Henoch-Schonlein Purpura

Background/Epidemiology

  • Most common vasculitis in children, although can be found in all ages (75% of cases <10) (incidence ~10-20/100,000)
    • Younger children are less likely to have complications e.g. nephritis of abdomincal complications
  • Commonly follows an infective episode (usually Group A β-haemolytic streptococcus) or vaccination

Pathology

  • IgA complexes are deposited in small vessels in the skin, gut, kidney (identical to IgA nephropathy- showing focal/segmental proliferative glomerulonephritis) and joints

Clinical Presentation

  • Skin- palpable purpuric rash is always present (part of diagnostic criteria)
    • May precede or be preceded by other systemic symptoms
    • Usually symmetrical over the extensor surfaces, lower limbs and buttocks, arms, face and ears but usually spares the trunk
    • Can range from petechiae to large ecchymoses
  • GI
    • Colicky abdominal pain occurs in 50-75% of cases
    • Vomiting and GI bleeding (either overt (rare- 2% of cases) or as faecal occult blood)
  • Joints
    • Many patients will have joint involvement; which may precede the onset of a rash in up to a quarter of patients
    • Usually arthritis of the large joints of the lower limb (e.g. knees, ankle, hips) although can affect upper limbs
    • Pain, swelling, decreased ROM; rarely causes any permanent damage
  • Renal
    • Occurs in between 20 and 60% of patients, can be 4-12 weeks after the initial rash and other symptoms
    • A range of renal symptomology can occur in HSP, including haematuria, proteinuria, nephrotic syndrome, nephritis, renal impairment and hypertension

Diagnosis

  • Palpable purpura + one of
    • Diffuse abdominal pain
    • Arthritis or arthralgia
    • Renal involvement (any haematuria and/or proteinuria)
    • Any biopsy showing predominant IgA deposition

Investigations

  • Majority of cases are clinical diagnoses
  • FBC, U&Es
  • Urinalysis
  • Renal biopsy may be indicated in patients with renal failure and nephritic syndrome

Management

  • Most patients will just require supportive treatment
  • Steroids may be useful in patients with severe renal involvement and severe abdominal pain
    • Patients should be closely monitored for renal involvement and followed up for up to year for this
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