Focal and Segmental Glomerulosclerosis

Primary focal segmental glomerulosclerosis (p-FSGS) is an orphan renal disease in both children and adults, but it is a frequent cause of proteinuria. The secondary form is due to hyperfiltration, which results from increased glomerular capillary pressure, viral infection, or toxin exposure. Primary forms typically do not respond well to corticosteroid treatment and may even be corticosteroid-dependent; in such cases, a combination of calcineurin inhibitors and corticosteroids or rituximab and corticosteroids may be beneficial, but corticosteroid treatment remains the first-line treatment. The primary form often recurs after transplantation.