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©The Author(s) 2015.
World J Gastroenterol. Feb 14, 2015; 21(6): 1956-1971
Published online Feb 14, 2015. doi: 10.3748/wjg.v21.i6.1956
Published online Feb 14, 2015. doi: 10.3748/wjg.v21.i6.1956
Table 1 Epidemiology primary sclerosing cholangitis - inflammatory bowel disease n (%)
| Ref. | Country | Period | Incidence1 | PSC | Male Gender | PSC-IBD | PSC-UC | PSC-CD | PSC-IBD-U | Diagnosis IBD |
| Rabinovitz et al[100], 1990 | United States | NA | NA | 66 | NA | 47 (71.2) | 39 (83.0) | 8 (17.0) | 0 | IBD23 |
| Farrant et al[19], 1991 | United Kingdom | 1972-1989 | NA | 126 | 78 (61.9) | 85 (67.5) | 83 (97.6) | 2 (2.4) | NA | IBD2 |
| Schrumpf et al[18], 1994 | Norway | 1975-1989 | NA | 77 | 51 (66.2) | 76 (98.7) | 58 (76.3) | 11 (14.5) | 7 (9.2) | IBD23 |
| Escorsell et al[17], 1994 | Spain | 1984-1988 | 0.07 | 43 | 26 (60.5) | 20 (46.5) | 19 (95.0) | 1 (5.0) | NA | IBD4 |
| Broomé et al[2], 1996 | Sweden | ? - 1992 | NA | 305 | 195 (63.9) | 249 (81.6) | 220 (88.4) | 20 (8.0) | 9 (3.6) | IBD2 |
| Boberg et al[4], 1998 | Norway | 1986-1995 | 1.31 | 17 | 12 (70.6) | 12 (70.6) | 9 (75.0) | 2 (16.7) | 1 (8.3) | IBD4 |
| Ponsioen et al[20], 2002 | The Netherlands | 1979-1999 | NA | 174 | 105 (60.3) | 114 (65.5) | 83 (72.8) | 28 (24.6) | 3 (2.6) | IBD4 |
| Bambha et al[5], 2003 | United States | 1976-2000 | 0.90 | 22 | 15 (68.3) | 16 (72.7) | 12 (75.0) | 3 (18.8) | 1 (5.0) | IBD2 |
| Kingham et al[101], 2004 | United Kingdom | 1984-2003 | 0.91 | 53 | 33 (62.3) | 33 (62.3) | 30 (90.9) | 3 (19.1) | 0 | IBD4 |
| Bergquist et al[102], 2005 | Sweden | 1984-1999 | NA | 145 | 100 (69.0) | 126 (86.9) | 107 (84.9) | 19 (15.1) | 0 | IBD23 |
| Tischendorf et al[103], 2007 | Germany | 1978-2004 | NA | 273 | 195 (71.4) | 172 (63.0) | 141 (82.0) | 29 (16.9) | 2 (1.2) | IBD23 |
| Bergquist et al[104], 2007 | Sweden | 1984-2004 | NA | 246 | 165 (67.1) | 195 (79.3) | 166 (85.1) | 21 (10.8) | 8 (4.1) | IBD23 |
| Kaplan et al[14], 2007 | Canada | 2000-2005 | 0.92 | 49 | 27 (55.1) | 36 (73.5) | 17 (47.2) | 19 (52.8) | 0 | IBD23 |
| Card et al[3], 2008 | United Kingdom | 1987-2002 | 0.41 | 223 | 141 (63.2) | 108 (48.4) | 67 (62.0) | 13 (12.0) | 28 (25.9) | IBD4 |
| Lindkvist et al[105], 2010 | Sweden | 1992-2005 | 1.22 | 199 | 142 (71.4) | 152 (76.4) | 129 (84.9) | 17 (11.2) | 5 (3.3) | IBD4 |
| Bowlus et al[106], 2010 | United States | 1995-2009 | NA | 6767 | 4475 (66.1) | 4637 (68.5) | 3067 (66.1) | 1090 (23.5) | 480 (10.4) | IBD4 |
| Toy et al[107], 2011 | United States | 2000-2006 | 0.41 | 169 | 101 (59.8) | 109 (64.5) | 95 (87.2) | 13 (11.9) | 1 (0.9) | IBD4 |
| Boonstra et al[22], 2013 | The Netherlands | 2000-2007 | 0.50 | 590 | 375 (63.6) | 402 (68.1) | 308 (76.6) | 78 (19.4) | 16 (4.0) | IBD23 |
