Test Catalog

Interpretive Handbook

Test 9278 :
Centromere Antibodies, IgG, Serum

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Centromere antibodies occur primarily in patients with the calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasis (CREST) syndrome variant of systemic sclerosis (scleroderma). CREST syndrome is characterized by the following clinical features: calcinosis, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia.(1) Centromere antibodies were originally detected by their distinctive pattern of fine-speckled nuclear staining on cell substrates used in the fluorescent antinuclear antibody test.(2) In subsequent studies, centromere antibodies were found to react with several centromere proteins of 18 kDa, 80 kDa, and 140 kDa named as CENP-A, CENP-B, and CENP-C, respectively.(3) Several putative epitopes associated with these autoantigens have been described. The CENP-B antigen is believed to be the primary autoantigen and is recognized by all sera that contain centromere antibodies.

Useful For Suggests clinical disorders or settings where the test may be helpful

Evaluating patients with clinical signs and symptoms compatible with systemic sclerosis including skin involvement, Raynaud phenomenon, and arthralgias


As an aid in the diagnosis of calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasis (CREST) syndrome

Interpretation Provides information to assist in interpretation of the test results

In various reported clinical studies, centromere antibodies occur in 50% to 96% of patients with calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasis (CREST) syndrome.


A positive test for centromere antibodies is strongly associated with CREST syndrome. The presence of detectable levels of centromere antibodies may antedate the appearance of diagnostic clinical features of CREST syndrome by several years.

Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

Centromere antibodies have also been described in some patients with primary biliary cirrhosis, and may occur in patients with rheumatoid arthritis or lupus erythematosus.

Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

<1.0 U (negative)

> or =1.0 U (positive)

Reference values apply to all ages.

Clinical References Provides recommendations for further in-depth reading of a clinical nature

1. White B: Systemic sclerosis. In Clinical Immunology Principles and Practice. Second edition. Edited by R Rich, T Fleisher, W Shearer, et al. St. Louis, Mosby-Year Book, 2001, Chapter 64, pp 64.1-64.10

2. Tan EM, Rodnan GP, Garcia I, et al: Diversity of antinuclear antibodies in progressive systemic sclerosis. Anti-centromere antibody and its relationship to CREST syndrome. Arthritis Rheum 1980;23:617-625

3. Kallenberg CG: Anti-centromere antibodies (ACA). Clin Rheum 1990;9:136-139

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