What are the clinical features of Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Jan 08, 2021
  • Author: Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Craig B Langman, MD  more...
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Answer

The dominant clinical features of IgAV include cutaneous purpura, arthritis, abdominal pain, and nephritis. These manifestations may develop over the course of days to weeks and may vary in their order of presentation. The classic rash of IgAV is not the initial presenting sign in approximately one-quarter of patients. In one half to two thirds of children, an upper respiratory tract infection (URTI) precedes the clinical onset of IgAV by 1-3 weeks. In general, patients with IgAV appear mildly ill. They often have a low-grade fever, with a temperature that usually does not exceed 38°C (100.4°F).

IgAV is typically an acute, self-limited illness, and treatment is primarily supportive. In the majority of children, symptoms and signs of IgAV resolve within several days or months and the outcome is excellent. However, one third of patients have 1 or more recurrences.

Kidney involvement is the most important determinant of long-term morbidity. Up to 30-50% of children present with hematuria and/or proteinuria, or develop it within 4-6 weeks of the initial presentation. This is usually mild and self-limiting.  However, approximately 20% of IgAV children with nephritis (7% of all IgAV cases) will develop either a nephritis or nephrotic syndrome. [16, 17, 18, 19, 20, 21]

Keratitis and uveitis are rare sequelae of eye involvement.


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