The exact pathophysiology of HSN remains to be elucidated. Hence, a therapeutic strategy that enables curative treatments for all the various grades of HSN patients has yet to be established. We report our experience performing
tonsillectomy combined with
steroid therapy for 16 pediatric proteinuric Henoch-Schönlein
nephritis (HSN) patients. All patients exhibited
hematuria and
proteinuria in their first HSN attack with the mean age of onset 7.7 years (range 4.75 - 13.9 years). Nine patients were diagnosed with clinically severe HSN presenting with massive
proteinuria (> 1 g/m(2)/day). Renal biopsy findings performed in 6 patients were Grade II (3), Grade III (2) and Grade IV (1) according to the International Study of
Kidney Diseases in childhood classification.
Tonsillectomy was performed after 1-4 cycles of
methylprednisolone pulses during oral
prednisolone (0.5 - 1.5 mg/kg/day)
therapy. In 2 patients, oral
cyclophosphamide therapy was added before the
tonsillectomy. The interval between the onset of HSN and
tonsillectomy was 97.4 +/- 24.5 days (range 27 424 days). In all patients,
proteinuria had disappeared by 6 months after the
tonsillectomy and the urine findings had normalized. The interval between
therapy initiation and complete remission was 9.6 +/- 2.0 months (range 2 - 26 months). Over follow-up periods of 4.9 +/- 0.6 years (range 2.2 - 9.3 years), no recurrences of
Henoch-Schonlein purpura or HSN were observed. There was a significant correlation between early
tonsillectomy performance and decreased time until normalization of the urine findings, indicating that the tonsils may have pivotal roles in the initiation and progression of HSN. Their elimination might promote the reversal of
nephritis. Although this study is retrospective, we suggested that
tonsillectomy at an early stage of HSN may be beneficial by shortening the period of illness and contributing to clinical recovery. Randomized controlled trials will be needed to confirm this supposition.