onset is usually sudden and frequently preceded by an acute
illness, often of the upper respiratory tract.
Though the acute illness is often predominated by rash, joint pain, and
gastrointestinal symptoms, the long-term prognosis for children with HSP
depends upon the extent of the renal involvement. Nephritis occurs in 30%
to 50% of affected patients, and the severity of renal involvement is not
correlated with the severity of the other symptoms. Most children who
develop nephritis will have relatively mild renal involvement such as
asymptomatic hematuria with mild or no proteinuria and preserved or
mildly and transiently impaired renal function. These patients are expected
to have a favorable long-term prognosis. A subset of children will develop
more severe renal involvement and develop nephritic syndrome or
combined nephritic–nephrotic syndrome. Severe disease can be associated
with decreased renal function, hypertension, hypoalbuminemia, and edema,
and severe acute involvement would increase the risk for long-term renal
sequelae. Approximately 1% develop end-stage renal disease (ESRD).
Clinical assessment. The diagnosis of HSP is clinical, and the history and
physical examination should focus on evaluation for the clinical
manifestations of the small-vessel vasculitis associated with HSP. The
hallmark signs and symptoms include nonthrombocytopenic purpuric rash,
arthralgias, nonerosive arthritis, abdominal pain, and nephritis in
individuals ≤20 years of age. The rash, often the most distinctive feature of
the disease, characteristically involves the buttocks and extensor surfaces of
the lower extremities. Purpura and joint pain are the most common
presenting symptoms, though studies have revealed that abdominal
symptoms may precede the rash in as many as 15% to 35% of cases.
Overall, gastrointestinal symptoms occur in approximately half of children