What are the KDIGO treatment recommendations for Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Oct 28, 2020
  • Author: Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Craig B Langman, MD  more...
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Answer

Kidney Disease Improving Global Outcomes (KDIGO) guidelines include the following recommendations [117] :

  • Treat children with IgAV who have proteinuria > 0.5g/1.73m (including those with nephrotic syndrome) with ACEIs or ARBs for 3.-6 months. [117] However, treatment with those drugs is unlikely to improve the acute glomerular inflammation and may delay resolution of sclerotic changes. Beneficial effects of methylprednisolone pulses with oral prednisone was reported in a prospective case series and also suggested in patients receiving combinations of multiple immunosuppressive drugs. [118, 119, 120, 121, 122]
  • Provide more-aggressive treatment using pulse methylprednisolone in combination with cyclophosphamide in patients with > 50% crescentic glomeruli and rapidly progressive deterioration in kidney function with or without nephrotic syndrome.  
  • Plasma exchange is usually added to the treatment regimen if plasma creatinine is > 500 μmol/L.

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