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

IgA Vasculitis Nephritis with >50% Crescents on Renal Biopsy: Second-Line Approach After First-Line Induction Has Not Achieved Renal Remission

Clinical Scenario

This protocol addresses Henoch-Schönlein purpura presenting as IgA vasculitis nephritis with severe histological involvement — specifically, more than 50% crescents on renal biopsy — combined with impaired eGFR (below 60 mL/min) or severe persistent proteinuria.

When First-Line Induction Has Not Worked

This second-line protocol is indicated when induction therapy with intravenous cyclophosphamide, pulsed intravenous methylprednisolone, and oral prednisolone — followed by maintenance with mycophenolate and steroid tapering, and ACE inhibitors or ARBs for persistent proteinuria — has failed to achieve stable renal remission at 12 months. The targets that were not met include: eGFR above 60 mL/min/1.73 m², urinary protein-to-creatinine ratio below 30 mg/mmol, proteinuria below 1+ on dipstick, and haematuria within defined thresholds.

Second-Line Treatment Direction

The next step involves an immunosuppressive combination with corticosteroid therapy. Which agents are selected, how criteria are applied to individual patients, and the complete treatment algorithm are detailed in the full protocol.

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References

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

2nd line: Azathioprine or mycophenolate mofetil + steroid therapy.

Children with IgAV nephritis and persistent proteinuria >1 g/d after a trial of ACE-I or ARBs, and GFR >50 mL/min per 1.73 m² should be treated with a 6-month course of corticosteroid therapy (either pulse followed by oral steroid 0.5 mg/kg prednisone on alternate days for 6 months or oral prednisone 0.8–1 mg/kg/d for 2 months and then reduced by 0.2 mg/kg/d per month for the next 4 months)

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