Cohort study of advanced IgA nephropathy: efficacy and limitations of corticosteroids with tonsillectomy.

BACKGROUND: Elevated serum creatinine is associated with poor outcome in IgA nephropathy (IgAN). The efficacy and limitations of corticosteroids in advanced IgAN (Cr >or=1.5 mg/dl), however, remains controversial. METHODS: We retrospectively investigated 70 patients with advanced IgAN (Cr >or=1.5 mg/dl) classified into three groups according to their treatment regimens, that is, steroid pulse with tonsillectomy, conventional steroid, and supportive therapy. We evaluated the three groups to elucidate predictors for the endpoints ESRF and doubled serum creatinine from baseline. RESULTS: Steroid pulse with tonsillectomy, conventional steroid and supportive therapy were performed in 30, 25 and 15 patients, respectively. During the mean follow-up period of 70.3 (12-137) months, 41.4% of patients reached ESRF (13.3 vs. 56.0 vs. 73.3%, p < 0.001) and 45.7% doubled serum creatinine from baseline (16.7 vs. 64.0 vs. 73.3%, p < 0.001). The incidence of ESRF in the patients treated by steroid pulse with tonsillectomy was significantly lower than the incidences in the patients treated by conventional steroid and supportive therapy at a baseline creatinine level of 1.5-2 mg/dl, but no statistical difference was observed at a level of >2 mg/dl. The Kaplan-Meier estimated probability of renal survival without ESRF was 89.2, 74.1 and 72.2% at 5 years and 82.8, 51.0 and 45.1% at 8 years, respectively (p = 0.017). The predictors for ESRF, identified in a Cox proportional hazards model, were baseline serum creatinine (p < 0.001) and interstitial infiltrate (p = 0.003). Steroid pulse with tonsillectomy also had a protective effect on the risk of reaching ESRF (p = 0.013). By target cross-stratification, the patients with baseline creatinine of 1.5-2 mg/dl who underwent steroid pulse with tonsillectomy showed a better renal survival rate than the others (p < 0.001). CONCLUSION: Steroid pulse therapy combined with tonsillectomy may be more effective than conventional steroid therapy in patients with a baseline creatinine level of

Decreased CD4 lymphocyte count as a marker predicting high mortality rate in managing ANCA related rapidly progressive glomerulonephritis.

As antineutrophil cytoplasmic antibody positive rapidly progressive glomerulonephritis (ANCA-RPGN) has a high risk of end stage renal failure and is a potentially life threatening disease, early aggressive therapy is recommended. However, aggressive immunosuppressive therapy may lead to immunodeficiency and subsequent mortality in the patients with this disease. Therefore, we need the index of immunodeficiency to cure the disease. To evaluate any risk factors, including therapies, on mortality in ANCA-RPGN, we conducted a retrospective investigation on patient survival in 32 patients with ANCA-RPGN by Kaplan-Meier analysis and the Cox regression model. Fourteen patients were treated with leucocytapheresis (LAP group) and the 18 patients were treated by steroid pulse therapy (steroid pulse group) as initial treatment. The patients were chosen for the different therapies at random. Two patients in the LAP group, and eight patients in the steroid pulse group had died within 6 months. The lymphocyte counts and CD4 cell counts after complete course of therapy were lower in the patients who died than in those who survived in the steroid pulse group. Patient survival was higher in the LAP group than in the steroid pulse group, but did not reach statistical significance. Multivariate Cox regression analysis showed that the factors influencing patient survival were initial serum creatinine, LAP therapy, CD4 cell counts, and lymphocytes at the end of treatment. Age, titer of MPO-ANCA, and percent of glomerular crescents were not found to have an effect on the patient survival. We recommend: that early diagnosis should be established, and immunosuppressive therapy may be done with monitoring of the lymphocyte and CD4 cell count.