Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis is caused by an injury to podocytes in the renal glomeruli.

May be primary (idiopathic) due to an unknown cause or secondary to an underlying condition, such as HIV, medicines, or a maladaptive response to decreased renal mass.

May be asymptomatic or present with nephrotic syndrome.

First-line therapy in primary focal segmental glomerulosclerosis with nephrotic-range proteinuria is corticosteroid therapy. The secondary form requires treatment of the underlying cause.

Corticosteroid-dependent and corticosteroid-resistant patients require treatment with other immunosuppressants such as a calcineurin inhibitor.

Prognosis is extremely variable and depends on the levels of proteinuria and plasma creatinine, the morphological subtype, the degree of renal fibrosis, and the response to therapy.

Focal segmental glomerulosclerosis is a morphological pattern of glomerular injury primarily directed at the podocytes in the renal glomeruli.[1]Jefferson JA, Shankland SJ. The pathogenesis of focal segmental glomerulosclerosis. Adv Chronic Kidney Dis. 2014 Sep;21(5):408-16. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4149756 http://www.ncbi.nlm.nih.gov/pubmed/25168829?tool=bestpractice.com ​ It manifests initially with proteinuria, which progresses to nephrotic syndrome and ultimately to end-stage renal failure.