Table 1.
Clinical characteristics of patients with autoimmune hepatitis with liver failure at initial presentation
| Villamil et al[22]1(n = 28) | Kessler et al[23](n = 10) | Miyake et al[24](n = 11) | Ichai et al[25](n = 16) | Verma et al[7](n = 20) | |
| Study design | Retrospective | Retrospective | Retrospective | Retrospective | Retrospective |
| Age (yr)2 | 41 | 40 ± 15.9 | 53 (16-75) | 36 ± 13.1 | 41.3 ± 14.2 |
| Definition of LF | NA | NA | PT < 40% and HE ≥ grade 2 | HE within 12 wk of jaundice | Any grade HE and/or INR > 2 |
| Symptoms duration2 | NA | 3.2 wk | 24 (16-52) d | NA | 2.1 ± 2.5 mo3 |
| Female | NA | 8 (80%) | 11 (100%) | 14/16 (87.5%) | 15 (75%) |
| Ethnicity or country of origin | South American | 80% White | Japanese | French | 70% black |
| Definite/probable AIH (IAIHG4 criteria) | NA | NA5 | 3(36%)/8 (64%) | NA | 9(45%)/11(55%) |
| LC/LKM6 positive ANA/SMA7 positive | 6 (21.4) | 1 (10%) | 3 (18.7%) | NA | |
| 22 (78.5%) | 7 (70%) | NA | 11 (68.7%) | 20 (100%) | |
| Bilirubin2 (mg/dL) | 3988 | 16.97 ± 9.83 | 20.6 (5.9-31) | 425 (278-850)8 | 19.3 ± 10.3 |
| AST or ALT2 | NA | 1179 ± 1127.17 | 220 (59-1094) | 678 (60-2867) | 1147.1 ± 711.4 |
| INR2 or PT | 30% | 49.3 ± 66.9 | 29% (6%-38%) | 5.36 (1.7-12.2) | 2.7 ± 1.4 |
| HE9 at onset | 28 (100%) | 8 (80%) | 11 (100%) | 10 (62.5%) | 19 (95%) |
| Cirrhosis | None | 2/10 (20%) | NA | None | 8/20 (40%) |
| MELD2 | NA | NA | NA | 37 (24-47) | 28 ± 7.41 |
| Sub-massive or massive necrosis (SMN, MN) | 19/23 (82.6%) | 5/10 (50%) | NA | 16/16 (100%) 15 needed LT and/or died | 12/19 (63.1%), 10 needed LT and or died |
| 17 needed LT and/or died | |||||
| Immunosuppressant regimen used | Prednisone 60 mg/d | Corticosteroids (Dose NA) and other10 | Prednisolone | Prednisone 1 mg/kg per day and other10 | Corticosteroids11 |
| 40-60 mg/d and steroid pulse | 20-1250 mg/d | ||||
| Poor prognostic criteria | 1: PT < 20%; 2: Grade 4 HE; 3: SMN at diagnosis; 4: 20% increase in PT at day 3 of steroids | NA | 1: High bilirubin at onset; 2: Worsening bilirubin during days 8-15 of steroid therapy | NA | 1: Absence of cirrhosis; 2: MELD > 28; 3: Worsening trend in bilirubin and INR after 3.7 ± 0.6 d of steroid therapy |
| Septic events | NA | NA | NA | 7 (43.7%), of whom 6 had received steroids | 2 (10%), of whom 1 received steroids |
Published only in abstract form;
Data presented as mean ± SD or median (range);
Duration from first symptom (and not necessarily jaundice/hepatic encephalopathy) to hospitalization;
IAIHG: International Autoimmune Hepatitis Group;
Met IAIHG criteria, data on probable or definite disease unavailable;
LKM/LC: Liver kidney microsomal antibody/liver cytosol antibody;
ANA/SMA: antinuclear antibody/anti-smooth muscle antibody;
Values in µmol/L;
HE: Hepatic encephalopathy;
Additional immunosuppression was used in nine patients in the study of Kessler et al (azathioprine, tacrolimus, mycophenolate mofetil, 6-mercaptopurine, cyclosporine) and in one patient in the study of Ichai et al (azathioprine and cyclosporine);
Included prednisone, hydrocortisone and methylprednisone, (converted to equivalent doses of prednisone); LT:Liver transplantation; PT: Prothrombin time; AIH: Autoimmune hepatitis.