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PekkaLahdenne

Henoch-Schönlein Purpura

Essentials

  • The disease most commonly affects children between 2 and 10 years of age. Purpuric skin changes caused by vasculitis are typical symptoms.
  • Articular symptoms, abdominal pains and haematuria or proteinuria also often occur.
  • Patients who, in addition to the typical skin changes, have mild abdominal and/or articular symptoms but no haematuria or proteinuria can be treated by a general practitioner.

Epidemiology, symptoms and signs

  • The vasculitis is common and is often preceded by a bacterial or viral upper respiratory tract infection.
  • Papules that develop into petecchias appear most commonly on the lower extremities and buttocks (pictures 1 2 3 4).
  • Extensive periarticular swelling and tenderness may be observed in the ankles and knees, and sometimes also in wrists and elbows. Three patients out of four have articular symptoms.
  • Abdominal pain occurs in about half of the patients. About 10% of the patients have severe colicky pains.
  • Almost half of the patients have haematuria and/or proteinuria during the disease. Overt nephritis is rare, and occurs at a later stage of the disease.
  • Basic investigations include complete blood count, CRP, throat culture, antistreptolysin titre, anti-streptococcal DNase antibodies and a urine dipstick test.

Differential diagnosis

  • Consider another disease if
    • the patient has a fever exceeding 38.5°C
    • the plasma CRP concentration exceeds 20 mg/l or the ESR is above 35 mm/h
    • the platelet count is decreased or anaemia or leukopenia is detected.
  • Remember that meningococcal sepsis causes petecchias and sometimes also joint symptoms. If the platelet count is low (< 100) the patient may have ITP or a haematological malignancy.

Complications

  • Intestinal intussusception and melena are in rare cases seen as acute complications of the disease, and very rarely bleeding in other parts of the body.

Treatment and follow-up Interventions for Preventing and Treating Kidney Disease in Iga Vasculitis (Henoch-Schönlein Purpura)

  • Mild abdominal and/or articular symptoms, no haematuria or proteinuria: paracetamol 15 mg/kg 3 times daily as needed. In addition, possible underlying tonsillitis or sinusitis is diagnosed and treated as necessary. Urine dipstick tests are taken in association with febrile diseases in the following 6 months.
  • Abnormal finding in a urine test or severe abdominal or articular symptoms: the patient is referred to a specialist. In these cases, prednisolone is started at discretion of the specialist, 1 mg/kg for 2 weeks, then 0.5 mg/kg for 2 weeks and then 0.5 mg/kg every second morning for 1 week.
  • The treatment of a severe HSP glomerulonephritis is in the responsibility of a paediatric nephrologist.
  • If there are signs of renal involvement - particularly if they are significant and last for more than one month - the patient's renal function must be followed up throughout life, as the risk of renal failure is significant. All women who had even mild renal symptoms at onset of Henoch-Schönlein purpura should be carefully observed during and after pregnancy.

References

  • Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet 2002;360:666-670
  • Jauhola O, Ronkainen J, Koskimies O et al. Outcome of Henoch-Schönlein purpura 8 years after treatment with a placebo or prednisone at disease onset. Pediatr Nephrol 2012;27(6):933-9. [PubMed]

Evidence Summaries