Rituximab in The Management of Pediatric Steroid-Resistant Nephrotic Syndrome: A Systematic Review

Efficacy of Rituximab in Treatment-Resistant Focal Segmental Glomerulosclerosis With Elevated Soluble Urokinase-Type Plasminogen Activator Receptor and Activation of Podocyte β3 Integrin

“…In a recent systematic review that included 226 children, response to J o u r n a l P r e -p r o o f Rituximab was noted in 39.2% of children with steroid-resistant nephrotic syndrome secondary to FSGS. 32 Studies that include only adult patients are more limited. A recent systematic review and meta-analysis included 16 observational studies that described the outcome after Rituximab therapy in 51 adult patients with FSGS.…”

Changing epidemiology of nephrotic syndrome in Nigerian children: A cross-sectional study

“…Most children with SRNS have focal segmental glomerulosclerosis (FSGS) and about 10-30% are reported to have a single gene disorder as the cause of the nephrotic syndrome [4]. These children require additional second line agents such as calcineurin inhibitor (CNI), and monoclonal antibodies to achieve complete or partial remission of the nephrotic syndrome and halt disease progression [5,6]. In the absence of remission following treatment with immunosuppressants, most children with SRNS/FSGS develop progressive disease and will reach end stage kidney disease within 5 to 10 years of diagnosis [7,8].…”

Current understandings in treating children with steroid-resistant nephrotic syndrome

“…Although rituximab is generally safe and well tolerated in most children, there are potentially serious adverse events that require caution: hypogammaglobulinemia, late-onset neutropenia, hepatitis B reactivation, serious infusion-related adverse events and infections, the latter with potentially fatal outcome with reports of Pneumocystis jirovecii pneumonia and progressive multifocal leukoencephalopathy [8, 58,59,61,62,117,118] hematologic adverse effects are reported more often and more severely in younger children. In previous studies with SRNS [41] as well as with SDNS [119], those patients who developed severe neutropenia and hypogammaglobulinemia were aged under 10 years.…”

“…However, contrastingly, non-response rates of 81% [ 63 ] and 71% [ 112 ] were found by other publications. A recent systematic review of RCTs reported remission with RTX in 46% of SRNS (63% with MCD and 39% with FSGS) and sustained remission among responders in up to ~ 94% [ 58 ]. However, an open-label RCT in patients with refractory SRNS by Magnasco et al demonstrated no additional benefit of RTX over CNI [ 63 ].…”

“…Although rituximab is generally safe and well tolerated in most children, there are potentially serious adverse events that require caution: hypogammaglobulinemia, late-onset neutropenia, hepatitis B reactivation, serious infusion-related adverse events and infections, the latter with potentially fatal outcome with reports of Pneumocystis jirovecii pneumonia and progressive multifocal leukoencephalopathy [ 8 , 58 , 59 , 61 , 62 , 117 , 118 ]. In the study by Fujinaga et al, although all six patients achieved complete remission, four of the six had hypogammaglobulinemia requiring intravenous immunoglobulins (IVIG), and one of them developed persistent hypogammaglobulinemia demanding regular IVIG treatment (despite re-emergence of B-cells in the blood) [ 41 ].…”