A study from the Columbia group demonstrated that the most common FSGS variant in all FSGS patients was NOS or perihilar variant (62.3%) followed by collapsing (23.7%), tip (9.4%), and cellular (3%) variants [30]. Another study reported similar data: NOS, 42%; perihilar, 26%; collapsing, 11%; tip, 17%; and cellular, 3% [31].
FSGS involves damage to the renal podocytes such that larger molecules, most notably proteins, are filtered and lost through the kidney. Thus, many of the signs and symptoms of FSGS are related to protein loss. On histology, FSGS manifests as damage ( sclerosis) to segments of glomeruli; moreover, only a portion of glomeruli are affected.
Furthermore, genetic testing is more likely to identify a genetic basis of FSGS in young children, patients with syndromic disease, or a positive family history.
Secondary FSGS is caused by an identifiable stress or toxin that injures podocytes. Many causes of secondary FSGS contribute to podocyte injury through hyperfiltration, which is a scenario of excess filtration by renal glomeruli.
FSGS is primarily a disease of the renal glomerulus, the site of filtration of ions and solutes. Podocytes are specialized cells lining the Bowman's capsule that contribute to the filtration barrier, preventing molecules larger than 5 nm from being filtered. FSGS involves damage to the renal podocytes such that larger molecules, ...
Thus, many of the signs and symptoms of FSGS are related to protein loss.
On histology, FSGS manifests as damage ( sclerosis) to segments of glomeruli; moreover, only a portion of glomeruli are affected. The focal and segmental nature of disease seen on histology help to distinguish FSGS from other types of glomerular sclerosis. FSGS can be classified by the putative cause of damage to podocytes.
Focal segmental glomerulosclerosis (FSGS), also known as “focal glomerular sclerosis” or “focal nodular glomerulosclerosis ,” is a histopathologic finding of scarring (sclerosis) of glomeruli and damage to renal podocytes. This process damages the filtration function of the kidney, resulting in protein loss in the urine.
Prognosis. The majority of untreated cases of FSGS will progress to end-stage kidney disease. Important prognostic factors include the degree of proteinuria and initial response to therapy. Patients with nephrotic-range (>3.5 g/day) proteinuria have over a 50% rate of progression to end-stage kidney disease at 10 years.
First-line treatment for primary FSGS consists of anti-inflammatory drugs. Specifically, glucocorticoids are begun in patients manifesting with nephrotic -range proteinuria (>3.5 g/day). For patients who maintain nephrotic-range proteinuria despite glucocorticoids, or for patients who demonstrate glucocorticoid intolerance, calcineurin inhibitors (e.g., tacrolimus) are initiated. Successful treatment is defined as a drop in proteinuria to sub-nephrotic ranges.
Diagnosis of FSGS is made by renal biopsy that includes at least 15 serial cuts with at least 8 glomeruli. Histologic features include sclerosis (scarring) of a portion (average: 15%) of the glomerular space, with only a portion of glomeruli manifesting any sclerosis.