EUH Krakow Conference: What are the diagnostic criteria for Henoch-Schonlein purpura (HSP)?

The Bottom Line: European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) criteria for diagnosis of HSP include both:

  • purpura or petechiae not related to thrombocytopenia with lower limb predominance (or demonstration of immunoglobulin A [IgA] deposits on biopsy in patient with atypical distribution)
  • ≥ 1 of
    • abdominal pain
      • diffuse, colicky, with acute onset
      • may include intussusception or gastrointestinal bleeding
    • histopathological findings of either
      • typical leukocytoclastic vasculitis with predominant IgA deposits
      • proliferative glomerulonephritis with predominant IgA deposits
    • arthritis or arthralgia (joint pain, swelling and/or limitation of motion) of acute onset
    • renal involvement defined as ≥ 1 of
      • proteinuria > 0.3 g/24 hours or spot morning urine sample with albumin/creatinine ratio > 3 mg/mmol (30 mg/g)
      • hematuria
        • > 5 red blood cells/high power field
        • red blood cell casts in urinary sediment
        • ≥ 2+ on dipstick

Reference: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 113911, Henoch-Schonlein purpura; [updated 2015 Nov 5, cited 2017 Aug 1].

Summary: The differential diagnosis include:

Other causes of purpura or petechiae

  • vasculitides
    • granulomatosis with polyangiitis
    • polyarteritis nodosa
    • Takayasu arteritis
    • acute hemorrhagic edema of childhood
    • hypersensitivity vasculitis
    • microscopic polyangiitis
    • cutaneous leukocytoclastic vasculitis
    • cryoglobulinemic vasculitis
  • infections
    • bacterial endocarditis
    • meningococcal disease
    • Rocky Mountain spotted fever
    • acute poststreptococcal glomerulonephritis
    • sepsis in children or adults
    • chronic active Epstein-Barr virus mimicking HSP
  • immune disorders
    • thrombocytopenic purpura
    • autoerythrocyte sensitization syndrome
    • systemic lupus erythematosus (SLE)
  • leukemia, lymphoma
  • hereditary hemorrhagic telangiectasia
  • child abuse
  • bleeding disorders

Other causes of abdominal pain

  • acute abdomen
  • familial Mediterranean fever (may coexist with HSP)
  • inflammatory bowel disease (may coexist with HSP)
  • polyarteritis nodosa
  • Kawasaki disease
  • testicular torsion

Other causes of nephritis

  • IgA nephropathy
  • proteinuria
  • hematuria in children
  • gross hematuria in adults
  • microhematuria in adults

Other causes of arthritis or arthralgia

  • juvenile idiopathic arthritis (JIA)
  • Kawasaki disease
  • granulomatosis with polyangiitis
  • cutaneous leukocytoclastic vasculitis
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