ANTI-CENTROMERE ANTIBODIES

CLINICAL UTILITY:

Anti-centromere antibodies (ACA) are a marker for the CREST syndrome variant of PSS. This syndrome is characterized by the presence of the following features: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. These patients often have limited skin involvement and less renal disease than found in more severe forms of PSS. Occasional patients with diffuse scleroderma also have ACA.

Patients who develop CREST often present with Raynaud’s phenomenon years before other features of the syndrome are manifest. The presence of ACA may help to identify such patients early in the course of the disease, prior to development of other features of CREST.

ACA have been found also in the sera of some patients with primary biliary cirrhosis (PBC). The majority of the PBC patients studied also had some features of CREST. The pulmonary complications of CREST resemble clinical and pathological features of primary pulmonary hypertension, and rare patients with primary pulmonary hypertension have been reported to have ACA in their sera.

METHOD DESCRIPTION:

Anti-centromere antibodies are detected in patients’ sera by the indirect immunofluorescence method, using HEp-2 cells, a tissue culture substrate rich in mitotic figures.

The assay for centromere antibodies is performed daily, and is also included in our routine ANA test, because ANAs are screened on both rat liver tissue and HEp-2 cells. Since centromere pattern may not be visible on liver tissue, an ANA result of negative on liver, positive on HEp-2, can occur.

REFERENCE RANGE:

Negative.

SPECIMEN REQUIREMENTS:

0.5 ml serum. Freeze.