TABLE 1.
Patient demographics, liver biochemistries, and histopathologic scores
| AIH (n = 32) | ASC (n = 12) | PSC (n = 19) | |
|---|---|---|---|
| Age, median (range) | 13 (3–24) | 13 (3–17) | 15 (3–19) |
| Sex: female, n (%) | 15 (47) | 6 (50) | 11 (58) |
| Race: White, n (%) | 29 (91) | 10 (83) | 18 (95) |
| Ethnicity: non-Hispanic, n (%) | 31 (97) | 11 (92) | 19 (100) |
| IBD, n (%) | 3 (9) | 9 (75) | 16 (84) |
| Simplified IAIHG score: | |||
| Score, median (range) | 6 (3–8) | 6.5 (3–8) | 3 (2–7) |
| % with ≥6 | 78 | 75 | 16 |
| IgG, median (range) | 1680 (356–5170) | 2270 (795–3420) | 1650 (1146–3680) |
| Cholangiopathy on MRCP/ERCP, % (n, patient with imaging) | 0 (n = 14) | 100% (n = 12) | 100% (n = 15) |
| Liver function at BL, median (range) | |||
| INR | 1.27 (1.03–3.69) | 1.08 (0.9–1.76) | 1.03 (0.84–1.92) |
| Total bilirubin (mg/dL) | 1.65 (0.1–26.5) | 0.6 (0.3–1) | 0.35 (0.2–1.8) |
| MELD | 12 (7–27) | 7 (6–13) | 7 (6–16) |
| Serum liver biochemistries at BL, median (range; U/L) | |||
| ALT | 268 (48–2814) | 119 (29–501) | 128 (17–490) |
| AST | 280 (33–2985) | 102 (29–528) | 89 (9–472) |
| ALP | 263 (56–886) | 615 (75–1402) | 332 (46–1163) |
| GGT | 101 (10–338) | 368 (20–796) | 355 (29–890) |
| Histopathology, median (range) | |||
| Number of biopsies scored | AIH (n = 31) | ASC (n = 11) | PSC (n = 18) |
| METAVIR classification | 2 (0–4) | 2 (1–3) | 1.5 (0–4) |
| Ishak stage | 3 (1–6) | 3 (1–5) | 2 (0–5) |
| HAI score | 10 (0–18) | 5 (1–11) | 1.5 (0–13) |
| Bile duct damage, n (%) | |||
| Pericholangitis | 14 (45) | 9 (82) | 10 (56) |
| Periductal fibrosis | 4 (13) | 8 (73) | 14 (74) |
| Ductal proliferation | 18 (58) | 9 (82) | 11 (61) |
Abbreviations: AIH, autoimmune hepatitis; ASC, autoimmune sclerosing cholangitis; BL, baseline; ERCP, endoscopic retrograde cholangiopancreatography; HAI, Histologic Activity Index; IAIHG, International Autoimmune Hepatitis Group; IBD, inflammatory bowel disease; IgG, immunoglobulin G; INR, international normalized ratio; MRCP, magnetic resonance cholangiopancreatography; PSC, primary sclerosing cholangitis.