Table 1 Characteristics of included studies
Author (Year) (Reference) | Country | Study design | Sample size (PSC cases) | Mean age (SD) year | Gender (Male n %) | Disease stage | Mayo risk score / MELD Score | IBD (Yes/No (n (%)) | LT (Yes/No (n (%)) | PROM | Rationale for Assessment | PROM administration |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
Gavaler (1991) [66] | USA | Cross- sectional study | 23 (23) | Quiescent group: 34.7 (6.2) Symptomatic group: 39.8 (1.6) | 15 (65%) | Symptomatic UC: Mild: 7 (40%) Moderate: 8 (47%) Severe: 2 (13%) | NR | Yes (23 (100%)) | Yes (23 (100%)) | Study questionnaire: symptoms of UC | A | Postal & telephone |
Gross (1999) [26] | USA | Before & after study | 157 (92) | Total sample: 50 (10) | 31 (34%) | NR | MRS: Mean 5.3 | NR | Yes (157 (100%)) | NIDDK-QA, pilot version NIDDKQA | A | Clinic |
Kim (2000) [28] | USA | Validation study | 96 (17) | 45 (9.3) | 7 (41%) | PSC undergoing LT: 17 (100%) | MRS: mean (SD) = −0.1(1.0) | NR | PSC patients undergoing LT: 17 (100%) | NIDDK-QA, SF- 36 | D | Clinic |
Bharucha (2000) [67] | USA | Pilot study | 20 (20) | 44 (11) | 12 (60%) | Early stage (1–2): 10 (50%), Late stage (3–4): 10 (50%) | MRS: mean (SD) = 2.87 (0.95) | Yes (14 (70%)) | No | Grading system fatigue & pruritus | B | Unclear |
Younossi (2000) [38] | USA | Cross-sectional study | 104 (29) | Total sample:: 55 (12) | Total sample 28 (97%) | NR | NR | NR | No | SF- 36, CLDQ | A | Unclear |
Younossi (2001) [39] | USA | Cross-sectional study | 353 (45) | Total sample: 54 (11) | Total sample 38 (30%) | Total sample: Child-pugh class: no cirrhosis: 47 (13%) class A: 43 (12%) class B-27 (8%) class C-4 (1%) | NR | NR | NR | SF-36, CLDQ | A | Clinic |
Longworth (2003) [45] | England and Wales | Cost effectiveness study | 347 (70) | NR | 48 (69%) | NR | Of 41 patients MELD score median/IQR = 10/6–16 | NR | Yes (45) 64%)) | EuroQol EQ. 5D | C | Postal |
Bjornsson (2004) [44] | England & Sweden | RCT | 93 (20) | NR | 13 (65%) | Cirrhosis: 5 (1%),Ludwig’s fibrosis score stage 1: 9 (44%), stage 2: 4(21%), stage 3:6(30%) | NR | Yes (16 (80%)) | No | PGWB, FIS, BDI, GSRS, Rome ll modular QA | A | Postal |
Ter Borg (2004) [36] | Netherlands | RCT | 33 (11) | NR | 10 (91%) | NR | NR | NR | No | VAS, FFSS, MFI | B | NR |
Ter Borg (2005) [48] | Netherlands | Cross-sectional study | 72 (27) | 45 (NR) | 19 (70%) | Cirrhosis: 15 (56%) | NR | Yes (2 (7%) | NR | VAS, FFSS, SF-36 | A | NR |
Olsson (2005) [33] | Sweden, Norway, Denmark | RCT | 198 (198) | UDCA: 43.6(12.7) Placebo: 43.1 (11.2) | 139 (70%) | NR | NR | Yes (168 (85%)) | NR | SF- 36 | B | Unclear |
Gorgun (2005) [21] | USA | Case matched study | 65 (65) | 43.37 (11.2) | 45 (69%) | NR | NR | Yes (65 (100%)) | No | FPQ, CGQOL | A | Â |
Mansour-Ghanaei (2006) [49] | Iran | RCT | 34 (6) | Total sample: 53.97 (11.93) | NR | NR | NR | NR | NR | VAS | B | Unclear |
Mayo (2007) [50] | USA | RCT | 21 (4) | Total sample: 53.97 (11.93) | Total sample 5 (15%) | NR | aTotal sample MELD mean (range): 11(6–24) | NR | NR | VAS, IDS-SR30 | B | Unclear |
Van os (2007) [52] | Netherlands | Cross-sectional study | 92(37) | 43.8(12.3) | 24 (65%) | Cirrhosis: 5 (13.5%) | NR) | NR | NR | BDI, SADS | A | Postal |
Tillman (2009) [37] | Germany | Cross-sectional study | 511(13) | 42 (NR) | NR | NR | NR | NR | NR | SF- 36, FIS, WHOQOL-BREF, HADS | A | In clinic |
Ananthakrishnan (2010) [47] | USA | Case-control study | 26 (26) | 40.7 (14.8) | 21 (80.8%) | NR | MELD score mean (range) 8 (6–20) | Yes (26(100%)) | No | SIBDQ, HBI, UCAI | A | Outpatient clinic |
Aberg (2012) [30] | Finland | Cross-sectional study | 401 (56) | 53 (9) | 36 (64%) | NR | NR | NR | Yes (56 (100%)) | 15D, ad hoc questionnaire | A | Postal |
