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Differentiating Primary, Genetic, and Secondary FSGS in Adults: A Clinicopathologic Approach.

Authors
  • De Vriese, An S1
  • Sethi, Sanjeev2
  • Nath, Karl A3
  • Glassock, Richard J4
  • Fervenza, Fernando C5
  • 1 Division of Nephrology, AZ Sint-Jan Brugge-Oostende, Brugge, Belgium; [email protected] [email protected] , (Belgium)
  • 2 Department of Anatomic Pathology and.
  • 3 Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota; and.
  • 4 Geffen School of Medicine at the University of California, Los Angeles, California.
  • 5 Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota; and [email protected] [email protected]
Type
Published Article
Journal
Journal of the American Society of Nephrology
Publisher
American Society of Nephrology
Publication Date
Mar 01, 2018
Volume
29
Issue
3
Pages
759–774
Identifiers
DOI: 10.1681/ASN.2017090958
PMID: 29321142
Source
Medline
Keywords
License
Unknown

Abstract

FSGS describes a renal histologic lesion with diverse causes and pathogenicities that are linked by podocyte injury and depletion. Subclasses of FSGS include primary, genetic, and secondary forms, the latter comprising maladaptive, viral, and drug-induced FSGS. Despite sharing certain clinical and histologic features, these subclasses differ noticeably in management and prognosis. Without an accepted nongenetic biomarker that discriminates among these FSGS types, classification of patients is often challenging. This review summarizes the clinical and histologic features, including the onset and severity of proteinuria as well as the presence of nephrotic syndrome, that may aid in identifying the specific FSGS subtype. The FSGS lesion is characterized by segmental sclerosis and must be differentiated from nonspecific focal global glomerulosclerosis. No light microscopic features are pathognomonic for a particular FSGS subcategory. The characteristics of podocyte foot process effacement on electron microscopy, while helpful in discriminating between primary and maladaptive FSGS, may be of little utility in detecting genetic forms of FSGS. When FSGS cannot be classified by clinicopathologic assessment, genetic analysis should be offered. Next generation DNA sequencing enables cost-effective screening of multiple genes simultaneously, but determining the pathogenicity of a detected genetic variant may be challenging. A more systematic evaluation of patients, as suggested herein, will likely improve therapeutic outcomes and the design of future trials in FSGS.

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