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Review
. 2017 May 10:10:97-107.
doi: 10.2147/IJNRD.S126844. eCollection 2017.

Optimal management of primary focal segmental glomerulosclerosis in adults

Séverine Beaudreuil  1   2 , Hans Kristian Lorenzo  1   2 , Michele Elias  1 , Erika Nnang Obada  1 , Bernard Charpentier  1   2 , Antoine Durrbach  1   2
Affiliations
Review

Optimal management of primary focal segmental glomerulosclerosis in adults

Séverine Beaudreuil et al. Int J Nephrol Renovasc Dis. .

Abstract

Focal segmental glomerulosclerosis (FSGS) is a frequent glomerular kidney disease that is revealed by proteinuria or even nephrotic syndrome. A diagnosis can be established from a kidney biopsy that shows focal and segmental glomerulosclerosis. This histopathological lesion may be caused by a primary podocyte injury (idiopathic FSGS) but is also associated with other pathologies (secondary FSGS). The first-line treatment for idiopathic FSGS with nephrotic syndrome is a prolonged course of corticosteroids. However, steroid resistance or steroid dependence is frequent, and despite intensified immunosuppressive treatment, FSGS can lead to end-stage renal failure. In addition, in some cases, FSGS can recur on a graft after kidney transplantation: an unidentified circulating factor may be implicated. Understanding of its physiopathology is unclear, and it remains an important challenge for the scientific community to identify a specific diagnostic biomarker and to develop specific therapeutics. This study reviews the treatment of primary FSGS and the recurrence of FSGS after kidney transplantation in adults.

Keywords: circulating factor; glomerulosclerosis; kidney transplantation; treatment.

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Conflict of interest statement

Disclosure The authors report no conflicts of interest in this work.

Figures

Figure 1
Figure 1
Algorithm for the management of primary FSGS. Abbreviation: ACTN4, α-Actinin 4; CD2AP, CD2-associated protein; FSGS, focal segmental glomerulosclerosis; WT1, Wilms tumor protein.
See this image and copyright information in PMC

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