Clin Mol Hepatol > Volume 24(1); 2018 > Article |
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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% |
Multicenter study | Single center study | RID analysis study | |
---|---|---|---|
Kim KA, et al. [28] (2010) | Park Y, et al. [29] (2015) | Kim KA, et al. [25] (2016) | |
Study design | Retrospective, multicenter | Retrospective single center | Administrative database analysis |
Year of enrollment | 1997-2008 | 2001-2011 | 2009-2013 |
Number of enrolled patients | 251 | 81 | 2824 (.20 year old) |
Diagnostic criteria | Diagnostic criteria (≥ 2/3) | Paris criteria (≥ 2/3) | ICD-10 code registered in Rare intractable disease registry |
1) Elevated ALP with GGT | 1) ALP > 2 x UNL or GGT > 5 xUNL | ||
2) AMA+ (≥1:40) | 2) AMA+ | ||
3) Compatible histology | 3) florid bile duct lesion on liver biopsy | ||
Age (year), mean/median | 54 | 53 | 57.4 |
Female/male ratio | 6.6 | 5.2 | 6.2 |
Cirrhosis at presentation | 23.5% | 32.3% | |
Asymptomatic | 60.6% | ||
Hepatic decompensation | 12.3% | ||
Autoantibody positive rate | Autoantibody testing rate | ||
ANA | 63.5% | 47% | 74% |
AMA | 98.4% | 100% | 92.3% |
Alkaline phosphatase, mean | 2.6 x UNL | 265 IU/L | |
Total bilirubin (mg/dL), mean/median | 1.9 | 0.9 | |
Immunoglobulin M >1xUNL | 74% | Mean 533 mg/dL | |
Liver biopsy performed | 40% | 35% | |
Comorbidities | Hyperlipidemia, 17% | ||
Autoimmune hepatitis, 11% | |||
Thyroid disease, 6.5% | |||
Sjogren disease, 0.8% | |||
Systemic lupus erythematosus, 0.5% | |||
Systemic sclerosis, 0.3% | |||
Rheumatoid arthritis, 0.5% | |||
Therapy regimen | (n=244) | 2013 prescription | |
UDCA | 86% | 100% | 93% |
Corticosteroid/ cyclosporine/ azathioprine/ colchicine | 16%/7%/2%/0.4% | 9%/- / 5%/- | |
7% | |||
UDCA response at 1yr | (n=221) | (n=65) | |
70% (ALP reduction >40% or normal range) | 72.3% (ALP <3 xUNL, AST < 2UNL, Bilirubin <1 mg/dL) | ||
Overall survival rate | |||
5 year | 95% | 95% | |
Case fatality rate, 2.2% during 5 years | |||
Liver transplantation | 2.5% | ||
Hepatocellular carcinoma at diagnosis | 1.3% |
Sook-Hyang Jeong
https://orcid.org/0000-0002-4916-7990
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