TABLE 2.
Number of patients, deaths and mortality rate per 1000 person‐years and risk estimates for death
| Group | N (%) | N deaths (%) | Mortality rate (95% CI) per 1000 PY | Hazard ratio (95% CI) a | Hazard ratio (95% CI) b | Hazard ratio (95% CI) c | Hazard ratio (95% CI) d | |||
|---|---|---|---|---|---|---|---|---|---|---|
| Follow‐up with scheduled imaging | Clinical follow‐up | Follow‐up with scheduled imaging | Clinical follow‐up | Follow‐up with scheduled imaging | Clinical follow‐up | |||||
| Overall | 2625 (100%) | 350 (100%) | 218 (8.3%) | 82 (23.4%) | 11.0 (9.5–12.4) | 23.1 (18.1–28.1) | 0.53 (0.41–0.68) | 0.61 (0.47–0.80) | 0.64 (0.49–0.84) | 0.57 (0.44–0.75) |
| Sex | ||||||||||
| Male | 1716 (65.4%) | 237 (67.7%) | 157 (9.1%) | 56 (23.6%) | 12.1 (10.2–14.0) | 23.2 (17.1–29.3) | 0.58 (0.43–0.80) | 0.69 (0.50–0.95) | 0.71 (0.52–0.99) | 0.55 (0.40–0.77) |
| Female | 907 (34.6%) | 113 (32.3%) | 61 (6.7%) | 26 (23.0%) | 8.9 (6.7–11.2) | 23.0 (14.1–31.8) | 0.42 (0.26–0.67) | 0.43 (0.26–0.70) | 0.45 (0.27–0.74) | 0.50 (0.30–0.84) |
| Age (years) at start of follow‐up | ||||||||||
| 0–<18 y | 126 (4.8%) | 20 (5.7%) | 2 (1.6%) | 1 (5.0%) | 1.6 (0.0–3.9) | 3.9 (0.0–11.4) | 0.42 (0.04–4.59) | 0.43 (0.04–4.99) | 0.40 (0.04–4.48) | 0.44 (0.04–5.01) |
| 18–<30 y | 644 (24.5%) | 69 (19.7%) | 26 (4.0%) | 8 (11.6%) | 5.3 (3.3–7.4) | 11.5 (3.5–19.4) | 0.56 (0.25–1.28) | 0.49 (0.22–1.11) | 0.51 (0.22–1.14) | 0.34 (0.14–0.82) |
| 30–<40 y | 734 (28.0%) | 65 (18.6%) | 53 (7.2%) | 13 (20.0%) | 9.1 (6.7–11.6) | 19.5 (8.9–30.1) | 0.56 (0.30–1.03) | 0.56 (0.30–1.03) | 0.55 (0.30–1.03) | 0.42 (0.22–0.79) |
| 40–<50 y | 532 (20.3%) | 81 (23.1%) | 50 (9.4%) | 25 (30.9%) | 12.1 (8.8–15.5) | 27.4 (16.7–38.2) | 0.53 (0.32–0.87) | 0.51 (0.31–0.84) | 0.50 (0.30–0.84) | 0.50 (0.30–0.83) |
| 50–<60 y | 361 (13.8%) | 65 (18.6%) | 45 (12.5%) | 18 (27.7%) | 18.1 (12.8–23.4) | 26.2 (14.1–38.3) | 0.76 (0.43–1.33) | 0.77 (0.43–1.41) | 0.82 (0.45–1.50) | 0.93 (0.50–1.74) |
| 60–<70 y | 184 (7.0%) | 41 (11.7%) | 28 (15.2%) | 12 (29.3%) | 25.1 (15.8–34.4) | 41.2 (17.9–64.6) | 0.63 (0.32–1.25) | 0.50 (0.24–1.05) | 0.59 (0.28–1.24) | 0.43 (0.20–0.92) |
| ≥70 y | 40 (1.5%) | 9 (2.6%) | 13 (32.5%) | 5 (55.6%) | 71.6 (32.7–110.6) | 156.1 (19.3–292.9) | 0.47 (0.17–1.32) | 0.21 (0.06–0.80) | 0.19 (0.04–0.88) | 0.26 (0.07–1.02) |
