Presence and absence of characteristic signs of autoimmune disease in PBC
| Autoimmune disease signs | The presence or absence of a sign in the PBC | Explanation | References |
|---|---|---|---|
| Immune dysfunction leading to loss of tolerance to certain tissues and proteins | In doubt | AMAs persist after liver transplantation but do not correlate with disease recurrence | [1, 9, 10] |
| Autoimmune autoantibodies | Yes | АМАs, 95% (+); 5% (–) | [7, 21] |
| Approximately 50% of patients with PBC have antinuclear autoantibodies: anti-gp210, anti-Sp100, anti-p62 | [22] | ||
| Development of immune complex-mediated inflammation; damaging effect of immune complexes on healthy tissues | In doubt | AMAs are formed in experimental models of PBC after stimulation with recombinant E2 pyruvate dehydrogenase protein without causing inflammation or bile duct destruction | [1] |
| Systemic (multiple organ) dysfunction of the internal organs | No | Local lesion of BECs of small bile ducts | [9] |
| There is a correlation between disease severity and antibody titers | No | Antibody titers are not correlated with disease severity | [1, 4] |
| Identifying the antigen has not led to a clear understanding of the primacy origin of AMAs and the disease | |||
| Women more likely to be affected | Yes | Female to male ratio 9:1 | [23] |
| Chronic course of disease | Yes | Chronic, gradually progressive course of the disease | [1, 10, 23] |
| Spontaneous remissions are possible, alternating with exacerbations | No | Disease course without remissions and exacerbations of disease activity | [4] |
| May occur during the childhood years of life | No | PBC has no analogs in children | [10] |
| PBC is more common between 40 and 60 years | |||
| Positive effects of immunosuppressive therapy and genetically engineered biologics (targeted therapy) | No | Immunotherapy is not effective | [24–26] |
| Occasionally seen associated with other autoimmune diseases | Yes | The probability of association with extrahepatic autoimmune diseases in patients with PBC is over 60% | [27–29] |
| The most common combination is PBC with Sjögren’s syndrome |
+: presence of antimitochondrial autoantibodies; –: absence of antimitochondrial autoantibodies. PBC: primary biliary cholangitis; AMAs: antimitochondrial autoantibodies; E2: dihydrolipoyl transacetylase; BECs: biliary epithelial cells
