Table 1.
Clinical characteristics of autoimmune hepatitis (AIH) in South Korea: Result of multicenter studies supported by the Korean Association for the Study of the Liver (KASL), a single center study, and a population-based study using rare intractable disease (RID) registry
| KASL 1st Study | KASL 2nd Study | Single center study | RID analysis study | |
|---|---|---|---|---|
| Lee JH, et al. [6] (2004) | Kim BH, et al. [7] (2013) | Kil JS, et al. [8] (2010) | Kim BH, et al. [2] (2017) | |
| Study design | Retrospective, multicenter | Retrospective, multicenter | Retrospective, single center | Administrative database analysis |
| Year of enrollment | 1993-2004 | 2005-2009 | 1994-2008 | 2009-2013 |
| Number of enrolled patients | 172 | 343 | 86 | 4085 |
| Diagnostic criteria | Revised original | Revised original (n=311) 25%/65% | Revised original | ICD-10 code registered in Rare |
| Definite/probable AIH | 53%/ 47% | or simplified (n=250) 34%/39% | 21%/79% | intractable disease registry |
| Age (year), mean/median | 47.8 | 52.8 | /51 | /56 |
| Female/male ratio | 9 | 7.3 | 5.1 | 6.4 |
| Cirrhosis at presentation | 22% | 23% | 12.8% | 32.3% |
| Acute onset | 46% | |||
| Asymptomatic | 31% | 37% | ||
| Hepatic decompensation | 4.3% | |||
| Autoantibody positive rate | Autoantibody testing rate | |||
| ANA | 92% | 94% | 81.4% | 93.90% |
| SMA | 31% | 23% | 44.2% | 81.7% |
| Anti-LKM1 | 1% | 3% | 88.4% | |
| AMA | 7% | 11% | 3.4% | 67.5% |
| ALT (U/L), mean/median | 284 | /182 (31-1,251) | ||
| Total bilirubin (mg/dL), mean/median | 4.5 | /2.3 (0.3-39) | ||
| Immunoglobulin G (g/dL), mean/median | 1.72 UNL | 2,356 (1.4 UNL) | /2380 (1,330-4,640) | |
| Liver biopsy performed | 74% | 75.2% | 70.9% | 54.2% |
| Interface hepatitis (86%) | ||||
| Plasma cell (58%) | ||||
| Rosette (15%) | ||||
| Septal fibrosis/cirrhosis (20%/5%) | ||||
| Comorbidities | Thyroid disease, 6.4% | Thyroid disease, 18% | Rheumatoid arthritis, 8.1% | Thyroid disease, 6.9% |
| Systemic lupus erythematosus, 3.0% | Systemic lupus erythematosus,15.2% | Thyroid disease, 7.0% | Systemic lupus erythematosus, 3.0% | |
| Hemolytic anemia, 1.2% | Sjogren’s syndrome, 8.0% | Systemic sclerosis, 1.7%. | ||
| Primary biliary cholangitis, 0.6% | Rheumatoid arthritis, 4.8% | Rheumatoid arthritis, 0.3% | ||
| Raynaud’s synd, 3.2% | Dyslipidemia 17.9% | |||
| Systemic sclerosis, 2.4% | Primary biliary cholangitis, 7.4% | |||
| Therapeutic regimen | 2013 prescription | |||
| Corticosteroid | 33% | 38% | 14% | Corticosteroid 44.1% |
| Corticosteroid+Azathioprine | 37% | 37% | 86% | Azathioprine 38.0% |
| Other immunosuppressive therapy | 3% | |||
| No immunosuppressive therapy | 24% | 25% | ||
| Treatment response** | ||||
| Remission | (n=124) | |||
| Incomplete response | 70% | 85.70% | 83.70% | |
| Treatment failure | 10.50% | 12.80% | ||
| Relapse rate after drug. | 26% (n=112) | 3.90% | 3.50% | |
| withdrawal | 54.2% (n=24) | |||
| Overall survival rate | Progression free survival rate | Case fatality rate, 6.63% during 5 years | ||
| 5 year | 91.20% | |||
| 10 year | 85.50% | |||
| (median follow-up) | (43 months) | |||
| Liver transplantation | 1.1% | |||
| Hepatocellular carcinoma at diagnosis | 0.8% |
ALT, alanine aminotransferase; ANA, anti-nuclear antibody; SMA, anti-smooth muscle antibody; anti-LKM1, anti-liver kidney microsome type 1 antibody; AMA, anti-mitochondrial antibody.