Patient characteristics at diagnosis
| Derivation cohort (n=692) | Missing values (n (%)) | Validation cohort (n=264) | Missing values (n (%)) | |
| Male (n (%)) | 447 (65) | 165 (63) | ||
| Large duct PSC (n (%)) | 630 (91) | 231 (88) | ||
| Age at diagnosis PSC (years) (median (IQR)) | 37 (27–49) | 45 (33–57) | ||
| AIH overlap (n (%)) | 37 (5) | 5 (2) | ||
| IBD (n (%)) | 480 (70) | 195 (74) | ||
| UC (n (%)) | 373 (54) | 143 (54) | ||
| Crohn’s disease (n (%)) | 89 (13) | 39 (15) | ||
| Unspecified (n (%)) | 18 (3) | 13 (5) | ||
| Ursodeoxycholic acid (n (%))* | 326 (80) | 281 (41) | ||
| Follow-up time (months) (median (IQR)) | 110 (69–184) | 103 (53–153) | ||
| PSC-related death (n (%)) | 71 (10) | 37 (14) | ||
| Liver transplantation (n (%)) | 121 (18) | 18 (7) | ||
| Alkaline phosphatase (xULN) (median (IQR)) | 1.97 (1.43–3.28) | 255 (37) | 2.27 (1.27–3.83) | 177 (67) |
| Aspartate aminotransferase (xULN) (median (IQR)) | 1.55 (1.0–3.03) | 169 (24) | 1.21 (0.79–2.05) | 198 (75) |
| Alanine aminotransferase (xULN) (median (IQR)) | 2.29 (1.34–4.66) | 268 (39) | 1.40 (0.88–2.58) | 238 (90) |
| Bilirubin (xULN) (median (IQR)) | 0.82 (0.53–1.71) | 208 (30) | 0.71 (0.47–1.29) | 178 (67) |
| Gamma-glutamyl transpeptidase (xULN) (median (IQR)) | 6.11 (3.36–11.88) | 282 (41) | 7.15 (1.98–14.30) | 210 (80) |
| Albumin (xLLN) (median (IQR)) | 1.14 (1.02–1.23) | 312 (45) | 1.23 (1.11–1.31) | 182 (69) |
| Platelets (xLLN) (median (IQR)) | 1.79 (1.36–2.29) | 375 (54) | 1.89 (1.53–2.75) | 189 (71) |
| AIH, autoimmune hepatitis; PSC, primary sclerosing cholangitis; xLLN, lower limit of normal; xULN, upper limit of normal. | ||||