Abstract: Objective To study the relationship between intrahepatic cholestasis in patients with Sjogren's syndrome (SS) and primary biliary cholangitis (PBC).Methods 32 SS patients with chronic intrahepatic cholestasis were divided into two groups according to whether anti-myocardial antibody (AMA)/AMA-M2 was positive or not. The clinical characteristics, laboratory examination and liver pathology of the two groups were compared.Results The clinical conditions of 32 patients were good, including 18 cases of fatigue (56%), 6 cases of pruritus (19%), 8 cases of abdominal distension (25%), 8 cases of splenomegaly (25%), and no patient had liver failure. There was no significant difference in TBIL [(62.4 ± 41.1) μmol/L, (59.6 ± 53.2) μmol/L], ALP [(324.6 ± 230.2) U/L, (305.4 ± 198.8) U/l] and GGT [(298.5 ± 203.2) U/L, (287.9 ± 194.2) U/L] (t=0.561, P=0.546; t=0.647, P=0.449; t=0.736, P=0.485). There was no significant difference between the two groups (χ²=0.029, P=0.985). All patients underwent liver biopsy. The liver biopsy results of all patients in AMA/AMA-M2 positive group were consistent with PBC. In the AMA/AMA-M2 negative group, the liver biopsy results of 9 patients were consistent with PBC, and the liver biopsy results of the remaining 2 patients showed nonspecific inflammation. The stage of PBC in the two groups was mostly in stage I or stage II.Conclusion Clinically, for SS patients with intrahepatic cholestasis, whether AMA is positive or not, liver biopsy should be considered to further determine whether PBC is accompanied.

Key words: Sjogren's syndrome, primary biliary cholangitis, Cholestasis