Table 1. Characteristics of included studies.
| Author (Year) | Country | Mean age (range) | Treatment/Control | Dose | Treatment duration | Study size | Mortality or liver transplantation (%) | Adverse events (%) |
|---|---|---|---|---|---|---|---|---|
| Treatment/Control | Treatment/Control | |||||||
| Itakura (2004) | Japan | 57 (47–67) | BEF plus UDCA/UDCA | BEF: 400 mg per day; UDCA: 600 mg per day | 6 months | 16 | 8/6 | 11/14 |
| Iwasaki (a) (2008) | Japan | 56 (45–67) | BEF plus UDCA/UDCA | BEF: 400 mg per day; UDCA: 600 mg per day | 52 weeks | 22 | 0/0 | 42/10 |
| Iwasaki (b) (2008) | Japan | 56(28–75) | BEF/UDCA | BEF :400 mg daily orallyUDCA;600 mg daily | 52 weeks | 45 | NR/NR | |
| Kanda (2003) | Japan | 57 (52–72) | BEF plus UDCA/UDCA | BEF: 400 mg per day; UDCA: 600 mg per day | 6 months | 22 | 11/11 | 18/9 |
| Leuschner (1999) | German | 50 (40–60) | COT plus UDCA/UDCA | COT: 3 mg three times daily; UDCA: 10–15 mg/kg/day in three divided doses | 2 years | 40 | 5/5 | 10/5 |
| Combes (2005) | United States | 53 (42–60) | MTX plus UDCA/UDCA | MTX: in four doses over 48 hours (total dose 10 mg/week); UDCA: 500 mg per day | 117 months | 265 | NR/NR | 10/9 |
| Gonzalez-Koch (1997) | Chile | 53 (45–75) | MTX plus UDCA/UDCA | UDCA: 13–15mg/kg/day MTX: 0.25mg/kg/week | 48 weeks | 25 | 12/10 | 25/8 |
| Lindor (1995) | United States | 59.5 (40–75) | MTX plus UDCA/UDCA/OBS | UDCA: 13–15mg/kg/day MTX: 0.25mg/kg/week | 2 years | 121 | NR/NR/NR | 22/NR/NR |
| Leung (2011) | United States | 54 (52–56) | MTX plus UDCA/COC plus UDCA | UDCA:13–15mg/kg/day; MTX:0.25mg/kg/week; COC: 1 mg/day | 10 years | 29 | NR/NR | NR/NR |
| Battezzati (1993) | Italy | 57 (42–60) | UDCA/OBS | 500 mg per day | 1 year | 88 | 43/43 | 9/2 |
| Combes (1995) | United States | 49 (52–56) | UDCA/OBS | 10 to 12 mg/kg/day | 2 years | 151 | 70/70 | 17/16 |
| Eriksson (1997) | Sweden | 57 (46–70) | UDCA/OBS | 500 mg per day | 2 years | 116 | 58/52 | 12/7 |
| Heathcote (1994) | Canada | 56 (50–62) | UDCA/OBS | 14mg/kg/day | 2 years | 222 | 91/94 | 16/24 |
| Hwang (1993) | China | 58 (50–66) | UDCA/OBS | 600 mg/day. | 3 months | 12 | 6/6 | 17/17 |
| Leuschner (1989) | German | 53 (53–73) | UDCA/OBS | 10 mg/kg/day | 9 months | 20 | 9/9 | 10/10 |
| Lindor (1994) | United States | 53 (51–55) | UDCA/OBS | 13 to 15mg/kg/day | 4 years | 180 | 75/68 | 8/13 |
| Oka (1990) | Japan | 59 (51–67) | UDCA/OBS | 600 mg/day | 24 weeks | 52 | 25/25 | 15/4 |
| Papatheodoridis (2002) | Greece | 54 (44–64) | UDCA/OBS | 12 to 15 mg/kg/day | 92 months | 86 | 26/29 | 53/40 |
| Pares (2000) | Spain | 54 (44–64) | UDCA/OBS | 14 to 16 mg/kg/day | 2 years | 192 | 89/89 | 26/18 |
| Poupon (1991) | France | 56 (47–65) | UDCA/OBS | 13 to 15 mg/kg/day | 2 years | 146 | 67/63 | 15/26 |
| Turner (1994) | England | 57 (54–60) | UDCA/OBS | 10mg/kg/day | 2 years | 46 | 21/21 | 27/29 |
| Vuoristo (1995) | Finland | 55 (50–60) | UDCA/OBS | 12 to 15 mg/kg/day | 2 years | 59 | 29/26 | 3/29 |
| Chazouilleres (1998) | France | 50 (47–53) | UDCA/COT plus UDCA | UDCA: 13 to 15 mg/kg/day; COT: 0.5 mg/kg/day | 23 months | 11 | 3/5 | 20/33 |
| Gunsar (2002) | Turkey | 44 (40–55) | UDCA/COT plus UDCA | UDCA: 13 mg/kg/day; COT: 0.5 mg/kg/day | 28 months | 20 | 12/5 | 15/14 |
| Chazouilleres (2006) | France | 41 (39–57) | UDCA/COT plus UDCA | UDCA: 13 to 15 mg/kg/day; COT: 0.5 mg/kg/day | 90 months | 17 | NR/NR | NR/NR |
