Anti-centromere antibodies (ACA) are directed against centromere proteins. The serological detection of ACA is relevant for both diagnostics and differentiation. ACA can be found in 20% to 30% of SSc patients, most frequently in Caucasians. In most cases, ACA are associated with LSSc.
The presence of ACA, with a prevalence of 80% to 95%, is considered an indicator of a mild disease course and good prognosis. In DSSc, which also includes lung fibrosis, ACA are detected in around 8% of patients.
Furthermore, 15% to 30% of patients with primary biliary cirrhosis (PBC), which is also an autoimmune disease, express ACA. Antibodies against Disease Prevalence Centromeres Systemic sclerosis (Ssc) - limited form (LSSc) - diffuse form (DSSc) Primary biliary cirrhosis (PBC) 20% - 30% 80% - 95% approx. 8% 15% - 30%.
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Systemic sclerosis (SSc) is a chronic inflammatory autoimmune disease which occurs in phases and is characterised by accumulation of collagen in the skin and inner organs. Main symptoms of SSc include skin thickening and episodes of disturbed blood flow in the fingers (Raynaud's syndrome), particularly in cold weather or if the patient suffers from stress. SSc is further characterised by arthritic joint pains and symptoms in the gastrointestinal tract, lungs, heart, kidneys and other inner organs.
SSc is divided into:
- the diffuse form (DSSc),
- the limited form (LSSc)
- and PM/SSc or PM/SLE/SSc overlap syndrome.
DSSc affects the connective tissue of the lungs, kidneys, oesophagus and heart, with lung sclerosis being the most frequent cause of death. LSSc, which is equated to a large extent with CREST syndrome (calcinosis cutis, Raynaud's phenomenon, oesophagus motility disorder, sclerodactyly, teleangiectasis), affects the extremities rather than the inner organs. PM/SSc overlap syndrome is characterised by myositis, interstitial lung disease, arthritis, Raynaud's phenomenon, fever and hyperkeratosis of the hands.
Centromere
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