Yuko Iwabuchi

Long-Term Prognosis of Adult Patients with Steroid-Dependent Minimal Change Nephrotic Syndrome Following Rituximab Treatment

AbstractThis study was to evaluate the long-term efficacy and safety of a single-dose rituximab regimen rituximab treatment in adult patients with steroid-dependent minimal change nephrotic syndrome (MCNS).We conducted a prospective cohort study with historical controls to evaluate the effect of single-dose infusions of rituximab at 375 mg/m2 BSA per dose administered at intervals of 6 months for a period of 24 months. At the end of the 24-month period, the patients were divided into the treatment continuation (n = 20) and treatment discontinuation (n = 5) groups according to their intention to continue/discontinue the treatment.A significant reduction in the total number of relapses was observed during the 24-month period after the first rituximab infusion as compared with that during the 24-month period before the first rituximab infusion (108 vs. 8, P < 0.001). Complete remission was induced/maintained in all patients from 12 to 24 months after the first rituximab infusion. In regard to the clinical course after 24 months, 4 of the 20 patients in the treatment continuation group discontinued the rituximab treatment after the fifth infusion and 2 patients discontinued the treatment after the sixth infusion. However, complete remission was maintained in all the 20 patients of this group during the 12-month observation period after the first four single-dose rituximab infusions. On the other hand, 1 of the 5 patients in the treatment discontinuation group developed relapse during the observation period after the first four rituximab infusions, and the rituximab treatment was resumed.In our trial, rituximab therapy was associated with maintenance of complete remission. Complete remission was maintained even in most of the patients who showed B-cell repletion after discontinuation of rituximab therapy. Thus, rituximab may be considered as a radical therapeutic agent for patients with steroid-dependent MCNS.

Left ventricular end-diastolic diameter is an independent predictor of mortality in hemodialysis patients.

Left ventricular (LV) function is impaired in most hemodialysis (HD) patients. We conducted an observational cohort study to investigate whether LV end‐diastolic diameter (LVDd) could predict all‐cause mortality in a cohort of 166 HD patients. The LVDd values (5.06 ± 0.64 cm) of the non‐survivor group were significantly greater than in the survivor group (4.78 ± 0.71 cm). The area under the receiver operating characteristic curve for an LVDd cut‐off value of 5.01 cm was 0.6145 (P = 0.0234). The sensitivity and specificity of the LVDd threshold of 5.01 cm were 75.7% and 50.4%, respectively. The 4‐year survival rate was significantly lower in the group with LVDd ≥ 5.01 cm than in the group with LVDd < 5.01 cm (log‐rank test, P = 0.0047). Multivariate analysis with adjustments for clinical and echocardiographic parameters showed that increased LVDd was an independent predictor of all‐cause mortality (hazard ratio 2.363, 95% CI 1.320–4.228, P = 0.0013). The results of the present study showed that increased LVDd predicts the all‐cause mortality of chronic HD patients better than other echocardiographic parameters. Our findings suggest that LVDd measurements may be helpful for risk stratification and providing therapeutic direction for the management of HD patients.

Successful treatment with steroid and cyclosporine A in a patient with immunoglobulin A-proliferative glomerulonephritis with monoclonal immunoglobulin deposits: IgA-PGNMID with steroid and cyclosporine A

We report a case of glomerulonephritis with monoclonal immunoglobulin (Ig) A deposits as a form of monoclonal gammopathy of renal significance (MGRS) caused by monoclonal immunoglobulins without blood disorders in a 41‐year‐old woman. She developed lower leg oedema and was hospitalized because of nephrotic syndrome. Serum and urine were negative for M protein, and the free light chain κ/λ ratio was within the normal range. Renal histopathological findings included mesangial proliferation, endocapillary cell proliferation, and a double‐contour appearance of the capillary walls. Immunofluorescent staining indicated IgA and C3 deposits on the mesangium and capillary walls. Only λ chain and IgA1 deposits were noted. Fine granular sub‐endothelial deposits with no specific structure were observed under electron microscopy. The patient was diagnosed with IgA–proliferative glomerulonephritis with monoclonal immunoglobulin deposits (IgA‐PGNMID). The patient had decreased urine protein and sediment erythrocytes after she underwent two rounds of steroid pulse therapy and oral steroid therapy, but proteinuria and haematuria still remained. Four months later, the patient was administered 50 mg/day cyclosporine (CsA), and proteinuria and haematuria dramatically decreased. Only a few case reports have been published on IgA‐PGNMID. This case is rare in that the patient achieved successful treatment using a combination of steroids and CsA.