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The M2 antibodies belong to the group of Anti-Mitochondrial Antibodies (AMA) and are strongly associated with PBC. At least 9 distinct AMA have been identified, which have been classified M1-M9 according to their antigen specificity and disease association. Of these, only the M2 subtype approaches absolute specificity for PBC. Indeed, about 95 % of PBC patients have M2 autoantibodies and, conversely, about 90 % of asymptomatic individuals who are found to be M2-positive on routine screening can be shown to have underlying PBC on further investigation.

Centromere antibodies, also called Anti-Centromere Antibodies (ACA), are directed against Centromere-associated Proteins (CENP) and mainly two polypeptides designated CENP-A (19 kD) and CENP-B (80 kD). The combined detection of CENP-A and CENP-B antibodies is highly specific for systemic sclerosis (diagnostic specificity up to 99 %). With the determination of CENP-A and CENP-B antibodies nearly all of the systemic sclerosis relevant anti-centromere antibody reactivities as seen by indirect immunofluorescnce on HEp-2 cells can be detected. ACA are also detectable in Primary Biliary Cirrhosis (PBC) patients. In this case, half of the patients have also a Scleroderma.

Centromere antibodies, also called Anti-Centromere Antibodies (ACA), are directed against Centromere-associated Proteins (CENP) and mainly two polypeptides designated CENP-A (19 kD) and CENP-B (80 kD). Antibodies against CENP-A are most of the time detected in combination with antibodies against CENP-B. However, it is reported that CENP-A antibodies can be found in Scleroderma patients before the expression of CENP-B antibodies. The combined detection of CENP-A and CENP-B antibodies is highly specific for systemic sclerosis (diagnostic specificity up to 99 %). With the determination of CENP-A and CENP-B antibodies nearly all of the systemic sclerosis relevant anti-centromere antibody reactivities as seen by indirect immunofluorescence on HEp-2 cells can be detected.

Sm/RNP antibodies are directed against the U1 snRNP complex which is composed of Sm and the three polypeptides of the RNP antigen. Anti-Sm/RNP antibodies are very sensitive markers for Sharp Syndrome. Their sensitivity is 100 %.

The anti-SSA antibodies are directed against SSA (Ro), a protein of 60 kD which belongs to small cytoplasmic ribonucloprotein complexes, the hY-RNP complexes also known as Ro/SSA hY-RNA. These autoantibodies are diagnostic markers and classification criteria for Sjögren’s Syndrome. They are also associated with SLE and Subacute Cutaneous Lupus Erythematosus (SCLE) and present in 90 % of patients with Neonatal Lupus Erythematosus (NLE).