- Published on
Pathology - Focal segmental glomerulosclerosis
It is a primary glomerulopathy defined histologically by sclerosis affecting some, but not all, glomeruli (focal) and engaging just a section of the glomerular tuft (segmental). 2 Segmental glomerulosclerosis is simply an indicator of glomerular damage; it can occur in several circumstances and is not exclusive to FSGS. Meticulous elimination of alternative recognized etiologies of segmental glomerulosclerosis is necessary before to diagnosing FSGS, as this designation indicates a condition anticipated to swiftly advance to end-stage renal failure.
Epidemiology: Rare occurrence.
Aetiology • The cause remains unidentified, however the recurrence of the condition in transplanted kidneys implies the presence of a circulating factor. • An uncommon hereditary variant of the disease is also present. Pathogenesis Recent investigations indicate that the podocyte is the principal source of harm. Podocyte damage results in significant proteinuria, subsequently leading to glomerular scarring and quickly progressive renal failure.
Presentation of nephrotic syndrome.
Light microscopy • Affected glomeruli exhibit collapse of a portion of the tuft, replaced by sclerosis. • The sclerotic segment frequently demonstrates adhesion to the adjacent epithelial cells of Bowman’s space. •
Glomerulosclerosis is typically associated with tubulointerstitial fibrosis surrounding the affected glomerulus. Immunofluorescence • Non-specific trapping of IgM and C3 may be observed in regions of sclerosis. Electron microscopy reveals podocyte foot process effacement. • Immune deposits are absent.
Prognosis: Nearly all instances of FSGS are progressive, with the majority of patients attaining end-stage renal failure within a few years post-diagnosis.
It is a primary glomerulopathy defined histologically by sclerosis affecting some, but not all, glomeruli (focal) and engaging just a section of the glomerular tuft (segmental). 2 Segmental glomerulosclerosis is simply an indicator of glomerular damage; it can occur in several circumstances and is not exclusive to FSGS. Meticulous elimination of alternative recognized etiologies of segmental glomerulosclerosis is necessary before to diagnosing FSGS, as this designation indicates a condition anticipated to swiftly advance to end-stage renal failure.
Epidemiology: Rare occurrence.
Aetiology • The cause remains unidentified, however the recurrence of the condition in transplanted kidneys implies the presence of a circulating factor. • An uncommon hereditary variant of the disease is also present. Pathogenesis Recent investigations indicate that the podocyte is the principal source of harm. Podocyte damage results in significant proteinuria, subsequently leading to glomerular scarring and quickly progressive renal failure.
Presentation of nephrotic syndrome.
Light microscopy • Affected glomeruli exhibit collapse of a portion of the tuft, replaced by sclerosis. • The sclerotic segment frequently demonstrates adhesion to the adjacent epithelial cells of Bowman’s space. •
Glomerulosclerosis is typically associated with tubulointerstitial fibrosis surrounding the affected glomerulus. Immunofluorescence • Non-specific trapping of IgM and C3 may be observed in regions of sclerosis. Electron microscopy reveals podocyte foot process effacement. • Immune deposits are absent.
Prognosis: Nearly all instances of FSGS are progressive, with the majority of patients attaining end-stage renal failure within a few years post-diagnosis.
0 Comments
