title: Treatment Strategies of Adult Primary Focal Segmental Glomerulosclerosis: A Systematic Review Focusing on the Last Two Decades.
abstract: Adult primary focal segmental glomerulosclerosis (FSGS) remains a therapeutic challenge for the treating physician. With the advent of novel immunosuppressive measures, our arsenal of therapeutic options increased considerably. The aim of this review was to summarize reports published over the last two decades which reported on treatment outcome. Most reports included patients with a steroid-resistant (SR) disease course, yet the cohort with the highest unmet need, since persistent nephrotic range proteinuria is associated with a poor renal prognosis and portends a high risk of developing end-stage renal disease. While in first-line treatment, steroid treatment remains the recommended standard with an overall remission rate of 50% and higher, optimal treatment strategies for steroid-dependent/multirelapsing (SD/MR) and SR patients have to be defined. In both entities, calcineurin inhibitors showed good efficacy, while mycophenolate mofetil was less effective in SR cases compared to those with SD/MR. The same was true for rituximab, a monoclonal antibody targeting B-cells. In resistant cases, addition of extracorporeal treatment options or treatment with alkylating agents may be considered. To shape the future for treatment of FSGS, international collaborations to conduct larger clinical trials are needed to identify potential novel efficacious immunosuppressive or immunomodulatory therapies.
authors.affiliation: Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria.
authors.forename: Arno
authors.initials: A
authors.lastname: Beer
authors.affiliation: Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria.
authors.forename: Gert
authors.initials: G
authors.lastname: Mayer
authors.affiliation: Department of Internal Medicine IV (Nephrology and Hypertension), Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria.
authors.forename: Andreas
authors.identifier: 0000-0002-2945-2946
authors.initials: A
authors.lastname: Kronbichler
mesh_terms: D065095:Calcineurin Inhibitors / Q000627:therapeutic use*; D004351:Drug Resistance; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000139:chemically induced / Q000188:drug therapy* / Q000473:pathology; D000069283:Rituximab / Q000627:therapeutic use; D013256:Steroids / Q000009:adverse effects / Q000627:therapeutic use
chemical_list: D065095:Calcineurin Inhibitors; D013256:Steroids; D000069283:Rituximab