Disease activity and cancer risk in inflammatory bowel disease associated with primary sclerosing cholangitis

Table 1 Demographical and clinical characteristics of the two groups

	PSC-IBD (n = 75)		IBD without PSC (n = 150)		P
Male (%)	53.3		53.3		1
Age at IBD diagnosis (yr) (mean + SD)	27.8 (14)		28.5 (14)		0.75
Age at PSC diagnosis (yr) (mean + SD)	35.8 (15.6)		NA
IBD Follow up (mo) (mean + SD)	172 (133)		132 (123)		0.025
Initial/Cumulative IBD location (%)
Pancolitis	41 (55.4)	49 (66.2)	74 (49.3)	91 (60.7)	0.39	0.65
Rectal sparing	18 (24.3)	15 (20.3)	30 (20)	20 (13.3)	0.46	0.18
Sigmoid colon involvement	64 (86.5)	68 (91.9)	132 (88.6)	138 (92.6)	0.65	0.85
Left colon involvement	60 (81.1)	68 (91.9)	113 (75.8)	130 (87.2)	0.38	0.3
Right colon involvement	55 (74.3)	61 (82.4)	93 (62.4)	109 (72.7)	0.076	0.13
Ileum involvement	9 (12)	14 (18.7)	19 (12.7)	36 (24)	0.88	0.36
Jejunum involvement	0	1 (1.3)	0	3 (2)	1	0.59
Upper gastrointestinal tract involvement	1 (1.3)	1 (1.3)	4 (2.7)	8 (5.3)	0.46	0.14
Extra-intestinal manifestations (%)
Articular	16 (21.3)		34 (22.7)		0.82
Oral aphtae	3 (4)		4 (2.7)		0.58
Skin	8 (10.7)		7 (4.7)		0.09
Muscular	0		1 (0.7)		0.67
Ophthalmological	0		4 (2.7)		0.19
At least one extra intestinal lesion	22 (29.3)		39 (26)		0.59
Initial/Final diagnosis (%)
CD	19 (25.3)	21 (28)	43 (28.7)	62 (41.3)	0.6	0.051
UC	40 (53.3)	42 (56)	94 (62.7)	80 (53.3)	0.18	0.7
IC	1 (1.3)	1 (1.3)	1 (0.7)	3 (2)	0.61	0.72
IBDu	15 (20)	11 (14.7)	12 (8)	5 (3.3)	0.009	0.002
Smoking At IBD diagnosis/After IBD diagnosis	4 (5.3)	6 (8)	45 (30)	49 (32.7)	< 0.001	< 0.001
Colorectal neoplasia (%)
Dysplasia	2 (2.7)		1 (0.7)		0.2
Carcinoma	8 (10.7)		1 (0.7)		< 0.001
Appendectomy (%)	9 (12.7)		30 (20.3)		0.25

PSC-IBD: Inflammatory bowel disease associated with primary sclerosing cholangitis; CD: Crohn’s disease; UC: Ulcerative colitis; IC: Indeterminate colitis; IBDu: Unclassified IBD; NA: Not applicable.

Table 2 Clinical features and outcomes of PSC-IBD patients

Clinical features	n = 75
Age at PSC diagnosis (yr) (SD)	35.8 (15.6)
Age at IBD diagnosis (yr) (SD)	27.8 (14)
Disease topography	Intrahepatic	42%
	Intra- and extrahepatic	55.10%
	Extrahepatic	2.90%
Morphologic examinations	ERCP	50.70%
	MRCP	77.30%
	Endoscopic ultrasound	17.30%
	Liver biopsy	81.30%
Delay between diagnosis and UDCA treatment (mo) (SD)	13.5 (38.8)
Complications	Acute cholangitis	15.10%
	Cholangiocarcinoma	2.80%
	Cirrhosis	20.80%
	Ascites	9.70%
	Gastrointestinal bleeding	5.70%
Non UDCA treatments	Corticosteroid	12.70%
	immunosuppressors	7%
	Liver transplantation	17.30%
Death	8.50%

PSC-IBD: Inflammatory bowel disease associated with primary sclerosing cholangitis; UDCA: Ursodeoxycholic acid; ERCP: Endoscopic retrograde cholangiopancreatography; MRCP: Magnetic resonance cholangiopancreatography.

Table 3 Univariate analysis for the risk of colorectal neoplasia n (%)

	Neoplasia	No neoplasia	Log rank
	(n = 12)	(n = 213)	P
Male gender	8 (66.7)	112 (52.6)	> 0.20
Age at IBD diagnosis below 16 yr	3 (25)	52 (24.4)	> 0.20
Age at IBD diagnosis below 40 yr	9 (75)	165 (77.5)	> 0.20
Initial diagnosis of CD	2 (16.7)	60 (28.2)	> 0.20
IBD familial history	1 (8.3)	27 (12.7)	> 0.20
Initial IBD location
Ileum involvement	1 (8.3)	27 (12.7)	> 0.20
Right colon involvement	9 (75)	139 (65.3)	> 0.20
Left colon involvement	9 (75)	164 (77)	> 0.20
Sigmoid colon involvement	12 (100)	184 (86.4)	0.12
Rectum involvement	10 (83.3)	166 (77.9)	> 0.20
Anoperineal involvement	0	21 (9.9)	> 0.20
5 ASA during more than 2 yr	6 (54.6)	79 (42.5)	> 0.20
Immunosuppressant during more than 2 yr	3 (25)	69 (32.4)	> 0.20
Appendectomy before IBD onset	2 (16.7)	37 (17.4)	> 0.20
Current smoking	2 (16.7)	53 (24.9)	> 0.20
Extra-intestinal manifestations	2 (16.7)	61 (28.6)	0.09
PSC	10 (83.3)	65 (30.5)	0.004

IBD: Inflammatory bowel disease associated; PSC: Primary sclerosing cholangitis; CD: Crohn’s disease.