Abstract
BACKGROUND: The treatment of idiopathic refractory nephrotic syndrome (IRNS) remains a difficult problem in clinical practice. This study aims to determine the efficacy and safety of combining low-dose glucocorticoids with rituximab in IRNS treatment. METHODS: This prospective, single-center cohort study enrolled 60 patients who were diagnosed with refractory IRNS with minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS) and treated at First Affiliated Hospital of Sun Yat-sen University. All patients received a treatment regimen consisting of rituximab (375 mg/m(2)/week × 4) and low-dose glucocorticoids. RESULTS: 46 complete remissions and 4 partial remissions were observed within 6 months of treatment. Within 12 months of treatment, 48 patients achieved complete remission, and 4 achieved partial remission. The complete remission rate for steroid-dependent/frequently relapsing nephrotic syndrome was significantly higher than that for steroid-resistant nephrotic syndrome (88.24% vs. 33.33%, p < .01). Following up for 12 months, 16 patients relapsed, accounting for 30.76% of the total, with a mean time to relapse of 10.97 months. Compared with baseline data, 24-hour urine protein quantification, total cholesterol and triglycerides significantly decreased, while serum albumin, globulin and IgG significantly increased at 12 months after treatment. All follow-ups were without serious adverse events. Twenty-four patients experienced infusion-related adverse reactions, which could be relieved by slowing down the infusion rate or suspending the infusion. Nine patients experienced infection-related adverse reactions; six of them were relieved with antibiotic treatment, and 3 patients were controlled by symptomatic treatment. CONCLUSIONS: Rituximab combined low-dose glucocorticoids therapy is effective and safe in idiopathic refractory nephrotic syndrome.