What is new in the pathogenesis and treatment of IgA glomerulonephritis

Table 4 Investigation agents in clinical development for the treatment of IgAN

Agent	Phase	Registration number	Mechanism of action
Inhibition of endothelin A receptor and angiotensin II subtype 1 receptor inhibitor
Sparsentan	III	NCT03762850	Endothelin A receptor and angiotensin II subtype 1 receptor inhibitor
Atrasentan	III	NCT04573478	Endothelin A receptor antagonist
Plasma cell depletion
Felzartamab	II	NCT05065970	Monoclonal IgG1 antibody targeting CD38
Bortezomib	NA	NCT05383547	Proteasome inhibitor that depletes plasma cells
Inhibition of BAFF/APRIL signaling
Blisibimod	II/III	NCT02062684	Monoclonal antibody against both soluble and membrane BAFF
Sibeprenlimab		NCT05248646	Monoclonal IgG2k antibody targeting APRIL
BION-1301	I/II	NCT03945318	Monoclonal IgG4 antibody targeting APRIL
Atacicept	IIb	NCT04716231	BAFF/APRIL dual inhibitor
Telitacicept	II	NCT04905212	BAFF/APRIL dual inhibitor
Povetacicept	I	NCT05034484	BAFF/APRIL dual inhibitor
Zigakibart (BION-1301)	III	NCT05852938	Monoclonal IgG4 antibody targeting APRIL
Inhibition of immune complex-activated complement activity
Avacopan	II	NCT02384317	Anti-C5ab receptor antagonist
Ravulizumab	II	NCT04564339	Long-acting C5-blocking antibody
Cemdisiran	II	NCT03841448	Small interfering RNA-targeting C5
APL-2	II	NCT03453619	Cyclic peptide inhibitor of C3 and C3b
Iptacopan	III	NCT04578834	Small-molecule inhibitor of complement factor B
IONIS-B-LRx	II	NCT04014335	Antisense inhibitor of complement factor B messenger ribonucleic acid
Narsoplimab	III	NCT03608033	Human monoclonal antibody against MASP-2

MASP-2: Mannan-associated lectin-binding serine protease-2; BAFF: B cell-activating factor; APRIL: A proliferation-inducing ligand.