Histological findings at presentation, HLA data, and outcome following standard steroid therapy in the 12 patients
| Liver histology | HLA | |||||||
|---|---|---|---|---|---|---|---|---|
| Patient No | Grade | Stage | Biliary changes | A | B | DR | Outcome | IAIHG score |
| Histological grading and staging according to Batts and Ludwig,19 as described in patients and methods. | ||||||||
| Biliary changes include one or more of the following: pericholangitis, apparent ductopenia, ductular proliferation, copper associated protein deposition, cholestasis. | ||||||||
| na, data not available; OLT, orthotopic liver transplant; IAIHG score, International Autoimmune Hepatitis Group score (including scoring for initial response to therapy).120 | ||||||||
| 1 | 3 | 0 | No | na | na | na | Complete response + relapse | 20 |
| 2 | 4 | 3 | No | na | na | na | No response. Still requires multiple therapy after 3 y. | 19 |
| 3 | 3 | 4 | Yes | 1, 10 | 7, 17 | 4, 13 | Partial response. Complete response after 2 y. | 18 |
| 4 | 4 | 0 | No | 3, 28 | 18, 35 | 4, 11 | Complete response + relapse. OLT after 4 y. Died 3 y post-OLT. | 21 |
| 5 | 4 | 0 | No | na | na | na | No response. Still requires multiple therapy after 5 y. | 19 |
| 6 | 3 | 4 | No | 1, 24 | 8, 39 | 4, 4 | Complete response. No relapse. Recent OLT (after 14 y). | 21 |
| 7 | 4 | 0 | No | na | na | na | Partial response. Still requires multiple therapy after 10 y. | 19 |
| 8 | 3 | 3 | Yes | 1, 2 | 35, 35 | 7, 13 | Complete response + relapse | 25 |
| 9 | 2 | 4 | Yes | na | na | na | Partial response. Still requires multiple therapy after 4 y. | 21 |
| 10 | 4 | 0 | No | 1, 32 | 51, 51 | 3, 7 | No response. Emergency OLT. Died 10 days post-OLT . | 16 |
| 11 | 0 | 4 | No | na | na | na | No response. Listed for OLT 3 y later but died pre-OLT. | 14 |
| 12 | 1 | 4 | No | 1, 2 | 5, 7 | 4, 11 | No response. OLT after 3 mo. Still alive 7 y post-OLT. | 16 |