Available and incoming therapies for idiopathic focal and segmental glomerulosclerosis in adults

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Abstract

Focal and segmental glomerulosclerosis (FSGS) is a histological pattern clinically characterized by nephrotic proteinuria, hypoalbuminemia, edema and dyslipidemia. Approximately 50% of patients progress to end-stage renal disease within 5–10 years, particularly those not responding to the therapies. FSGS pathogenesis is largely unknown and therapy is symptomatic and unspecific. The podocyte is considered as the pathogenetic main target and FSGS is now categorized as a podocytopathy together with minimal change disease, diffuse mesangial proliferation and collapsing glomerulonephritis. This paper provides an overview on the treatment of idiopathic FSGS in adults, citing the latest published trials and the most reliable pathogenetic hypotheses of the disease. A large part of the review then focuses on emerging therapies, specifying for each new drug the assumed mechanism of action and the data available in the literature on the drug’s use in experimental animals and humans.

Original languageEnglish
Pages (from-to)37-45
Number of pages9
JournalJournal of Nephrology
Volume31
Issue number1
DOIs
Publication statusPublished - 2018

Fingerprint

Focal Segmental Glomerulosclerosis
Lipoid Nephrosis
Podocytes
Hypoalbuminemia
Therapeutics
Dyslipidemias
Glomerulonephritis
Proteinuria
Pharmaceutical Preparations
Chronic Kidney Failure
Edema

Keywords

  • Focal and segmental glomerulosclerosis
  • Nephrotic syndrome
  • Podocyte
  • Therapy

ASJC Scopus subject areas

  • Nephrology

Cite this

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abstract = "Focal and segmental glomerulosclerosis (FSGS) is a histological pattern clinically characterized by nephrotic proteinuria, hypoalbuminemia, edema and dyslipidemia. Approximately 50{\%} of patients progress to end-stage renal disease within 5–10 years, particularly those not responding to the therapies. FSGS pathogenesis is largely unknown and therapy is symptomatic and unspecific. The podocyte is considered as the pathogenetic main target and FSGS is now categorized as a podocytopathy together with minimal change disease, diffuse mesangial proliferation and collapsing glomerulonephritis. This paper provides an overview on the treatment of idiopathic FSGS in adults, citing the latest published trials and the most reliable pathogenetic hypotheses of the disease. A large part of the review then focuses on emerging therapies, specifying for each new drug the assumed mechanism of action and the data available in the literature on the drug’s use in experimental animals and humans.",
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