Table 2 Phenotypic features primary sclerosing cholangitis - inflammatory bowel disease n (%)
| Ref. | IBD (n) | PSC-IBD (n) | Proctitis | Leftsided | Pancolitis | Backwash | Rectal Sparing | Diagnosis IBD | |||||
| IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | ||||
| Olsson et al[9], 1991 | 1445 | 55 | 552 (38.2) | 3 (5.5) | NA | NA | 893 (61.8) | 52 (94.5) | NA | NA | NA | NA | IBD12 |
| Loftus et al[11], 2005 | 142 | 71 | NA | NA | NA | NA | 76 (53.5) | 60 (84.5) | 10 (7.0) | 20 (28.2) | 8 (5.6) | 34 (47.9) | IBD12 |
| Kaplan et al[14], 2007 | 0 | 36 | NA | NA | NA | NA | NA | 17 (47.2) | NA | 4 (11.1) | NA | 2 (5.6) | IBD12 |
| Sokol et al[15], 2008 | 150 | 75 | 138 (92.0) | 68 (90.7) | 130 (86.7) | 68 (90.7) | 91 (60.7) | 49 (65.3) | 36 (24.0) | 14 (18.7) | 20 (13.3) | 15 (20.0) | IBD12 |
| Joo et al[31], 2009 | 40 | 40 | 0 | 0 | 14 (35.0) | 3 (7.5) | 18 (45.0) | 34 (85.0) | 3 (7.5) | 4 (10.0) | 10 (25.0) | 11 (27.5) | IBD12 |
| Sano et al[32], 2010 | 60 | 20 | 18 (30.0) | 1 (5.0) | 19 (31.7) | 1 (5.0) | 21 (35.0) | 7 (35.0) | NA | NA | NA | NA | IBD12 |
| Ye et al[25], 2011 | 63 | 21 | NA | NA | NA | NA | 35 (55.6) | 20 (95.2) | 2 (3.2) | 9 (42.9) | 1 (1.6) | 8 (38.1) | IBD12 |
| Jørgensen et al[33], 2012 | 0 | 110 | NA | NA | NA | 3 (2.7) | NA | 60 (54.5) | NA | 17 (15.5) | NA | 73 (66.4) | IBD12 |
| O'toole et al[35], 2012 | 2649 | 103 | 209 (7.9) | 1 (1.0) | 649 (24.5) | 23 (22.3) | 663 (25.0) | 56 (54.4) | NA | NA | NA | NA | IBD12 |
| Boonstra et al[12], 2012 | 0 | 380 | NA | 9 (2.4) | NA | 34 (8.9) | NA | 219 (57.6) | NA | NA | NA | NA | IBD12 |
| Boonstra et al[12], 20123 | 80 | 80 | 4 (5.0) | 2 (2.5) | 16 (20.0) | 2 (2.5) | 35 (43.8) | 52 (65.0) | 2 (2.5) | 4 (5.0) | 1 (1.3) | 8 (10.0) | IBD12 |
| Schaeffer et al[34], 2013 | 0 | 97 | NA | 0 | NA | 17 (17.5) | NA | 42 (43.3) | NA | NA | NA | NA | IBD12 |
| Sinakos et al[30], 2013 | 0 | 129 | NA | NA | NA | 16 (12.4) | NA | 76 (58.9) | NA | 15 (11.6) | NA | 31 (24.0) | IBD12 |
| Mean | 28.8% | 13.4% | 39.6% | 18.8% | 47.5% | 64.7% | 12.3% | 16.7% | 9.9% | 30.9% | |||
Table 3 Dysplasia or colorectal cancer in primary sclerosing cholangitis - inflammatory bowel disease n (%)
| Ref. | IBD (n) | PSC-IBD (n) | Country | Dysplasia | Risk/incidence | Proximal colon1 | Age dysplasia diagnosis (yr) | IBD duration (yr) | Diagnosis IBD | |||||
| IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | |||||
| Broomé et al[45], 1995 | 80 | 40 | Sweden | 7 (8.8) | 11 (27.5) | CR = 10 yr: 2% | CR = 10 yr: 9% | NA | NA | NA | NA | 26.02 | 18.02 | IBD34 |
| Gurbuz et al[49], 1995 | 0 | 35 | United States | NA | 13 (37.1) | NA | CR = 10 yr: 0% | NA | NA | NA | 51.42 | NA | 12.22 | IBD34 |
| Brentnall et al[56], 1996 | 25 | 20 | United States | 4 (16) | 9 (45) | NA | OR = 4.9 | NA | NA | NA | NA | NA | 20.02 | IBD34 |
