These pathological and clinical findings were compatible with the C1qN diagnostic criteria proposed by Jennette and Hipp [2]. The pathogenesis of C1qN involves the deposition of C1q, IgG, IgM, and C3 in the mesangial cells [2,13]. Therefore, to suppress IgG and/or IgM production and then inactivate the classical pathway through C1q, MMF at a dosage of 720 mg/day (1176 mg/m2/day) was initiated with PSL 28 mg/day. The MMF dose was determined with reference to the 1200 mg/m2/day recommended by KDIGO for nephrotic syndrome (NS) [11]. Here, CD40LG is linked to nephrotic syndrome.