Optimal management of primary focal segmental glomerulosclerosis in adults

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.

Chlormethine Hydrochloride is Not Inferior to Tacrolimus in Treating Steroid-Resistant Nephrotic Syndrome

“…Some retrospective studies have suggested that steroid and alkylating agents, such as chlorambucil, can favor remission of FSGS [34,35]. Alkylating agents are associated with higher remission rates and a longer time of remission than calcinuurin inhibitors [36], and the high costs of tacrolimus treatment may be less effective in patients with FSGS than other types, such as minimal change nephropathy [32].…”

Rituximab use in adult glomerulopathies and its rationale

“…In steroid resistant, steroid-dependent or steroid-contraindicated patients, other immunosuppressive therapies are suggested 46 . Adverse effects of the long-term use of such toxic and "non-specific" therapies have led to a demand for more selective immunomodulating and immunosuppressive regimens 47,48 .…”

The Metabolomics Signature Associated with Responsiveness to Steroid Therapy in Focal Segmental Glomerulosclerosis: A Pilot Study

“…Clinically, FSGS is often associated with nephrotic syndrome, which is characterized by proteinuria of > 1 g/ m 2 /24 h, hypoalbuminemia, generalized edema, and hyperlipidemia 5,6 . The other clinical presentations of FSGS are hypertension, microscopic hematuria, renal failure, and serum creatinine increment 7 . The first-line treatment strategy for FSGS is oral corticosteroids, although it still seems to be far from satisfactory and remains a challenge for nephrologists 7 .…”

“…The other clinical presentations of FSGS are hypertension, microscopic hematuria, renal failure, and serum creatinine increment 7 . The first-line treatment strategy for FSGS is oral corticosteroids, although it still seems to be far from satisfactory and remains a challenge for nephrologists 7 . The rate of spontaneous remission in this glomerular disease is <5%, whereas a complete or partial remission with steroid therapy occurs in 40-60% of cases 7,8 .…”

“…The first-line treatment strategy for FSGS is oral corticosteroids, although it still seems to be far from satisfactory and remains a challenge for nephrologists 7 . The rate of spontaneous remission in this glomerular disease is <5%, whereas a complete or partial remission with steroid therapy occurs in 40-60% of cases 7,8 . Other immunosuppressive drugs such as calcineurin inhibitors and cytotoxic agents (e.g., cyclophosphamide or chlorambucil) are also frequently used 9,10 .…”