A Clinicla Study of Autoimmune Cholangitis

Lee, Young Sang; Suh, Dong Jin; Chung, Young Wha; Yoo, Eun Sil; Lee, Geun Chan; Kim, Hyung Geon; Park, Neung Hwa; Kang, Gyeong Hoon

Abstract

BACKGROUND/AIMS: Autoimmune cholangitis is a disease with clinical and pathologic features of primary biliary cirrhosis(PBC) but with negative antimitochondrial antibody(AMA) and positive antinuclear antibody(ANA) tests. The terms such as AMA negative PBC, immunocholangitis, autoimmune cholangiopathy, or autoimmune hepatobiliary overlap syndrome(AH-OS) type 1 have been suggested. Autoimmune cholangitis may be immunologically distinct from PBC because an antibody to carbonic anhydrase II in the bile duct epithelium, which is uncommon in PBC, is frequently detected in the sera. The aim of this study is to assess the clinical characteristics and pathologic features of 7 cases with autoimmune cholangitis.
METHODS
We retrospectively analyzed 7 cases whose clinical and pathologic features were suggestive of PBC but serum antimito- chondrial antibody(AMA) was absent and antinuclear antibody(ANA) was present. Age and sex, initial symptoms and signs, associated diseases, biochemical and serologic data, and liver biopsy features were studied.
RESULTS
All 7 patients were women ranging in age from 40 years to 57 years(mean, 48.7 years). Of 7 patients, 5 were symptomatic: pruritus and chronic fatigue were common(4 cases respectively). All patients were negative for AMA by immunofluorescence studies. HBs Ag and anti-HCV were negative. Radiological studies including ultrasonography and ERCP revealed no abnormality in biliary tree. All patients were positive for antinuclear antibody: five at a titer of 1: 640, one at l: 320, and one at 1: 80. Speckled ANA pattem was noted in 4 patients while a homogenous pattern in 3. All the patients had marked elevation of alkaline phos- phatase(means: 777 IU/L) and gamma glutamyl transferase(means: 217 IU/L) with only modest elevation of aminotransferases. Serum IgM values were normal in three patients studied. Anti- smooth muscle antibody was found positive in 1 among 5 patients studied. Associated diseases were adenomatous goiter(1 case), Hashimoto's thyroiditis(l case), and sicca syndrorne(1 case). All 7 liver biopsy specimens showed interlobular bile ductopenia, portal inflammatory infiltration and piecemeal necrosis. Bile ductular proliferation and portal fibrosis were noted in six cases. Piecemeal necrosis, granuloma, and fibrosis were commonly found.
CONCLUSIONS
It is suggested that autoimmune cholangitis can be found in Korea. It should be considered in AMA-negative patients with clinical and pathologic features of PBC. Further studies are needed to assess the causes, pathogenesis, and treament of autoimmune cholangitis.