Background and Aims In Japan, tonsillectomy and corticosteroid therapy is widely performed in patients with IgA nephropathy, to improve kidney outcomes and reduce proteinuria and hematuria. Over the last decade, patients with IgA nephropathy with ≥1g/day of proteinuria are treated with corticosteroid globally. However, after the results of the STOP-IGA and TESTING trials regarding efficacy and safety, the KDIGO 2021 clinical practice guideline suggest the use of immunosuppressive drugs only in patients who remain at high-risk for progressive chronic kidney disease despite maximal supportive care, after considering the risks and benefits. The TESTING trial showed that even in the reduced-dose group, severe adverse events occurred. In order to reduce steroid-induced toxicity, we administrated steroid pulse therapy monthly for 3 consecutive months and tonsillectomy without post-treatment. Thereafter, the effects and adverse events were evaluated. Method Forty-two patients diagnosed with IgA nephropathy on biopsy at our hospital, with ≥0.3g/day of proteinuria, and who had undergone tonsillectomy and steroid pulse therapy were included in the study. Of these patients, 16 were treated with three months of consecutive steroid pulse therapy without follow-up steroid administration (MSP); the remaining patients were administrated the conventional steroid pulse therapy (SP), including modified Pozzi's (Lancet 1999) and Hotta's (AJKD 2001) regimens. We evaluated and compared the time to proteinuria remission and both hematuria and proteinuria remission between the two groups using Kaplan-Meier curve and log-rank test. Remission of proteinuria was defined as three consecutive urinary protein results of <0.3g/day over 6 months, and that of hematuria was defined as three consecutive results of <5 RBCs/high-power field in the urinary sediment. In addition to efficacy, we compared the incidence rates of adverse events between the two groups. Results There were no significant differences in the age, sex, time to diagnosis, histopathological findings according to the Oxford classification, or RAS inhibitor use between the MSP and SP groups. The eGFR(ml/min/1.73m2) was 81.3 and 78.5, and the urinary protein excretion (g/day) was 1.01 and 1.18, in the MSP and SP groups, respectively. The proteinuria remission rates in the MSP and SP groups were 81% and 69% after one year and 94% and 81% after two years, respectively, with no significant difference. The remission rates of both hematuria and proteinuria were 47% and 46% after one year and 77% and58% after two years, respectively, with no significant differences. The incident rates of adverse events, including diabetes and infections, was 13% and 54% in the MSP and SP groups, respectively, and was significantly lower in the MSP group (p = 0.005). Conclusion Tonsillectomy with monthly steroid pulse therapy was effective and safer than the conventional steroid pulse therapy in patients with IgA nephropathy.

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Background and Aims In the treatment of idiopathic focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome, the remission of proteinuria is considered to be an important goal. The partial remission of proteinuria improves renal survival, whereas it may progressively reduce the renal function. A study searched for a novel partial remission more accurately reflecting the long-term renal outcome. The goal of proteinuria reduction for improving the renal prognosis remains to be clarified. We examined factors associated with the long-term renal outcome of idiopathic FSGS. Method Of 148 patients with FSGS diagnosed based on kidney biopsy between 1981 and 2018, a retrospective cohort study was conducted involving 33 who had undergone immunosuppressive therapy for nephrotic syndrome, and had been followed-up for ≥1 year, excluding those with secondary FSGS. We examined the renal prognosis, regarding a 50% decrease in the estimated glomerular filtration rate (eGFR) as an outcome. We calculated the rate of decrease in the urinary protein level 4 and 8 months after the start of treatment, and estimated the rate of decrease associated with renal hypofunction using ROC analysis. Based on the results of ROC analysis, Cox’s proportional hazard analysis was performed using factors contributing to renal hypofunction as covariates. Results Concerning the background of the 33 patients, the mean follow-up period was 11.4 years, and there were 24 males. The mean age was 49.8 years, and the mean blood pressure was 100.5 mmHg. The mean urinary protein level, albumin (Alb) level, eGFR, and total cholesterol (TCho) level were 7.4 g/day, 2.1 g/dL, 44.3 mL/min/1.73 m2, and 369 mg/dL, respectively. Corticosteroid therapy was selected in 21 patients, whereas it was combined with steroid pulse therapy in 12. The daily dose of prednisolone was 37.3 mg. On ROC analysis, the rate of decrease in the urinary protein level after 4 months was 83.1% (AUC: 0.74, sensitivity: 0.80, specificity: 0.74), and that after 8 months was 85.7% (AUC: 0.78, sensitivity: 0.90, specificity: 0.65). Cox’s proportional hazard analysis, in which the data were adjusted with the sex, blood pressure, urinary protein level at the start of treatment, Alb level, eGFR, and treatment methods, showed that the rate of decrease in the urinary protein level after 4 months was significantly correlated with renal hypofunction: after 4 months: hazard ratio, 0.19 (95%CI: 0.04-0.77); p=0.0202; after 8 months: hazard ratio, 0.34 (95%CI: 0.05-1.37); p=0.1359. Conclusion In the treatment of idiopathic FSGS with nephrotic syndrome, the rate of decrease in the urinary protein level 4 months after the start of treatment was correlated with the long-term renal outcome.

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