Open AccessFocal segmental glomerular sclerosis can be effectively treated using an intensive B-cell depletion therapy
Author(s) -
Dario Roccatello,
Alessandra Baffa,
Carla Naretto,
Antonella Barreca,
Raffaella Cravero,
Elisabetta Roscini,
Savino Sciascia,
Roberta Fenoglio
Publication year - 2022
Publication title -
clinical kidney journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.033
H-Index - 40
eISSN - 2048-8513
pISSN - 2048-8505
DOI - 10.1093/ckj/sfac207
Subject(s) - medicine , rituximab , proteinuria , nephrotic syndrome , urology , cyclophosphamide , creatinine , renal function , minimal change disease , focal segmental glomerulosclerosis , podocyte , gastroenterology , kidney , chemotherapy , lymphoma
Focal segmental glomerular sclerosis (FSGS) is a histologic lesion rather than a specific disease entity and represents a cluster of different conditions affecting both children and adults that includes primary, secondary and genetically mediated forms. These forms can be distinguished by electron microscopy and genetic assessment and show different responsiveness to steroids and immunosuppressants. Despite some promising effects of rituximab in nephrotic syndrome in children, the results in adults with FSGS are disappointing. Our group previously explored the effectiveness of rituximab in eight adult patients with unselected forms of FSGS and achieved a consistent reduction in proteinuria in one case. Following this experience, we developed an alternative therapeutic option intended to enhance the potential of rituximab with the support of other synergic drugs. We herein report the results of this therapeutic protocol (six administrations of rituximab plus two of intravenous cyclophosphamide plus glucocorticoids) in seven prospectively enrolled patients with extensive podocyte effacement and recurrent relapses or steroid dependence.