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Objective To analyze the clinical characteristics of 178 primary biliary cholangitis (PBC) patients with features of autoimmune hepatitis (AIH). Methods Four hundred and sixty-one PBC patients diagnosed in the Department of Gastroenterology, Xijing Hospital Affiliated to Air Force Medical University from December 2008 to December 2018 were included in this study. According to the diagnostic criteria of PBC-AIH, AIH-PBC and PBC, they were divided into PBC-AIH OS group(n=50), AIH-PBC group (n=178) and pure PBC group (n=233). Comparison of general clinical symptoms of three groups (jaundice, fatigue, pruritus, etc.); biochemical and immunological indices [alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), gamma glutamyltranspeptidase (GGT), immunoglobulin G/M (IgG, IgM), total/direct bilirubin (TBIL, DBIL), albumin/globulin (ALB, GLB)]; autoantibodies [antinuclear antibody (ANA), antimitochondrial antibody (AMA), anti-smooth muscle antibody (ASMA)]; stage and grade of the inflammation and fibrosis of the liver, noninvasive fibrosis score of fibrosis index based on the four factors (FIB-4) and aspartate aminotransferase/platelet ratio index (APRI); 5-year cumulative incidence of adverse events. Results The incidence of jaundice and inappetence in AIH-PBC group and PBC group was significantly lower than that in PBC-AIH OS group (P<0.05). The differences of ALT, AST, TBIL, IgG, IgM and GLB between the groups were statistically significant (P<0.05), and AIH-PBC group and PBC-AIH OS group were mostly higher than pure PBC group. Autoantibody detection showed that the positivity rates of SSA and Ro-52 antibodies in AIH-PBC group were significantly higher than those in pure PBC group, while the positivity rates of ASMA, soluble liver antigen/liver pancreatic antigen antibody (SLA/LP) and SSB were significantly lower than those in PBC-AIH OS group (P<0.05). The IgG and IgM of AIH-PBC group were significantly higher than those of pure PBC group (P<0.05). The liver histopathological stage, FIB-4 and APRI indexes of AIH-PBC group and PBC-AIH OS group were significantly higher than those of pure PBC group (P<0.05). The 5-year cumulative adverse event incidence of PCB-AIH OS group was significantly higher than that of AIH-PBC group, and that of AIH-PBC group was significantly higher than that of pure PBC group (P<0.05). Conclusions The clinical manifestations of AIH-PBC patients are significantly different from those of PBCAIH OS and pure PBC patients, and their long-term prognosis is worse than that of pure PBC patients.
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| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |
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