Henoch-Schönlein purpura
ICD-10 D69.0 · ICD-11 4A44.92

Treatment of Henoch-Schönlein purpura with IgA vasculitis nephritis and >50% crescents on renal biopsy

This protocol addresses the severe end of the renal spectrum in Henoch-Schönlein purpura: patients with IgA vasculitis nephritis confirmed to have more than 50% crescents on renal biopsy, together with impaired eGFR (<60 mL/min) or severe persistent proteinuria. Early, structured management is critical to preserving renal function.

Clinical Scenario

The defining features of this presentation are more than 50% crescents on renal biopsy alongside impaired eGFR (<60 mL/min) or severe persistent proteinuria — markers of severe renal involvement in IgA vasculitis nephritis.

Renal biopsy >50% crescents eGFR <60 mL/min Severe persistent proteinuria
Treatment Approach

Management in this setting involves immunosuppressive therapy combined with corticosteroids. The complete regimen — including agent selection, sequencing, duration, and monitoring — is available in the full protocol.

Treatment Goals

Target: stable renal remission at 12 months:

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References

Severe: >50% crescents on renal biopsy and impaired eGFR (<60 mL/min) or severe persistent proteinuria

1st line: Induction therapy: IV Cyclophosphamide with pulsed methylprednisolone and oral prednisolone for 6–9 months guided by response. Maintenance therapy: mycophenolate with steroid tapering

Children with IgAV nephritis and persistent proteinuria 0.5–1 g/d should be treated with ACE-I or ARBs.

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