What are the diagnostic criteria for 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 diagnosis of IgAV, as revised by the European League Against Rheumatism (EULAR), Paediatric Rheumatology International Trial Organisation (PRINTO), and Paediatric Rheumatology European Society (PRES), requires the presence of palpable purpura or petechiae—with lower limb predominance and without thrombocytopenia or coagulopathy; usually in clusters—along with at least one of the following [5, 6, 7] :

  • Diffuse abdominal pain of acute onset
  • Any biopsy sample showing leukocytoclastic vasculitis, or proliferative glomerulonephritis with predominant IgA deposition in a kidney biopsy sample in patients with atypical distribution of purpura
  • Acute arthritis or acute arthralgia in any joint 
  • Kidney involvement (any hematuria or proteinuria) and IgA deposition

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