Table 1

Final EULAR/PRINTO/PRES HSP criteria (with glossary) and classification definition (sample 973)

CriterionGlossarySensitivity (%)Specificity (%)AUC (%)
Purpura (mandatory criterion)Purpura (commonly palpable and in crops) or petechiae, with lower limb predominance, * not related to thrombocytopenia898687.5
 1. Abdominal painDiffuse abdominal colicky pain with acute onset assessed by history and physical examination. May include intussusception and gastrointestinal bleeding616462.2
 2. HistopathologyTypically leucocytoclastic vasculitis with predominant IgA deposit or proliferative glomerulonephritis with predominant IgA deposit938991.1
 3. Arthritis or arthralgiasArthritis of acute onset defined as joint swelling or joint pain with limitation on motion784259.9
Arthralgia of acute onset defined as joint pain without joint swelling or limitation on motion
 4. Renal involvementProteinuria >0.3 g/24 h or >30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample337051.4
Haematuria or red blood cell casts: >5 red blood cells/high power field or red blood cells casts in the urinary sediment or ≥2+ on dipstick
HSP EULAR/PRINTO/PRES Ankara 2008 classification definition: κ 0.90 (95% CI 0.84 to 0.96)Purpura or petechiae (mandatory) with lower limb predominance* and at least one of the four following criteria:1008793.5
Abdominal pain
Arthritis or arthralgia
Renal involvement
  • * For purpura with atypical distribution a demonstration of an IgA deposit in a biopsy is required.

  • AUC, area under the curve; EULAR, European League Against Rheumatism; HSP, Henoch–Schönlein purpura; PRES, Paediatric Rheumatology European Society, PRINTO, Paediatric Rheumatology International Trials Organisation.