Early Treatment with Methylprednisolone Pulse Therapy Combined with Tonsillectomy for Heavy Proteinuric Henoch-Schönlein Purpura Nephritis in Children

BACKGROUND: There is no clear consensus as to which patients with Henoch-Schönlein purpura nephritis (HSPN) at risk of a poor outcome should be treated and what therapeutic regimen should be used. METHODS: Nine children with heavy proteinuric HSPN received prompt initiation of methylprednisolone pulse therapy (MPT) combined with tonsillectomy in a prospective study. RESULTS: At presentation, the mean values for the patients’ urine protein excretion (early-morning urinary protein/creatinine ratio), serum IgA, activity index (AI), and chronicity index (CI) were 5.0 ± 5.6 g/g Cr, 135.6 ± 56.5 mg/dl, 4.0 ± 0.7, and 1.7 ± 1.3, respectively. At the second biopsy, conducted approximately 24 months after initiation of therapy, the patients’ serum albumin had significantly increased (4.4 ± 0.2, p < 0.01), and the serum IgA and AI had significantly decreased (88.1 ± 30.8 mg/dl, p < 0.05; 2.0 ± 1.2, p < 0.01, respectively), whereas the CI remained unchanged. Proteinuria disappeared within 24 months in all but 1 patient, and hematuria disappeared within 38 months in all patients. No patient showed renal impairment or experienced a recurrence and/or exacerbation of HSP/HSPN. CONCLUSIONS: Early treatment with MPT combined with tonsillectomy is effective in ameliorating the histopathological progression and improving the clinical course of children with heavy proteinuric HSPN.