[Autoimmune liver disease: diagnosis and therapy].
Autoimmune Hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and overlap syndromes of these three disease entities are regarded as autoimmune liver diseases. These conditions are important differential diagnoses of elevated liver function tests as about 10 % of liver transplantations in Europe and North America are for these indications. The diagnosis is often difficult but can be facilitated by sequential measurement of relevant autoantibodies, exclusion of other liver disease, ultrasound, ERCP and liver histology. In AIH immunosuppressive therapy has been shown to prevent or stop the development of cirrhosis and improve the prognosis of the patients decisively. In other autoimmune liver diseases this evidence is missing making individual therapeutic decisions necessary. Ursodesoxycholic acid (UDCA) seems to slow disease progression in particular in early stages of PBC.
I. Medizinische Klinik und Poliklinik Johannes-Gutenberg-Universität Mainz, Germany.
E M
EM
Bayer
C
C
Schramm
S
S
Kanzler
A W
AW
Lohse
D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000628:therapy; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D002648:Child; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000276:immunology* / Q000628:therapy; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000628:therapy; D006801:Humans; D007118:Immunoassay; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D008111:Liver Function Tests; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D011379:Prognosis; D014463:Ultrasonography; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D001323:Autoantibodies; D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid; D009173:Mycophenolic Acid; D001379:Azathioprine