A STEROID-SPARING APPROACH FOR ADOLESCENT IGA NEPHROPATHY: TONSILLECTOMY, STEROID PULSE THERAPY, AND SUBSEQUENT MIZORIBINE

 

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https://storage.unitedwebnetwork.com/files/1099/f6c81504f0319604bac13d1235deb975.pdf
A STEROID-SPARING APPROACH FOR ADOLESCENT IGA NEPHROPATHY: TONSILLECTOMY, STEROID PULSE THERAPY, AND SUBSEQUENT MIZORIBINE

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Tomoko
Tanaka
Tomoko Tanaka tomoko.tanaka@ompu.ac.jp Osaka Medical and Pharmaceutical University Pediatrics Takatsuki Japan *
Hideki Matsumura hideki.matsumura@ompu.ac.jp Osaka Medical and Pharmaceutical University Pediatrics Takatsuki Japan -
Yuko Fujii yuko.fujii@ompu.ac.jp Osaka Medical and Pharmaceutical University Pediatrics Takatsuki Japan -
Satoshi Yamazaki satoshi.yamazaki@ompu.ac.jp Amanokawa Hospital Pediatrics Hirakata Japan -
Akihiko Shirasu whitenumber@hirakatacity-hp.osaka.jp Hirakata City Hospital Pediatrics Hirakata Japan -
Hyogo Nakakura hyogon@amanokawa.or.jp Amanokawa Hospital Blood Purify Center Hirakata Japan -
Akira Ashida akira.ashida@ompu.ac.jp Osaka Medical and Pharmaceutical University Pediatrics Takatsuki Japan -
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Although the combination of tonsillectomy and steroid pulse therapy is frequently employed in adult patients with IgA nephropathy in Japan, the Japanese pediatric guidelines recommend this approach only weakly and for a limited subset of cases owing to insufficient evidence. In severe pediatric cases, oral corticosteroids are often administered as long-term therapy; however, prolonged exposure to these agents is associated with substantial adverse effects, particularly impaired linear growth and metabolic complications, which are especially concerning during adolescence. To mitigate these risks, a regimen has been attempted in which mizoribine is administered after tonsillectomy and steroid pulse therapy, thereby avoiding the need for subsequent maintenance with oral steroids. The present study summarizes the clinical course and outcomes of five patients treated with this regimen at our institution.

We retrospectively reviewed the medical records of patients who underwent tonsillectomy followed by steroid pulse therapy and subsequent mizoribine administration without maintenance oral steroids. Clinical variables included age at disease onset, renal biopsy findings, concomitant therapies, follow-up duration, clinical response, and adverse events. Clinical remission was defined in accordance with standard criteria for IgA nephropathy: “complete remission” as resolution of both hematuria and proteinuria, and “partial remission” as improvement in either.

Five patients (three males and two females) were included. Age at onset ranged from 10.4 to 14.4 years. Renal biopsy revealed mesangial proliferation in ≥80% of glomeruli in two patients, ≥50% in two, and <50% with cellular crescents in 25% of glomeruli in one. All patients received angiotensin-converting enzyme inhibitors with mizoribine; one also received antiplatelet and anticoagulant agents. The median follow-up duration was 3.0 years.

Two patients achieved complete remission, including one who discontinued all medications, while the remaining three achieved partial remission. Notably, no patient experienced steroid-related adverse effects such as growth impairment or Cushingoid features. One patient developed transient glycosuria during pulse therapy, which resolved spontaneously.

The combination of tonsillectomy, steroid pulse therapy, and subsequent mizoribine administration yielded favorable outcomes in all patients, with either complete or partial remission. Compared with conventional long-term oral steroid regimens, this approach may reduce adverse effects, particularly growth impairment, which is a critical concern in adolescents. Nonetheless, the small sample size and retrospective design limit the generalizability of these findings, and larger prospective studies are warranted to confirm efficacy and safety.

In conclusion, our findings suggest that tonsillectomy combined with steroid pulse therapy and subsequent mizoribine administration may represent a promising therapeutic strategy for adolescent patients with IgA nephropathy, particularly those approaching adulthood, in whom growth and quality-of-life considerations are paramount.

Kewords