Although immunoglobulin (Ig) A vasculitis (IgAV) nephritis is a common form of secondary pediatric glomerulonephritis, there is no consensus on an appropriate therapeutic regimen for moderate-to-severe pediatric IgAV nephritis grade III or the effectiveness of aggressive immunosuppressive therapy. The objective is to evaluate the efficacy and renal outcomes of methylprednisolone pulse therapy with or without cyclophosphamide pulse therapy for grade III IgAV nephritis in children. This retrospective, single-center study included 115 children with IgAV nephritis grade III. The primary endpoint was proteinuria reduction from moderate or severe levels to a normal level. The secondary endpoint was stable renal function, that is, an increase of less than 25% from the baseline creatinine level over the 4-month follow-up period. Among 115 children with IgAV nephritis grade III, 59 received methylprednisolone and cyclophosphamide double-pulse treatment; methylprednisolone and cyclophosphamide double-pulses did not significantly improve proteinuria remission. Proteinuria improvement did not show any difference with or without cyclophosphamide treatment. Furthermore, methylprednisolone pulse therapy showed no benefit over steroid therapy alone. The demographic and baseline disease characteristics among the treatment groups were well-balanced. The rates of complete remission in 24-hour proteinuria excretion over the 4-month follow-up period in the methylprednisolone and cyclophosphamide double-pulse, methylprednisolone plus oral prednisolone, and oral prednisolone-only groups were 91.52%, 92.31%, and 100%, respectively. Renal function remained stable in all the patients. Most patients with IgAV nephritis grade III showed a good prognosis. However, the addition of methylprednisolone and/or cyclophosphamide pulses did not offer benefits over steroid-only therapy.
Keywords: Henoch-Schönlein purpura nephritis; IgAV nephritis; children; cyclophosphamide; immunosuppressive drug; treatment.