Clinical and pathological characteristics of the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome.
OBJECTIVE
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.
Suzuki
Yoshiyuki
Y
Department of Gastroenterology, Toranomon Hospital, Tokyo, Japan. suzunari@interlink.or.jp
Arase
Yasuji
Y
Ikeda
Kenji
K
Saitoh
Satoshi
S
Tsubota
Akihito
A
Suzuki
Fumitaka
F
Kobayashi
Masahiro
M
Akuta
Norio
N
Someya
Takashi
T
Miyakawa
Yuzo
Y
Kumada
Hiromitsu
H
D000328:Adult; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D013577:Syndrome