The quality of evidence is downgraded by study limitations (unclear allocation concealment and lack of blinding).
A Cochrane review [Abstract] 1 included 20 studies with a total of 1 963 subjects. Eleven studies examined the efficacy of therapies to prevent persistent kidney disease in IgA vasculitis (IgAV), previously known as Henoch-Schönlein purpura, with or without kidney involvement at presentation, and 9 studies examined therapies to treat established severe IgAV-associated kidney disease. One study included 54 adult patients, but the other studies only included children.
Preventing persistent kidney disease: In children given prednisone for 14 to 28 days at presentation of IgAV compared with placebo or supportive treatment, there was no significant difference in the risk of persistent kidney disease any time after treatment (RR 0.74, 95% CI 0.42 to 1.32; 5 studies, n=746). There were no significant differences in the risk of persistent kidney disease with antiplatelet therapy (3 studies) or heparin (2 studies) in children with or without kidney disease at entry, although heparin reduced the risk of proteinuria by 3 months compared with placebo or no specific treatment (RR 0.47, 95% CI 0.31 to 0.73; 2 studies, n=317).
Treating severe kidney disease: There was no differences in efficacy outcomes or adverse effects with cyclophosphamide compared to placebo or supportive treatment (2 studies, 1 involving 56 children and the other involving 54 adults). There were no differences in the numbers achieving remission of proteinuria with intravenous (IV) cyclophosphamide compared with mycophenolate mofetil (MMF) (65 children) or tacrolimus (142 children). In studies comparing cyclosporin with methylprednisolone (15 children), MMF with azathioprine (26 children), or MMF with leflunomide (19 children), it was unclear whether the treatment had any effect on the numbers in remission or the degree of proteinuria between treatment groups. In one study comparing plasmapheresis, cyclophosphamide and methylprednisolone with cyclophosphamide and methylprednisolone, there was no difference in the numbers achieving remission. Fosinopril compared with no specific therapy reduced the number of participants with proteinuria (1 study). No studies were identified that evaluated the efficacy of therapy on kidney disease in participants with recurrent episodes of IgAV.
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