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2017 ; 12
(3
): 502-517
Nephropedia Template TP
Clin J Am Soc Nephrol
2017[Mar]; 12
(3
): 502-517
PMID28242845
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Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease
worldwide. The presumed etiology of primary FSGS is a plasma factor with
responsiveness to immunosuppressive therapy and a risk of recurrence after kidney
transplant-important disease characteristics. In contrast, adaptive FSGS is
associated with excessive nephron workload due to increased body size, reduced
nephron capacity, or single glomerular hyperfiltration associated with certain
diseases. Additional etiologies are now recognized as drivers of FSGS:
high-penetrance genetic FSGS due to mutations in one of nearly 40 genes,
virus-associated FSGS, and medication-associated FSGS. Emerging data support the
identification of a sixth category: APOL1 risk allele-associated FSGS in
individuals with sub-Saharan ancestry. The classification of a particular patient
with FSGS relies on integration of findings from clinical history, laboratory
testing, kidney biopsy, and in some patients, genetic testing. The kidney biopsy
can be helpful, with clues provided by features on light microscopy (e.g,
glomerular size, histologic variant of FSGS, microcystic tubular changes, and
tubular hypertrophy), immunofluorescence (e.g, to rule out other primary
glomerulopathies), and electron microscopy (e.g., extent of podocyte foot process
effacement, podocyte microvillous transformation, and tubuloreticular
inclusions). A complete assessment of renal histology is important for
establishing the parenchymal setting of segmental glomerulosclerosis,
distinguishing FSGS associated with one of many other glomerular diseases from
the clinical-pathologic syndrome of FSGS. Genetic testing is beneficial in
particular clinical settings. Identifying the etiology of FSGS guides selection
of therapy and provides prognostic insight. Much progress has been made in our
understanding of FSGS, but important outstanding issues remain, including the
identity of the plasma factor believed to be responsible for primary FSGS, the
value of routine implementation of genetic testing, and the identification of
more effective and less toxic therapeutic interventions for FSGS.
|Angiotensin Receptor Antagonists/therapeutic use
[MESH]
|Angiotensin-Converting Enzyme Inhibitors/therapeutic use
[MESH]
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