| Loftus et al[29], 1996 | 0 | 143 | United States | NA | 27 (18.9) | NA | CR = 20 yr: 17% | NA | 42.9%5 | NA | 41.06 | NA | NA | IBD34 |
| Kornfeld et al[48], 1997 | 0 | 58 | Sweden | NA | 5 (8.6) | NA | NA | NA | NA | NA | NA | NA | NA | IBD7 |
| Leidenius et al[50], 1997 | 45 | 45 | Finland | 4 (8.9) | 13 (28.9) | CR = 10 yr: 3% | CR = 10 yr: 11% | NA | NA | NA | NA | 17.52 | 11.06 | IBD34 |
| Marchesa et al[54], 19978 | 1185 | 27 | United States | 145 (12.2) | 18 (66.7) | NA | RR = 10.4 | 40.8%5 | 100.0%5 | NA | NA | NA | NA | IBD4 |
| Shetty et al[84], 1999 | 196 | 132 | United States | 11 (5.6) | 33 (25) | RR = 1 | RR = 3.15 | 20.0%5 | 76.5%5 | NA | NA | NA | NA | IBD34 |
| Lindberg et al[46], 2001 | 103 | 19 | Sweden | 31 (30.1) | 12 (63.2) | CR = 20 yr: 16% | CR = 20 yr: 32% | 32.3% | 69.2% | NA | NA | NA | NA | IBD34 |
| Bergquist et al[108], 2002 | 0 | 477 | Sweden | NA | 35 (7.3) | NA | SIR = 10.3 | NA | NA | NA | NA | NA | NA | IBD34 |
| Loftus et al[11], 2005 | 142 | 71 | United States | 15 (10.6) | 18 (25.4) | CP = 10 yr: 20% | CP = 10 yr: 53% | 50%5 | 28.6%5 | NA | NA | 12.16 | 12.76 | IBD34 |
| Kaplan et al[109], 2007 | 0 | 45 | Canada | NA | 5 (11.1) | NA | IR = 3.1 | NA | NA | NA | NA | NA | NA | IBD34 |
| Lepistö et al[16], 2008 | 389 | 52 | Finland | 63 (16.2) | 17 (32.7) | CR = 20 yr: 0.39 | CR = 20 yr: 0.43 | NA | NA | NA | NA | NA | NA | IBD4 |
| Sokol et al[15], 2008 | 150 | 75 | France | 2 (1.3) | 10 (13.3) | CR = 25 yr: 1.5% | CR = 25 yr: 25.6% | 0.0%5 | 100.0%5 | NA | NA | 44.42 | 17.42 | IBD34 |
| Terg et al[51], 2008 | 64 | 39 | Argentina | 2 (3.1) | 7 (17.9) | CR = 10 yr: 2.0% | CR = 10 yr: 11% | NA | NA | NA | NA | NA | NA | IBD34 |
| Claessen et al[52], 2009 | 0 | 126 | The Netherlands | NA | 35 (27.8) | NA | CR = 10 yr: 9% | NA | 63.0%5 | NA | NA | NA | 12.66 | IBD34 |
| Lindström et al[38], 2011 | 46 | 28 | Sweden | 3 (6.5) | 9 (32.1) | NA | OR = 6.78 | 0.0% | 66.0% | NA | NA | 10.06 | 12.06 | IBD34 |
| Braden et al[58], 2012 | 216 | 166 | United Kingdom | 14 (6.5) | 14 (8.4) | CIR = 2.9% | CIR = 7.5% | 30.0% | 35.7% | NA | NA | NA | NA | IBD34 |
| Imam et al[53], 2012 | 0 | 784 | United States | NA | 10 (1.3) | NA | I = 0.4/yr | NA | 60.0% | NA | 37.42 | NA | NA | IBD34 |
| Jess et al[55], 2012 | 47374 | NA | Denmark | 329 (0.7) | 9 (?)9 | RR = 1.07 (UC) | RR = 9.13 | 38.0% | 100.0% | 71.66 | 64.06 | NA | 13.76 | IBD7 |
| de Valle et al[79], 2012 | 0 | 152 | Sweden | NA | 3 (2.0) | NA | SIR = 4.31 | NA | NA | NA | NA | NA | NA | IBD34 |
| Boonstra et al[22], 2013 | 722 | 402 | The Netherlands | 7 (1) | 19 (4.7) | SIR = 1.2 | SIR = 8.6 | NA | NA | 596 | 39.06 | 4.06 | 15.06 | IBD34 |
Table 4 Primary sclerosing cholangitis - inflammatory bowel disease and liver transplantation n (%)
