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Henoch-Schönlein purpura

Key sources
The following summarized guidelines for the evaluation and management of henoch-Schönlein purpura are prepared by our editorial team based on guidelines from the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019) and the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2012).
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Guidelines

1.Classification and risk stratification

Classification: use the EULAR/PRINTO/PReS-endorsed Ankara 2008 criteria for the classification of IgA vasculitis (formerly known as HSP).
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2.Diagnostic investigations

Evaluation for renal involvement: obtainEGFR measurement and urinalysis (for hematuria and urine protein/urine creatinine ratio or urine albumin/urine creatinine ratio) for the evaluation of renal involvement in patients with IgA vasculitis.
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  • Abdominal ultrasound

3.Diagnostic procedures

Skin biopsy: perform skin biopsy with specific immunofluorescence stating for IgA in patients with atypical rash and/or to exclude alternative diagnosis.
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  • Kidney biopsy

4.Medical management

Corticosteroids: initiate corticosteroids in patients with IgA vasculitis for the treatment of:
orchitis
cerebral vasculitis
pulmonary hemorrhage
other severe organ- or life-threatening vasculitis manifestations
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  • Analgesics

5.Specific circumstances

Patients with immunoglobulin A nephritis, angiotensin-converting enzyme inhibitors and angiotensin receptor blockers, SHARE initiative: consider administering ACEIs to prevent/limit secondary glomerular injury in patients with IgA nephritis with persistent proteinuria.
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  • Patients with IgA nephritis (corticosteroids)

  • Patients with IgA nephritis (immunosuppressants)

  • Patients with IgA nephritis (combination therapy)

  • Patients with crescentic nephritis

6.Preventative measures

Prevention of immunoglobulin A nephritis: as per SHARE initiative 2019 guidelines, do not administer prophylactic corticosteroids to prevent the development of IgA vasculitis-associated nephritis.
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7.Follow-up and surveillance

Indications for specialist consultation: consult with a pediatric nephrologist:
if the patient with IgA vasculitis has moderate proteinuria and/or impaired GFR
when starting treatment for IgA nephritis
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