Small duct primary sclerosing cholangitis: A discrete variant or a bridge to large duct disease, a practical review

Table 1 Baseline characteristics and outcomes of small duct primary sclerosing cholangitis cohorts

Ref.	Study design, population	Study location	Total sdPSC	Females	Age at Dx (yr)1	F/U (mo)1	UC	CD	HCC	CCA	AIH	LdPSC conversion	Conversion time (mo)1	Treatment			Liver transplant	Death
														UDCA	Steroids	AZA
Wee et al[31], 1984	Retrospective, Adult	Mayo Clinic	3	1	34	-	3	0	0	3	0	0	-	-	-	-	0	3
Broomé et al[11], 2002	Retrospective, Adult	Sweden	32	12	39	63	13	3	1	0	0	4	115	7	7	6	1	0
Björnsson et al[7], 2002	Retrospective, Adult	Oslo/Oxford	33	14	38	106	20	7	0	0	0	4	-	7	-	-	2	2
Angulo et al[9], 2002	Longitudinal cohort, Adult	Mayo Clinic	18	7	39	126	14	3	0	0	0	3	122	7	0	0	2	1
Nikolaidis et al[39], 2005	Retrospective, Adult	Greece	6	-	32	26	2	1	0	0	0	0	-	5	0	5	0	0
Charatcharoenwitthaya et al[12], 2007	Longitudinal cohort, Adult	Mayo Clinic	42	14	35	57	13	3	0	0	0	3	-	30	-	5	6	1
Miloh et al[32], 2009	Retrospective, Pediatric	New York	16	5	10	78	4	4	0	0	5	0	-	16	-	-	2	0
Olsson et al[28], 2009	Retrospective, Adult	Sweden	7	3	24	71	4	0	0	0	7	0	-	-	6	6	0	0
Singal et al[10], 2011	Retrospective, Adult	New York/Florida	25	12	37	39	13	0	0	0	0	0	-	15	8	12	1	1
Fevery et al[17], 2016	Retrospective, Adult	Belgium	33	-	35	144	3	9	0	0	0	0	-	-	-	-	-	-
Valentino et al[13], 2016	Longitudinal cohort, Ped	Boston	24	-	10	44	-	-	-	-	-	6	72	-	-	-	2	2
Liu et al[40], 2017	Retrospective, Adult	Australia	10	-	41	96	-	-	0	0	-	-	-	-	-	-	-	-
Deneau et al[27], 2017	Retrospective, Pediatric	Multi-institutional	98	34	10.5	-	-	-	-	-	36	-	-	75	-	-	-	-
Weismüller et al[30], 2017	Retrospective, Adult	Multi-institutional	254	96	37	-	67	24	0	0	-	-	-	-	-	-	-	-
Umetsu et al[41], 2019	Retrospective, Pediatric	Japan	3	-	9	66	-	-	0	0	-	-	-	-	-	-	0	0
Ringe et al[14], 2020	Retrospective, Adult	Germany	16	7	29	127	6	4	-	-	1	5	144	16	-	-	-	-

¹Indicates an average.

AIH: Autoimmune hepatitis; AZA: Azathioprine; CCA: Cholangiocarcinoma; CD: Crohn’s Disease; Conversion time (average time from sdPSC diagnosis to ldPSC conversion); F/U: Follow-up (months after initial diagnosis); HCC: Hepatocellular carcinoma; LdPSC: Large duct primary sclerosing cholangitis; SdPSC: Small duct primary sclerosing cholangitis; UC: Ulcerative colitis; UDCA: Ursodeoxycholic acid; -: Unknown.