Primary sclerosing cholangitis associated colitis: Characterization of clinical, histologic features, and their associations with liver transplantation

doi:10.3748/wjg.v26.i28.4126

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World Journal of Gastroenterology

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World J Gastroenterol. Jul 28, 2020; 26(28): 4126-4139
Published online Jul 28, 2020. doi: 10.3748/wjg.v26.i28.4126

Table 1 Clinical characteristics of Primary sclerosing cholangitis-Ulcerative colitis and Primary sclerosing cholangitis-Crohn disease patients (in all available patients, treatment data not mutually exclusive), n (%)

	PSC-UC	UC	P value	PSC-CD	CD	P value	P value (PSC-UC vs PSC-CD)
Male	17 (65)	18 (64)	0.9	5 (83)	2 (22)	0.03^a	0.4
Age at the time of biopsy (yr) (mean ± SD)	37 ± 14.61	41 ± 15.55	0.4	38 ± 14.93	32 ± 10.21	0.2	0.8
Age at IBD diagnosis (yr) (mean ± SD)	27± 14.38	30 ± 10.61	0.3	33 ± 14.77	22 ± 10.48	0.09	0.2
Age at PSC diagnosis (yr) (mean ± SD)	32 ± 13.96	NA	NA	31± 12.87	NA	NA	0.8
Interval between PSC and IBD diagnoses (yr) (mean ± SD)	9 ± 10.45	NA	NA	1.4 ± 1.6733	NA	NA	0.1
IBD duration at biopsy (yr) (mean ± SD)	10.8 ± 10.84	9 ± 9.19	0.5	7.00 ± 5.18	9.8 ± 4.06	0.5	0.3
PSC duration (yr) (mean)	6	NA	NA	12.33	NA	NA	0.8
Medical treatment
Ursodiol	9 (35)	0	0.0006^a	2 (33)	0	0.1	1
Steroids	8 (31)	19 (68)	0.01^a	2 (33)	2 (22)	1	1
ASA	15 (58)	17 (61)	1	2 (33)	6 (67)	0.3	0.4
Anti-TNF	5 (19)	7 (25)	0.7	1 (17)	7 (78)	0.04^a	1
Immunomodulators	4 (15)	8 (29)	0.3	2 (33)	3 (33)	1	0.3
Colectomy	10 (38)	19 (68)	0.03^a	0	1 (11)	0.9	0.9
OLT	10 (38)	NA	NA	0	NA	NA	0.1
Follow-up duration (yr) (mean ± SD)	8.2 ± 4.61	8.2 ± 5.75	0.9	4.8 ± 2.23	5.9 ± 3.14	0.7	0.1
Development of dysplasia/cancer	4 (15)	5 (18)	0.8	0	0	1	0.9
Resection used in histologic and IHC analysis	6 (23)	9 (32)	0.4	0	3 (33)	0.9	0.9

^aP < 0.05. PSC: Primary sclerosing cholangitis; UC: Ulcerative colitis; CD: Crohn disease; SD: Standard deviation; ASA: 5-aminosalicylic acid; TNF: Tumor necrosis factor; IBD: Inflammatory bowel disease; OLT: Orthotopic liver transplantation; IHC: Immunohistochemistry; NA: Not available.

Table 2 Histopathologic features of Primary sclerosing cholangitis-Ulcerative colitis and Primary sclerosing cholangitis-Crohn disease patients, n (%)

	PSC-UC¹	UC¹	P value	PSC-CD¹	CD¹	P value	P value (PSC-UC vs PSC-CD)
Distribution of disease
Right colon only	7 (29)	1 (4)	0.03^a	3 (50)	2 (33)	0.6	0.3
Left colon only	1 (4)	7 (26)	0.06	0	0	1	0.9
Left and right colon	16 (67)	17 (63)	0.8	3 (50)	4 (67)	0.6	0.4
Rectum	15 (68)	18 (90)	0.1	1 (20)	5 (83)	0.055	0.07
Ileitis	4 (31)	1 (7)	0.1	5 (100)	4 (67)	0.9	0.9
Severity of disease (active inflammation)
Right colon (average activity score)	1.25	0.96	0.2	1.5	1	0.3	0.5
Left colon (average activity score)	1.2	1.82	0.03^a	0.83	0.86	0.9	0.4
Rectum (average activity score)	0.96	2.05	0.0006^a	0.2	1.17	0.1	0.1
Ileum (average activity score)	0.31	0.07	0.2	1	1	1	0.01^a
Max average activity score at any site	1.46	2.29	0.0005^a	1.83	1.67	0.7	0.3
Architectural distortion
Right colon	24 (100)	21 (78)	0.9	6 (100)	6 (100)	1	1
Left colon	24 (96)	26 (93)	0.6	6 (100)	7 (100)	1	0.9
Rectum	18 (78)	19 (95)	0.1	5 (100)	5 (83)	0.9	0.9
Any site	26 (100)	28(100)	1	6 (100)	9 (100)	1	1
Paneth cell metaplasia
Left colon	11 (44)	14 (50)	0.7	1 (17)	4 (57)	0.1	0.2
Rectum	5 (22)	6 (30)	0.5	1 (20)	0	0.9	0.9
Basal plasmacytosis
Right colon	14 (58)	13 (48)	0.5	2 (33)	3 (50)	0.5	0.3
Left colon	11 (44)	22 (79)	0.01^a	0	4 (57)	0.9	0.9
Rectum	6 (26)	19 (95)	0.0004^a	1 (20)	3 (50)	0.3	0.7
Any site	16 (62)	27 (96)	0.01^a	3 (50)	7 (78)	0.2	0.6
Pyloric gland metaplasia
Right colon	2 (8)	1 (4)	0.7	1 (17)	1 (17)	1	0.6
Left colon	0	0	1	0	0	1	1
Any site	2 (8)	1 (4)	0.5	1 (17)	1 (11)	0.7	0.5
Granulomas
Any site	1 (4)	0	0.9	1 (17)	6 (67)	0.08	0.3

