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Background:

Multidrug-resistant nephrotic syndrome (MRNS) accounts for 15% of overall cases of nephrotic syndrome (NS) in childhood. MRNS is associated with high risk of kidney failure and recurrence after kidney transplant (KT). Common treatments, including rituximab and plasma exchange (PEX), are poorly effective.

Methods:

We ran a phase II proof-of concept clinical study (NCT05704400) including patients with MRNS (lack of antiproteinuric effect of therapy based on steroid plus CNI and MMF for at least 12 months before enrolment) and post-transplant FSGS recurrence (FSGSr) resistant to rituximab and PEX. We administered rituximab (single infusion 350mg/m 2, iv)+ daratumumab (single 16mg/kg infusion). Primary outcome was complete (CR) or partial remission (PR defined as> 50% proteinuria reduction).

Results:

We enrolled 8 patients: 5 with MRNS and 3 FSGSr (Fig. 1a). MRNS resulted resistant to …