Table 1.

Drug therapy for cholestatic diseases in clinical trials.

		Drug Name	Indication	Current Status	Clinical Trial	Sponsor [Reference]
FXR agonists	Bile acids	UDCA (Actigall/Ursodiol/Ursofalk)	ICP	Phase III Phase IV	NCT01576458 NCT01510860	Turku University Hospital [68] Pharma GmbH [69]
			PBC	Approved		Sanofi-Synthelabo [70]
			PFIC3			[71]
		Nor-UDCA	PSC	Phase II	NCT01755507	Pharma GmbH [41]
		TUDCA (Taurolite)	PBC	Phase III	NCT01857284	Beijing Friendship Hospital [43]
		OCA (INT-747/Ocaliva)	PBC	Phase II Phase III	NCT00570765 NCT01473524	Intercept Pharmaceuticals [44,45,72,73]
			PSC	Phase II	NCT02177136
	Non-bile acids	Cilofexor (CILO)	PSC	Phase I/II	NCT02943460	Gilead Sciences [49]
		Tropifexor (LJN452)	PBC	Phase II	NCT02516605	Novartis Pharmaceuticals [74]
		EDP-305	PBC	Phase II	NCT03394924	Enanta Pharmaceuticals
ASBT inhibitors		Odevixibat (A4250)	ALGS	Phase III	NCT04674761	Albireo [75,76]
			PFIC	Approved
		Maralixibat (LUM001)	ALGS	Approved		Mirum Pharmaceuticals, Inc. [61]
			PFIC	Phase III	NCT02057718 NCT03905330
		Linerixibat (GSK2330672)	PBC	Phase III	NCT02966834 NCT04167358	GlaxoSmithKline [77,78]
		Volixibat (SHP626)	ICP PBC PSC	Phase II	NCT04718961 NCT05050136 NCT04663308	Mirum Pharmaceuticals, Inc.
Other pharmacotherapeutic agents		Aldafermin (NGM282)	PBC	Phase II	NCT02026401	NGM Biopharmaceuticals, Inc. [79]
			PSC		NCT02704364
		Bezafibrate	PBC	Phase III	NCT01654731	Hôpitaux de Paris [80]
		Elafibranor	PBC	Phase II	NCT03124108	Genfit [81]
		Seladelpar (MBX-8025)	PBC	Phase III	NCT03602560	CymaBay Therapeutics, Inc. [82]