| Year of start of follow‐up | ||||||||||
| 2000–2004 | 688 (26.2%) | 187 (53.4%) | 94 (13.7%) | 57 (30.5%) | 11.2 (8.9–13.5) | 23.7 (17.5–29.8) | 0.50 (0.36–0.69) | 0.60 (0.42–0.84) | 0.59 (0.42–0.84) | 0.61 (0.43–0.86) |
| 2005–2009 | 604 (23.0%) | 77 (22.0%) | 66 (10.9%) | 12 (15.6%) | 12.4 (9.4–15.4) | 16.4 (7.1–25.7) | 0.78 (0.42–1.45) | 0.97 (0.52–1.81) | 1.10 (0.58–2.11) | 0.72 (0.38–1.37) |
| 2010–2014 | 898 (34.2%) | 63 (18.0%) | 50 (5.6%) | 11 (17.5%) | 10.0 (7.2–12.7) | 31.7 (13.0–50.4) | 0.32 (0.16–0.61) | 0.33 (0.17–0.64) | 0.36 (0.19–0.70) | 0.34 (0.17–0.67) |
| 2015–2018 | 435 (16.6%) | 23 (6.6%) | 8 (1.8%) | 2 (8.7%) | 7.2 (2.2–12.3) | 36.3 (0.0–86.6) | 0.16 (0.03–0.78) | 0.10 (0.02–0.52) | 0.12 (0.02–0.64) | 0.19 (0.02–1.64) |
| Main diagnosis | ||||||||||
| PSC | 2262 (86.2%) | 310 (88.6%) | 197 (8.7%) | 79 (25.5%) | 11.4 (9.8–13.0) | 25.1 (19.5–30.6) | 0.50 (0.38–0.65) | 0.58 (0.44–0.77) | 0.61 (0.46–0.80) | 0.54 (0.41–0.72) |
| Small duct PSC/possible small duct PSC | 105 (4.0%) | 5 (1.4%) | 4 (3.8%) | 0 | 4.9 (0.1–9.6) | 0 | ‐ | ‐ | ‐ | |
| PSC with features of AIH | 258 (9.8%) | 35 (10.0%) | 17 (6.6%) | 3 (8.6%) | 9.7 (5.1–14.3) | 8.2 (0.0–17.6) | 1.69 (0.48–6.01) | 2.28 (0.60–8.73) | 2.33 (0.61–8.99) | 2.19 (0.55–8.77) |
| Type of follow‐up | ||||||||||
| Scheduled MRI and/or US | 1897 (72.3%) | 350 (100%) | 158 (8.3%) | 82 (23.4%) | 12.5 (10.5–14.4) | 23.1 (18.1–28.1) | 0.61 (0.46–0.80) | 0.64 (0.48–0.86) | 0.69 (0.51–0.93) | 0.59 (0.44–0.80) |
| Follow‐up including scheduled ERCP | 728 (27.7%) | 350 (100%) | 60 (8.2%) | 82 (23.4%) | 8.4 (6.3–10.5) | 23.1 (18.1–28.1) | 0.38 (0.27–0.54) | 0.53 (0.37–0.75) | 0.51 (0.36–0.73) | 0.51 (0.36–0.72) |
Abbreviations: AIH, autoimmune hepatitis; CI, confidence intervals; ERCP, endoscopic retrograde cholangiopancreatography; MRI, Magnetic resonance imaging; PSC‐primary sclerosing cholangitis; PY, person years; US, ultrasound.
a
Unadjusted.
b
Adjusted for sex, age and start year of follow‐up.
c
Adjusted for sex, age, start year of follow‐up and first dominant stricture as a time‐dependent covariate.
d
Adjusted for sex, age, start year of follow‐up and cholangiocarcinoma or high‐grade dysplasia as a time‐dependent covariate.