Conclusions
-
1.
Anti-M2 and anti-M9 antibodies are specific marker for the diagnosis of PBC especially when demonstrated by ELISA and Western blotting in parallel.
-
2.
Anti-M4 and anti-M8 always occur in association with anti-M2 and can be related to disease activity. In contrast, anti-M9 can be positive in the absence of anti-M2 and seems to be associated with a benign course of PBC.
-
3.
The analysis of the clinical relevance of the four different AMA profiles revealed that they correlate with the two different courses of PBC. Thus, the profiles A/B are associated with a benign course, and the profiles C/D with a more progressive course of the disease. These AMA profiles did not change greatly during the observation period of up to 18 years.
-
4.
Among the different ANA types which can be detected by IFL on cell cultures only two are of diagnostic relevance, namely the antibodies against nuclear dots and the antibodies against nuclear lamina. The latter can be demonstrated also by Western blotting and react with the disease-specific determinant at 200 kDa.
-
5.
A variety of other antibodies can also be detected in sera from PBC patients which are directed against highly conserved antigens such as actin, laminin, nuclear antigens or thyroglobulin. They may indicate heightened B cell activity but are of no clinical or diagnostic relevance.
-
6.
In sera from contact persons of PBC patients a further AMA type could be detected which seems to belong to the group of NOMA. By Western blotting it revealed the same specificity as sera from patients with different infectious disorders.
-
7.
The increased NOMA production in family members and the low incidence of NOMA in PBC patients suggest the presence of a stimulatory agent as well as a B cell defect in PBC patients, which is defined by the lack of the production of NOMA. This defect may also be responsible for a lowered resistance, hereby facilitating the chronic inflammatory process affecting small- and medium-seized bile ducts.
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Berg, P.A., Klein, R. Autoantibodies in primary biliary cirrhosis. Springer Semin Immunopathol 12, 85–99 (1990). https://doi.org/10.1007/BF00192685
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DOI: https://doi.org/10.1007/BF00192685