Pre-emptive rituximab and plasma exchange does not prevent disease recurrence following living donor renal transplantation in high-risk idiopathic SRNS

BACKGROUND: Children with non-genetic steroid-resistant nephrotic syndrome (SRNS) are at high risk of disease recurrence (DR) and graft loss following renal transplant (RT). Although pre-emptive plasma exchange (PE) and rituximab have been suggested to prevent DR, there is insufficient published data to support this practice. The aim is to study the role of pre-emptive PE and rituximab in the prevention of DR in children with non-genetic SRNS undergoing living donor (LD) RT. METHODS: Prospective single-centre study of four consecutive children (age 6-17 years) with non-genetic SRNS (including two with previous graft loss due to DR) who underwent LD RT between July 2014 and September 2016. All patients received a single dose of rituximab 375 mg/m(2) 2-4 weeks prior to the RT and four sessions of PE in the week prior to RT. All patients had previously undergone bilateral native nephrectomies. RESULTS: All children had early DR (2-26 days) following LD RT. Following early initiation of PE, three children achieved partial remission (PR) or complete remission (CR) 5-22 days after commencing treatment. One child continued to have heavy proteinuria along with graft dysfunction despite 52 sessions of PE and lost the graft 5 months after RT. At the latest follow-up of 36-60 months following RT, one child remains in CR and two are in PR. The latest eGFR was 45-104 ml/min/1.73m(2). CONCLUSIONS: Pre-emptive rituximab and PE does not prevent DR in high-risk non-genetic SRNS. Prompt initiation of PE following DR appears to achieve PR or CR in the majority of patients.