Background: Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic smallvessel
vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys. It is the
most common form of systemic vasculitis in children.
Objective: The study aimed to familiarize physicians with the etiopathogenesis, clinical manifestations,
evaluation, and management of children with Henoch-Schönlein purpura.
Methods: A PubMed search was conducted in January 2020 in Clinical Queries using the key terms
“Henoch-Schönlein purpura” OR “IgA vasculitis” OR “anaphylactoid purpura”. The search
strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies,
and reviews published within the past 10 years. Only papers published in the English literature
were included in this review. This paper is based on, but not limited to, the search results.
Results: Globally, the incidence of HSP is 10 to 20 cases per 100, 000 children per year. Approximately
90% of cases occur in children between 2 and 10 years of age, with a peak incidence at 4 to
7 years. The diagnosis should be based on the finding of palpable purpura in the presence of at least
one of the following criteria, namely, diffuse abdominal pain, arthritis or arthralgia, renal involvement
(hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition. Most
cases are self-limited. The average duration of the disease is 4 weeks. Long-term complications are
rare and include persistent hypertension and end-stage kidney disease. Therapy consists of general
and supportive measures as well as treatment of the sequelae of the vasculitis. Current evidence
does not support the universal treatment of HSP patients with corticosteroids. Oral corticosteroids
may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemorrhage.
Conclusion: Most cases of HSP have an excellent outcome, with renal involvement being the most
important prognostic factor in determining morbidity and mortality. Unfortunately, early steroid
treatment does not reduce the incidence and severity of nephropathy in children with HSP. In HSP
children who have severe nephritis or renal involvement with proteinuria of greater than 3 months,
an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker should be considered in
addition to corticosteroids to prevent and/or limit secondary glomerular injury.