Abstract

Abstract Background and Aims Maintaining remission in a minority of adult patients with minimal change disease remains challenging. Prolonged corticosteroid use is associated with severe adverse effects. A limited number of observational studies suggest that rituximab may be an alternative therapeutic option for remission maintenance, allowing steroid withdrawal. Method We retrospectively analysed the data of all adult patients with minimal change disease, who received rituximab in our centre between 2017 and 2022. Results A total of 14 adults with minimal change disease were treated with rituximab, six of whom had childhood-onset nephrotic syndrome. Six patients were steroid dependent. The median number of relapses was 6.5 (IQR 4.5-15.2) prior to rituximab. Thirteen patients had received at least ≥ 2 different immunosuppressive agents during their disease. Corticosteroid-related adverse events, including osteopenia, hypertension, cataract and diabetes mellitus were observed in 10 subjects. Rituximab was administered in a median time of 10.6 years (IQR 4.5-16.3) after diagnosis. All patients received 2 doses of 1g rituximab 2 weeks apart. The median age at rituximab administration was 31 years (IQR 18.7-49.0); all patients were in remission. Besides corticosteroids, concomitant immunosuppressive therapy included calcineurin inhibitors (n = 6) and mycophenolate acid analogues (n = 1). The median follow-up time after the first dose of rituximab was 44 months (IQR 9.5-67.2). Relapses occurred in 6/14 patients in a median time of 17.5 months (IQR 10.2-26.5). The number of relapses per year decreased to 0.27 from 0.87 (p < 0.001) after rituximab. Six patients received a repeat course of rituximab. One patient developed Pneumocystis Jirovecii pneumonia, that was successfully treated with cotrimoxazole. At the last follow-up visit, corticosteroids were discontinued in 8 out of 14 patients and calcineurin inhibitors in 3 out of 7 patients. Conclusion Rituximab is a reasonable therapeutic approach for maintaining remission and avoiding steroid toxicity in adult minimal change disease. Randomized controlled studies are needed to further evaluate the safety and efficacy of rituximab in these patients.

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