Benito De Valle (2012) [29] | England & Sweden | Cross-sectional study | 182 (182) | 160 patients no LT: 50 (16) | 112 (70%) | Small duct disease: 17 (11%), Liver cirrhosis: 12 (8%), Decompensated liver disease: 9 (6%) | MRS mean (SD): 0.34 (1.10) | Yes (126 (79%)) | Yes (22 (12%)) | SF-36, CLDQ, FIS, HADS | A | Postal |
Hagstrom (2012) [68] | Sweden | Cross-sectional study | 96 (96) | 47 (13) | 63 (66%) | Cases child pugh score of 10, significant fibrosis: 26 (27%), non-significant fibrosis: 70 (73%) | NR | Yes (73 (76%)) | Yes (12 (12.5%)) | LDH | A | Interview |
Gulati (2013) [25] | USA | Cross-sectional study | 40 (24) | Total sample: 11.6 (4.5) | 17 (43%) | Total sample: Cirrhosis 22 (55%) | NR | Total sample: Yes (16 (65%)) | No | Â | A | Unclear |
Block (2014) [69] | Norway & Sweden | Case-control study | 48 (48) | NR | 40 (83%) | NR | NR | 48 | Yes (IPAA: 11, IRA: 7) | OS | A | Scheduled follow up visit |
Gotthardt (2014) [6] | Germany | Cross-sectional study | 113 (113) | 43.6 (14.2) | 81 (71.7%) | NR | MRS n: low/intermediate/ high =48 (42%) / 25 (22%) / 5 (4%) | Yes (71 (63%)) | NR | SF 36, PHQ-9 | A | Postal |
Hov (2014) [70] | Norway | Case-control study | 240 (240) | NR | 171 (71%) | NR | NR | Yes (183 (77%)) | Yes (94 (39%)) | Study questionnaire | A | Postal |
Pavlides (2014) [34] | England | Retrospective case note review | 40 (PSC-IPAA = 21 & PSC-UC = 19) | NR | 31 (78%) | PSC-IPAA had dysplasia: 2 (5%) | NR | Yes (19 (47.5%)) | No | OS, CGQOL, FSFI, IIEF | A | Postal |
Raszeja-Wyszomirska (2014) [35] | Poland | Cross-sectional study | 102 (102) | 36 (12) | 73 (72%) | Cirrhosis: 30 (29%) | NR | Yes (65 (64%)) | NR | SF 36, PBC-40, PBC-27 | A | Unclear |
Cheung (2015) [32] | Canada | Cross-sectional study | 162 (99) | 46.1 (15.1) | 50 (51%) | Cirrhosis: 47 (48%), Decompensated liver disease: 16 (16%) | NR | Yes (74) | No | SF-36, PBC-40, PHQ-9, LDQOL, SIBDQ, 10 peered-reviewed QA on emotional and psychosocial | A | Postal or clinic |
Dyson (2015) [20] | USA | Cross-sectional study | 40 (40) | 51 (13) | 31 (78%) | NR | NR | Yes (24 (60%)) | NR | FIS, ESS, HADs, COMPASS | A | Postal |
Eaton (2015) [71] | Canada & USA | Case-control study | 1000 (1000) | NR | 619 (72%) | NR | NR | Yes (741 ((74%)) | Yes (450 ((45%)) | HHQ | A | Postal or clinic |
Haapamaki (2015) [31] | Finland | Cross-sectional study | 341 (341) | 43.3 (13.7) | 183 (54%) | ERC-score mean (SD): 5.9 (3.4) | NR | Yes (237 (69.5%)) | Yes (9 (2.6%)) | 15D, study questionnaire | A | ERC examination at the HUGH endoscopy unit |
Kalaitzakis (2015) [27] | England and Sweden | Cross-sectional study | 163 (163) | No LT: 50 (16) | No LT 122 (75%) | No LT Small-duct disease: 15 (10%), Diver cirrhosis: 11 (8%), Decompensated liver disease: 8 (6%) | No LT MRS: mean (SD) = 0.11(1.42) | No LT Yes (116 (71%)) | Yes (19 (12%)) | SF 36, SF-6D, CLDQ, study questionnaire | A, C | Unclear |
Raszeja-Wyszomirska (2015) [41] | Poland | Cross-sectional study | 33 (33) | 35.3 (13.38) | 11 (33%) | Cirrhosis: 6 (18%) | NR | Yes (22 (67%) | NR | SF 36, PBC-40, PBC-27 | A | NR |
Carbone (2017) [46] | Italy | Longitudinal study | 227 (64) | 50(11) | 39 (66%) | NR | NR | NR | NR | EQ-5D | A | Clinic |
Kempinska (2017) [40] | Poland | Cohort study | 275 (275) | Median 55, range 28–90 | 182 (66%) | NR | NR | NR | NR | SF 36, PBC-40, PBC-27 | A | NR |
Kittanamongkolchai (2017) [51] | USA | Before and after study | 13 (5) | 46.4 (13.2) | 1 (20%) | NR | NR | NR | NR | Pruritus numerical rating scale | B | Physician administered |
Tabibian (2017) [42] | USA | Pilot study | 16 (16) | 40 (NR) | 13 (81%) | All patients had stage 1–3 PSC | NR | 13 (81%) | NR | FFSS, 5-D itch scale, CLDQ, SF-36 | B | NR |
Younossi (2017) [43] | USA | Validation study | 102 (102) | 44 (13) | 33 (32%) | Cirrhosis: 37 (39%) | NR | 67 (68%) | NR | PSC PRO, SF-36, CLDQ, PBC-40, 5-D Itch | D | ePRO website |