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Clinical and pathological characteristics of the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome
Author(s) -
SUZUKI YOSHIYUKI,
ARASE YASUJI,
IKEDA KENJI,
SAITOH SATOSHI,
TSUBOTA AKIHITO,
SUZUKI FUMITAKA,
KOBAYASHI MASAHIRO,
AKUTA NORIO,
SOMEYA TAKASHI,
MIYAKAWA YUZO,
KUMADA HIROMITSU
Publication year - 2004
Publication title -
journal of gastroenterology and hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.214
H-Index - 130
eISSN - 1440-1746
pISSN - 0815-9319
DOI - 10.1111/j.1440-1746.2004.03372.x
Subject(s) - medicine , overlap syndrome , autoimmune hepatitis , primary biliary cirrhosis , pathological , gastroenterology , hepatitis , pathology , bile duct , disease
Background and Aims: The defining of the autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) overlap syndrome as a separate clinicopathological entity has been controversial and temporally and geographically subjective. Methods: From 1979 until 2000, 227 patients diagnosed with AIH, PBC or the overlap thereof were treated. Cases with genuine AIH/PBC overlap syndrome were sorted out using close clinical follow up and serial liver biopsies. Results: Of the 227 patients, 19 (8.4%) were diagnosed with the AIH/PBC overlap syndrome. They all cleared a score >10 for the diagnosis of AIH, and tested positive for antimitochondrial antibodies during their courses. Long‐term follow up with frequent histological examinations, however, established the diagnosis of AIH/PBC overlap syndrome in only two (0.8%) patients. The most powerful factor distinguishing AIH from PBC was acidophilic bodies in lobules that were detected significantly more frequently in patients with AIH than PBC or spurious overlap syndrome (39/46 [85%] vs 3/85 [4%], P < 0.001). It was more reliable than bile‐duct lesions for the distinction of PBC from AIH. Conclusions: Although AIH/PBC overlap syndrome does exist, it is infrequent and needs to be diagnosed carefully using close clinical and histological follow up to enable timely and effective treatment.