Focal segmental glomerulosclerosis
Primary FSGS is itself a heterogeneous disease, likely with multiple pathogenic mechanisms resulting in a similar histologic pattern of injury. Secondary causes include chronic reflux nephropathy, chronic hypertension, and obesity.
Clinical presentations vary widely, but typically individuals with primary FSGS present with the nephrotic syndrome. Secondary FSGS is typically associated with less severe proteinuria, often without hypoalbuminemia or peripheral edema. Patients may require renal transplantation, which may have a high frequency of failure from recurrence of FSGS. An early trial of the small-molecule drug inaxaplin significantly reduced proteinuria for patients with apolipoprotein L1 (APOL1) risk variants G1 and G2, which commonly occur in people descended from sub-Saharan Africa. Studies are ongoing.
N26.9 – Renal sclerosis, unspecified
236403004 – Focal Segmental Glomerulosclerosis
- Minimal-change disease – sometimes can be very difficult to distinguish from early FSGS, but if Ki67 stain is completed and is positive, this is specific for FSGS
- Membranous nephropathy
- Diabetic glomerulosclerosis (see diabetic nephropathy)
- Lupus nephritis Class V (see systemic lupus erythematosus)
- Renal amyloidosis (AL amyloidosis, AA amyloidosis)
- Light-chain deposition disease
- IgA nephropathy (less common presentation)
- Membranoproliferative glomerulonephritis (less common presentation)
- Fibrillary glomerulonephritis (rare)
- Immunotactoid glomerulonephritis (rare)
- Fabry disease (rare presentation of a rare disease)