Variant Syndromes of Autoimmune Liver Diseases: Classification, Diagnosis and Management
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Variant Syndromes of Autoimmune Liver Diseases: Classification, Diagnosis and Management. / Weiler-Normann, Christina; Lohse, Ansgar W.
In: DIGEST DIS, Vol. 34, No. 4, 2016, p. 334-9.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Variant Syndromes of Autoimmune Liver Diseases: Classification, Diagnosis and Management
AU - Weiler-Normann, Christina
AU - Lohse, Ansgar W
N1 - © 2016 S. Karger AG, Basel.
PY - 2016
Y1 - 2016
N2 - The term 'overlap syndrome' has been used to describe the presence of both autoimmune hepatitis and primary biliary cholangitis or primary sclerosing cholangitis in the past. As this term is misleading, the term 'variant syndrome' should be used preferably. Laboratory features, serology, histology and bile duct imaging contribute to the diagnosis of 'variant syndromes'. Patients with a suspected variant syndrome should receive a complete work-up with liver histology, serology and - if not conclusive, bile duct imaging. Liver histology is usually reliable to recognize secondary autoimmune hepatitis in patients with primary cholestatic disease. An histological activitiy index of >4 usually is commonly seen in patients with variant syndrome. Identification of variant syndrome is very important, as appropriate - in most cases additional - immunosuppressive treatment is necessary and most patients will respond promptly.
AB - The term 'overlap syndrome' has been used to describe the presence of both autoimmune hepatitis and primary biliary cholangitis or primary sclerosing cholangitis in the past. As this term is misleading, the term 'variant syndrome' should be used preferably. Laboratory features, serology, histology and bile duct imaging contribute to the diagnosis of 'variant syndromes'. Patients with a suspected variant syndrome should receive a complete work-up with liver histology, serology and - if not conclusive, bile duct imaging. Liver histology is usually reliable to recognize secondary autoimmune hepatitis in patients with primary cholestatic disease. An histological activitiy index of >4 usually is commonly seen in patients with variant syndrome. Identification of variant syndrome is very important, as appropriate - in most cases additional - immunosuppressive treatment is necessary and most patients will respond promptly.
KW - Journal Article
U2 - 10.1159/000444472
DO - 10.1159/000444472
M3 - SCORING: Journal article
C2 - 27170386
VL - 34
SP - 334
EP - 339
JO - DIGEST DIS
JF - DIGEST DIS
SN - 0257-2753
IS - 4
ER -