| Ref. | n | Pre-OLT | Post-OLT | ||||||
| PSC only | PSC-IBD | Intact colon1 | De novo IBD | Exacerbation | Colectomy | Refractory IBD | Median follow-up (yr) | ||
| Dvorchik et al[65], 2002 | 192 | 0 | 192 | 169 (88.0) | NA | 22 (13.0) | 33 (19.5) | 22 (66.7) | 5.93 |
| Haagsma et al[59], 2003 | 48 | 25 (52.1) | 23 (47.9) | 23 (100.0) | 6 (24.0) | 9 (39.1) | 1 (4.3) | 0 | 7.2 |
| Verdonk et al[88], 2006 | 60 | 15 (25.0) | 45 (75.0) | 45 (100.0) | 3 (20.0) | NA | NA | NA | 6.1 |
| Cholongitas et al[62], 20072 | 56 | 18 (32.1) | 38 (67.9) | 33 (68.8) | 3 (16.6) | 17 (51.5) | 7 (16.7) | 3 (42.9) | 2.8 |
| Moncrief et al[61], 2010 | 59 | 16 (27.1) | 42 (71.2) | 32 (76.2) | 5 (31.3) | 13 (40.6) | 6 (18.8) | 4 (66.7) | 5.6 |
| Joshi et al[60], 2011 | 110 | 36 (32.7) | 74 (67.3) | 65 (87.8) | 6 (16.7) | 33 (50.8) | 7 (10.8) | 6 (85.7) | 6.53 |
| Navaneethan et al[63], 2012 | 77 | 0 | 77 | 58 (75.3) | NA | 5 (8.6) | 9 (15.5) | 3 (33.3) | 5.0 |
| Mosli et al[64], 2013 | 105 | 77 (73.3) | 28 (26.7) | 24 (85.7) | 1 (1.3) | 6 (25.0) | 2 (8.3) | 0 | 7.33 |
Table 5 Pouchitis in primary sclerosing cholangitis - inflammatory bowel disease n (%)
| Ref. | Country | IBD (n) | PSC-IBD (n) | IPAA | Pouchitis | Chronic pouchitis | Pouch failure | Diagnosis | ||||
| IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | IBD | PSC-IBD | |||||
| Kartheuser et al[74], 1993 | United States | NA | 40 | NA | 40 | NA | 19 (47.5) | NA | NA | NA | NA | D1 |
| Penna et al[69], 1996 | United States | 1043 | 54 | 1043 | 54 | 336 (32.2) | 34 (63.0) | ? (15.0)2 | ? (60.0)2 | NA | NA | D3 |
| Aitola et al[68], 1998 | Finland | 63 | 10 | 63 | 10 | 19 (30.2) | 9 (90.0) | 7 (11.1) | 7 (70.0) | NA | NA | D3 |
| Gorgun et al[71], 2005 | United States | 260 | 65 | 260 | 65 | 31 (11.9) | 9 (13.8) | 31 (11.9) | 9 (13.8) | 19 (7.3) | 1 (1.5) | D345 |
| Abdelrazeq et al[67], 2007 | United Kingdom | 182 | 16 | 182 | 16 | 53 (29.1) | 11 (68.8) | 18 (9.9) | 9 (56.3) | NA | NA | D345 |
| Lepistö et al[16], 2008 | Finland | 389 | 52 | 389 | 52 | 101 (26.0) | 25 (48.1) | NA | NA | 13 (3.3) | 2 (3.8) | D1 |
| Wasmuth et al[72], 2010 | Norway | 178 | 11 | 178 | 11 | 94 (52.8) | 8 (72.0) | 17 (9.6) | 4 (36.4) | 20 (11.2) | 1 (9.1) | D34 |
| Mathis et al[73], 2011 | United States | NA | 100 | NA | 100 | NA | 64 (64.0) | NA | 16 (16.0) | NA | 3 (3.0) | D345 |
| Block et al[70], 2013 | Norway | 113 | 48 | 62 | 31 | 20 (32.3) | 27 (87.1) | 8 (12.9) | 20 (64.5) | 4 (6.5) | 5 (16.1) | D345 |
- Citation: de Vries AB, Janse M, Blokzijl H, Weersma RK. Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis. World J Gastroenterol 2015; 21(6): 1956-1971
- URL: https://www.wjgnet.com/1007-9327/full/v21/i6/1956.htm
- DOI: https://dx.doi.org/10.3748/wjg.v21.i6.1956