^aP < 0.05.

¹In all biopsied sites. PSC: Primary sclerosing cholangitis; UC: Ulcerative colitis; CD: Crohn diseas; NA: Not available.

Table 3 Select clinical and histologic features of patients with orthotopic liver transplantation

Diagnosis	Gender	Age (yr)	Age at Dx of IBD (yr)	Age at Dx of PSC (yr)	Colitis duration (yr)	Colectomy	Time of sample	Distribution of IBD	Rectal involvement	Follow-up (yr)	Medical treatment for IBD
PSC UC	F	45	19	40	26	No	Post-OLT^f	Right and left	Not involved	8	Ursodiol
PSC UC	F	18	3	27	15	Yes	Pre-OLT	Right and left	Involved	15	Azathioprine
PSC UC	M	61	57	57	4	No	Pre-OLT	Right and left	Involved	10	Mesalamine
PSC UC	M	52	18	42	34	Yes	Pre-OLT	Right and left	Involved	16	Ursodiol, azathioprine
PSC UC	M	24	19	20	5	Yes	Pre-OLT	Right and left	Involved	7	Steroids, mesalamine
PSC UC	M	19	18	15	1	No	Post-OLT	Right and left	Involved	13	Mesalamine
PSC UC	M	22	20	20	2	Yes	Post-OLT	Right and left	Involved	4	Mesalamine, azathioprine, infliximab, steroids
PSC UC	M	55	52	40	3	No	Post-OLT	Right only	Involved	17	Mesalamine
PSC UC	M	60	34	58	26	No	Pre-OLT	Left only	Involved	4	Mesalamine
PSC UC	M	49	29	35	20	Yes	Post-OLT	Right and left	Not involved	10	Steroids
PSC NIBD	M	57	NA	57	NA	NA	Pre-OLT	NA	NA	14	NA

NIBD: No inflammatory bowel disease; F: Female; M: Male; Dx: Diagnosis; NA: Not available; PSC: Primary sclerosing cholangitis; UC: Ulcerative colitis; CD: Crohn disease; IBD: Inflammatory bowel disease; OLT: Orthotopic liver transplantation; NA: Not available.

Table 4 Select histologic and clinical features of Primary sclerosing cholangitis-Inflammatory bowel disease patients with and without orthotopic liver transplantation, n (%)

	PSC-IBD with OLT¹	PSC-IBD no OLT¹	P value (PSC-IBD with OLT vs PSC-IBD no OLT)
Distribution
Right colon only	1 (10)	9 (30)	0.1
Left colon only	1 (19)	0 (0)	0.3
Right and left colon	8 (80)	11(37)	0.2
Rectum	8 (80)	8 (44)	0.04^a
Ileum	2 (33)	7 (58)	0.6
Dysplasia	3 (30)	1 (4.7)	0.08
Treatment
Ursodiol	2 (20)	9 (45)	0.2
Steroids	3 (30)	7 (33)	1
ASA	6 (60)	11 (52)	1
Anti-TNF	1 (10)	5 (24)	0.6
Immunomodulators	3 (30)	3 (14)	0.3
Colectomy	5 (50)	5 (22)	0.2
Severity of acute inflammation (average activity score)
Right colon	1.5	1.2	0.4
Left colon	1.7	0.86	0.04^a
Rectum	1.3	0.6	0.04^b
Ileum	0.3	0.6	0.3
IBD duration (yr) (mean ± SD)	13.6 ± 12	8.5 ± 8.8	0.3

¹In all available patients and biopsied sites.

^aUni and multivariate analysis (P < 0.05).

^bUni and multivariate analysis (P < 0.05). SD: Standard deviation; ASA: 5-aminosalicylic acid; Anti-TNF: Anti-tumor necrosis factor; IBD: Inflammatory bowel disease; PSC: Primary sclerosing cholangitis; OLT: Orthotopic liver transplantation.

Citation: Aranake-Chrisinger J, Dassopoulos T, Yan Y, Nalbantoglu I. Primary sclerosing cholangitis associated colitis: Characterization of clinical, histologic features, and their associations with liver transplantation. World J Gastroenterol 2020; 26(28): 4126-4139
URL: https://www.wjgnet.com/1007-9327/full/v26/i28/4126.htm
DOI: https://dx.doi.org/10.3748/wjg.v26.i28.4126