Table 1.
Baseline characteristics of patients at diagnosis of AIH-PBC variant syndrome and control cohorts.
| Characteristics | AIH-PBC Cohort (n = 90) | AIH Control Cohort (n = 100) | PBC Control Cohort (n = 100) |
|---|---|---|---|
| Patient age at diagnosis (years), median (range) | 49 (19–77) | 64 (25–86) | 76 (47–96) |
| Male/female, n (%) | 10 (11)/80 (89) | 29 (29)/71 (71) | 0 (0)/100 (100) |
| Caucasian, n (%) | 67 (74) | 93 (93) | 88 (88) |
| BMI (kg/m2), median (range) | 26 (19–40) | 23 (17.7–28.3) | 22 (17.6–27.7) |
| Length of follow-up (years), median (range) | 7 (0.5–21) | 7 (0–20) | 8 (0–19) |
| Liver biochemical tests, n (%) | |||
| ALT level, ×ULN | 2.9 (1.1–55.7) | 6.1 (0.3–50.3) | 1 (0.2–11.2) |
| AST level, ×ULN | 4.2 (1.2–51.1) | 8.1 (0.5–85.0) | 1 (0.2–16.6) |
| ALP level, ×ULN | 1.4 (0.3–6.3) | 1.2 (0.3–8.0) | 1.5 (0.5–6.1) |
| GGT level, ×ULN | 3.5 (0.2–19.6) | 2.3 (0.2–18.1) | 1.5 (0.2–18.8) |
| Bilirubin level, ×ULN | 0.6 (0.2–18.6) | 0.9 (0.1–20.6) | 0.4 (0.1–5.6) |
| Albumin level, ×LLN | 0.9 (0.8–1.8) | 0.9 (0.7–1.5) | 0.8 (0.7–1.9) |
| IgM level, ×ULN | 1.3 (0–3.9) | 0.6 (0.1–1.6) | 1.2 (0.2–2.9) |
| IgG level, ×ULN | 1 (0.3–2.3) | 1.1 (0.4–2.9) | 0.7 (0.5–1.4) |
| Histological features | |||
| of AIH * | 15 (16.7) | 100 (100) | |
| of PBC ** | 16 (17.8) | ||
| of AIH and PBC | 59 (65.6) | 100 (100) | |
| Clinical manifestation of autoimmune diseases |
|||
| Initial manifestation of AIH | 14 (15.6) | 100 (100) | |
| Initial manifestation of PBC | 19 (21.1) | 100 (100) | |
| Simultaneous manifestation of AIH and PBC | 47 (52.2) | ||
| n. a. | 10 (11.1) | ||
| Significant liver fibrosis, n (%) | 18 (20.0) | 8 (8.0) | 13 (13.0) |
| Liver cirrhosis, n (%) | 16 (17.8) | 5 (5.0) | 11 (11.0) |
| MELD, median (range) | 10 (5–23) | 11 (11–27) | 10 (6–20) |
| Child class A/B/C, n (%) | 9 (18.9)/6 (6.7)/1 (1.1) | 3 (3.0)/1 (1.0)/1 (1.0) | 7 (7.0)/3 (3.0)/1 (1.0) |
| GLOBE Score pretreatment, median (range) | −3.56 (−8.99–2.66) | −6.24 (−9.44–4.29) |
* moderate or severe or severe periportal or periseptal lymphocytic piecemeal necrosis. ** florid bile duct lesions. AIH = Autoimmune Hepatitis; ALP = Alkaline Phosphatase; ALT = Alanine Aminotransferase; AST = Aspartate Aminotransferase; BMI = Body Mass Index; GGT = Gamma Glutamyltransferase; IgG = Immunoglobulin G; IgM = Immunoglobulin M; LLN = Lower Limit of Normal; MELD = Model for End-Stage Liver Disease; PBC = Primary Biliary Cirrhosis; ULN = Upper Limit of Normal.