Systematic Reviews
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©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 14, 2015; 21(6): 1956-1971
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 IBD2 3 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) IBD2 3 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 IBD2 3 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) IBD2 3 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) IBD2 3 Kaplan et al [14 ], 2007 Canada 2000-2005 0.92 49 27 (55.1) 36 (73.5) 17 (47.2) 19 (52.8) 0 IBD2 3 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) IBD2 3
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 IBD1 2 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) IBD1 2 Kaplan et al [14 ], 2007 0 36 NA NA NA NA NA 17 (47.2) NA 4 (11.1) NA 2 (5.6) IBD1 2 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) IBD1 2 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) IBD1 2 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 IBD1 2 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) IBD1 2 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) IBD1 2 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 IBD1 2 Boonstra et al [12 ], 2012 0 380 NA 9 (2.4) NA 34 (8.9) NA 219 (57.6) NA NA NA NA IBD1 2 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) IBD1 2 Schaeffer et al [34 ], 2013 0 97 NA 0 NA 17 (17.5) NA 42 (43.3) NA NA NA NA IBD1 2 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) IBD1 2 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 IBD3 4 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 IBD3 4 Brentnall et al [56 ], 1996 25 20 United States 4 (16) 9 (45) NA OR = 4.9 NA NA NA NA NA 20.02 IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4 Bergquist et al [108 ], 2002 0 477 Sweden NA 35 (7.3) NA SIR = 10.3 NA NA NA NA NA NA IBD3 4 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 IBD3 4 Kaplan et al [109 ], 2007 0 45 Canada NA 5 (11.1) NA IR = 3.1 NA NA NA NA NA NA IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4 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 IBD3 4
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 IBDExacerbation 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) D3 4 5 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 D3 4 5 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) D3 4 Mathis et al [73 ], 2011 United States NA 100 NA 100 NA 64 (64.0) NA 16 (16.0) NA 3 (3.0) D3 4 5 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) D3 4 5