Advances in the pathophysiology and treatment of focal segmental glomerulosclerosis: The importance of a timely and tailored approach

Table 2 Summary of ongoing clinical trials and investigational therapies in focal segmental glomerulosclerosis

Clinical trial	Targeted pathway	Findings/observations
DUET study	Endothelin and RAS receptors	Significant reduction in proteinuria observed; effectiveness on eGFR progression remains under evaluation
DUPLEX study	Sparsentan (endothelin and RAS)	Sustained antiproteinuric effect; no significant reduction in eGFR decline over two years
VX-147 trial	APOL1-targeting	Promising results in patients with APOL1 mutations; significant proteinuria reduction observed
Rituximab trials	B-cell depletion	Reduced relapses in steroid-dependent patients; limited efficacy in steroid-resistant cases
Anti-nephrin trials	Circulating anti-nephrin antibodies	Remission seen in patients with high antibody levels; further studies required to confirm pathogenic role
Adrenocorticotropic hormone gel studies	Mechanism not fully understood	Promising results in steroid-dependent patients; additional confirmation needed in larger clinical trials

APOL1: Apolipoprotein 1; EGFR: Estimated glomerular filtration rate; RAS: Renin-angiotensin system.