This textbook deals with the typical and atypical manifestations of autoimmune hepatitis. It offers the individual with a clinical interest in this condition a comprehensive review of many topics of relevance to clinical practice in the management of patients with autoimmune hepatitis taking into account the most recent scientific findings. In addition to efficient diagnostic investigation, the clinical course and current state of the art for therapy including liver transplantation are, in particular, described in detail.
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978-3-8374-5259-4 (9783837452594)
Schweitzer Klassifikation
1. History 14
2. Epidemiology 18
3. Clinical features of autoimmune hepatitis 20
3.1. Clinical presentation 20
3.2. Extrahepatic diseases 20
4. Biochemical findings 22
5. Histology 24
5.1. Relevance of histology in the diagnosis of AIH 24
5.2. Characteristic histological patterns of AIH 24
5.3. Histological scoring systems and their criteria 26
5.3.1. Morphology of AIH on therapy 26
5.4. Histological patterns in overlap syndromes 27
5.4.1. AIH/PBC overlap 27
5.4.2. AIH/PSC overlap 28
5.4.3. AIH and chronic hepatitis C 28
6. Autoantibodies 30
6.1. Antibodies to cell nuclei (ANA) 31
6.2. Antibodies to smooth muscle (SMA) 32
6.3. Liver-kidney-microsome antibodies (LKM) 32
6.4. Antibodies to liver cytosol antigen type 1 (LC-1) 33
6.5. Soluble liver antigen/liver-pancreas antigen antibodies (SLA/LP) 34
6.6. Antibodies to asialoglycoprotein receptor (ASGPR) 34
6.7. Anti-neutrophil cytoplasm antibodies (ANCA) 34
6.8. AIH subtypes 35
7. Genetics 38
7.1. Histocompatibility system (MHC system) 38
7.2. Autoimmune hepatitis and the MHC complex 40
7.3. AIH and genetic factors unrelated to the MHC complex 42
7.4. Autoimmune polyglandular syndromes 42
7.4.1. Clinical features of the APECED syndrome 42
7.4.2. Autoantibodies in the APECED syndrome 43
7.4.3. Genetics of the APECED syndrome 44
8. Diagnosis of autoimmune hepatitis 48
8.1. Descriptive criteria 48
8.2. The scoring system 50
8.3. The "simplified scoring system" 51
9. Treatment of autoimmune hepatitis 54
9.1. Indication for therapy 54
9.1.1. Absolute indication 54
9.1.2. Relative indication 54
9.2. Treatment in adults 55
9.3. Treatment in children 56
9.4. Side effects of drug therapy 56
9.4.1. Glucocorticoids 56
9.4.2. Azathioprine 57
9.5. Treatment - special situations 58
9.5.1. Hepatic cirrhosis 58
9.5.2. Elderly patients 58
9.5.3. Pregnancy 58
9.5.4. Incomplete response/incomplete remission 59
9.5.5. Treatment failure 59
9.6. Discontinuation of therapy 59
9.7. Relapse after discontinuation of immunosuppressant therapy 61
9.8. Alternatives and new therapies 61
9.8.1. Budesonide 65
9.8.2. Ciclosporin A 65
9.8.3. Tacrolimus 65
9.8.4. Mycophenolate mofetil (MMF) 65
9.8.5. Methotrexate (MTX) 66
9.8.6. Cyclophosphamide 66
9.8.7. Ursodeoxycholic acid 66
10. Clinical course 68
10.1. Symptomatic patients 68
10.2. (Asymptomatic) patients with mild disease 68
10.3. Elderly patients 69
10.4. Male patients 69
10.5. Children 70
10.6. Hepatic cirrhosis 70
10.7. Pregnancy 70
10.8. Tumours 71
11. Liver transplantation 74
11.1. Liver transplantation as a treatment option in AIH - who and when? 74
11.2. Course following transplantation 75
11.3. Recurrence of AIH after liver transplantation 75
11.4. De novo AIH following liver transplantation 77
12. Overlap syndrome 80
12.1. Primary biliary cirrhosis 80
12.1.1. AMA-negative PBC 81
12.2. Primary sclerosing cholangitis 82
12.2.1. IgG4-mediated sclerosing cholangitis 83
12.3. Overlap syndromes 83
12.3.1. AIH/PBC overlap syndrome 84
12.3.2. PSC/AIH overlap syndrome 85
12.3.3. PBC/PSC overlap syndrome 85
12.3.4. AIH/HCV overlap syndrome 85
13. References 88
Index 110
