anticentromère

Anticentromere antibodies are autoantibodies directed against centromere-associated proteins, most notably centromere protein B (CENP-B). They are commonly identified in autoimmune serology and serve as important biomarkers for certain connective tissue diseases.

Detection typically begins with indirect immunofluorescence on HEp-2 cells, which reveals a characteristic discrete centromere pattern. This pattern is then confirmed by specific immunoassays targeting CENP-B or other centromere proteins. Anticentromere antibodies can be present in a subset of patients with systemic sclerosis and other autoimmune conditions, though their detection is most clinically relevant in systemic sclerosis testing.

The strongest clinical association is with limited cutaneous systemic sclerosis (lcSSc), formerly known as CREST syndrome. ACA positivity correlates with features such as calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. In systemic sclerosis, anticentromere antibodies occur in roughly a fifth to a quarter of patients and are more characteristic of the limited disease subset than the diffuse form. Some studies suggest ACA-positive patients have a relatively favorable prognosis regarding rapidly progressive interstitial lung disease and scleroderma renal crisis, though vascular and gastrointestinal complications can contribute to morbidity.

Pathophysiology remains poorly understood; the antibodies serve mainly as reliable serologic markers rather than proven drivers of tissue damage. Clinically, the presence of anticentromere antibodies helps refine diagnosis, define the likely disease course, and guide monitoring and management in patients with symptoms suggestive of systemic sclerosis and related autoimmune conditions.