| Heurgue (2007) | France | 44 (41–56) | UDCA/COT plus UDCA | UDCA: 13 to 15 mg/kg/day; COT: 0.5 mg/kg/day | 60 months | 13 | NR/NR | NR/NR |
| Ozaslan (2010) | Turkey | 44 (40–51) | UDCA/COT plus UDCA | UDCA: 13 to 15 mg/kg/day; COT: 0.5 mg/kg/day | 31 months | 12 | 2/6 | NR/NR |
| Tanaka (2011) | Japan | 54 (50–60) | UDCA/COT plus UDCA | UDCA: 10 mg/kg/day; COT: 0.5 mg/kg/day | 73 months | 25 | 14/8 | NR/NR |
| Ikeda (1996) | Japan | 57 (55–60) | COC plus UDCA/UDCA | COC: 1 mg/kg/day; UDCA: 600 mg/day | 30 months | 22 | NR/NR | 30/8 |
| Poupon (1996) | France | 53 (51–55) | COC plus UDCA/UDCA | COC: 1 mg/d, 5 days per week; UDCA: 13–15 mg/day | 2 years | 74 | NR/NR | 5/3 |
| Almasio (2000) | Italy | 55 (45–65) | COC plus UDCA/UDCA | COC: 1 mg/daily; UDCA: 500 mg/daily | 3 years | 90 | 35/41 | 4/2 |
| Battezzati (2001) | Italy | 58 (48–68) | COC plus UDCA/UDCA | COC: 1 mg/daily; UDCA: 500 mg/daily | 10 years | 44 | 14/8 | NR/NR |
| Christensen(1985) | Denmark | 55 (25–78) | AZP/OBS | 300 to 700 mg/week | 11 years | 248 | 58/72 | 6/7 |
| Heathcote(1976) | UK | 51 (24–79) | AZP/OBS | 2 mg/kg/day | 5 years | 45 | 36/52 | 16/0 |
| Lombard(1993) | UK | 54 (44–64) | CYP/OBS | 3 mg/kg/day | 928 days | 349 | 17/18 | 56/42 |
| Minuk(1988) | Canada | 51 (41–61) | CYP/OBS | 2.5 mg/kg/day | 1year | 12 | 0/17 | 100/17 |
| Wiesner(1990) | USA | 46 (37–57) | CYP/OBS | 4 mg/kg/day | 2.7 years | 29 | 5/30 | 79/50 |
| Dickson(1985) | USA | 46 (36–56) | DPM/OBS | 250 mg/day | 10 years | 227 | 40/40 | 53/22 |
| Epstein(1981) | UK | 53 (43–63) | DPM/OBS | over 8 to 10 weeks from 150 mg/day to 600 mg/day | 6 years | 98 | 30/43 | 31/3 |
| Matloff(1982) | USA | 52 (42–62) | DPM/OBS | 1g/day | 28 months | 52 | 62/12 | 35/4 |
| Neuberger(1985) | UK | 53 (41–62) | DPM/OBS | 1.2 g/day, increased from 300 mg by 300 mg each fortnight until 1.2 g | 4 years | 189 | 54/32 | 36/8 |
| Taal(1983) | Netherlands | 51 (43–62) | DPM/OBS | 1 g/day (increased from 250 mg every month until 1 g for the first 6 months. After that, decreased to 500 mg/day for the remaining 6 months | 1 years | 24 | 18/31 | 100/85 |
| Mitchison (1992) | England | 52 (44–60) | COT/OBS | initially 30 mg/day then reduced by 5 mg/day every two weeks until a maintenance dose of 10 mg/day was reached. | 3 years | 36 | 16/29 | 79/24 |
| Hendrickse(1999) | England | 57 (55–59) | MTX/OBS | 7.5 mg/week. | 6 years | 60 | 40/37 | 83/73 |
| Kaplan(2003) | USA | 51 (50–53) | MTX plus COC | MTX: 15 mg/week, 5 mg every 12 hours 3 times.COC: 0.6 mg twice daily | 2 years | 85 | 57/44 | 17/7 |
| Kaplan(1999) | USA | 51 (50–53) | COC plus MTX | COC: 0.6 mg twice dailyMTX: 15 mg/wk, taken as 5 mg every 12 hours 3 times | 24 months | 85 | 23/29 | NR/NR |
| Kaplan(1986) | USA | 48 (40–68) | COC/OBS | 0.6 mg twice daily. | 2 years | 60 | 20/47 | 13/3 |
| Warnes(1987) | UK | 53 (47–64) | COC/OBS | 1 mg/day | 18 months | 64 | 15/30 | 56/7 |
| Bodenheimer(1988) | USA | 52 (34–59) | COC/OBS | 0.6 mg twice daily. | 5 years | 57 | NR/NR | 14/3 |
| Zifroni (1991) | USA | 57 (50–65) | COC/OBS | 0.6 mg twice daily. | 4 years | 57 | 46/48 | 11/0 |
Notes: COC: colchicine; BEF: bezafibrate; COT: corticosteroids; MTX: methotrexate; UDCA: ursodeoxycholic acid; OBS: observation; CYP: cyclosporin A; DPM: D-penicillamine; AZP: azathioprine; NR: not reported.