Overlap Syndromes of Autoimmune Hepatitis With Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

2008 ◽  
pp. 285-291
Author(s):  
Ulrich Beuers ◽  
Christian Rust
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis ◽  
Overlap Syndromes

scholarly journals Diagnosis and Management of the Overlap Syndromes of Autoimmune Hepatitis

2013 ◽  
Vol 27 (7) ◽  
pp. 417-423 ◽  
Author(s):  
Albert J Czaja
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Ursodeoxycholic Acid ◽  
Immunosuppressive Therapy ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Overlap Syndrome ◽  
Biliary Cirrhosis ◽  
Diagnostic Features ◽  
Overlap Syndromes

BACKGROUND: Autoimmune hepatitis may have cholestatic features that are outside the classical phenotype and that resemble findings in other immune-mediated liver diseases. These cholestatic phenotypes have been designated ‘overlap syndromes’.OBJECTIVES: To recognize the overlap syndromes in adults and manage them appropriately.METHODS: The MEDLINE database was reviewed for published experiences from 1984 to 2013.RESULTS: Patients with autoimmune hepatitis may exhibit features of primary biliary cirrhosis (7% to 13%), primary sclerosing cholangitis (6% to 11%) or a cholestatic syndrome without other diagnostic features (5% to 11%). These mixed phenotypes may represent classical autoimmune hepatitis with atypical features, transition states in the evolution of classical cholestatic syndromes, concurrent separate diseases or pathogenically distinct disorders. The ‘Paris criteria’ have been endorsed for the diagnosis of the overlap syndrome with primary biliary cirrhosis, and treatment with conventional immunosuppressive therapy alone or in combination with low-dose ursodeoxycholic acid can be guided by the serum alkaline phosphatase level. The overlap syndrome with primary sclerosing cholangitis or with cholestasis without diagnostic features is commonly treated with immunosuppressive therapy and ursodeoxycholic acid. Responses are variable and commonly incomplete (20% to 100% improvement) depending on the degree of cholestasis.DISCUSSION: The overlap syndromes are clinical descriptions rather than pathological entities, and the dominant component of the disease determines its designation and therapy. Cholestatic findings in autoimmune hepatitis influence the response to immunosuppressive therapy.CONCLUSION: The overlap syndromes must be considered in patients with autoimmune hepatitis and cholestatic findings, concurrent inflammatory bowel disease or steroid-refractory disease.

scholarly journals Overlap syndromes in hepatic autoimmune diseases

2017 ◽  
Vol 95 (10) ◽  
pp. 940-945
Author(s):  
V. I. Podzolkov ◽  
Natal’ya A. Dragomiretskaya ◽  
E. V. Volchkova ◽  
O. I. Mitrokhina
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Autoimmune Diseases ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Clinical Picture ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis ◽  
Overlap Syndromes

Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) comprise a group of autoimmune hepatic disorders. Sometimes, overlap syndromes occur, e.g. AIH/PBC or AIH/PSC. The authors consider autoimmune hepatic disorders with reference to pathogenesis, clinical picture, diagnostics, and treatment of overlap syndromes.

Autoimmunity in liver disease

2019 ◽  
pp. 245-252
Author(s):  
Gavin Spickett
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of liver diseases. Primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis are described.

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