Also Known As
Centromere Antibody
ACA
This article was last reviewed on
This article waslast modified on April 27, 2020.
At a Glance
Why Get Tested?

To detect the presence of anticentromere antibodies; to help diagnose limited cutaneous scleroderma, a subtype of scleroderma

When To Get Tested?

When you have one or more symptoms that suggest CREST syndrome; when you have a positive result on an antinuclear antibody (ANA) test

Sample Required?

A blood sample drawn from a vein in your arm

Test Preparation Needed?

None

What is being tested?

The anticentromere antibody (ACA) is an autoantibody, a protein produced by the immune system that mistakenly targets the body's own tissues. ACA is one of several antinuclear antibodies. It targets the centromere, a component of the chromosomes (structures in the nucleus) found in all nucleated cells in our bodies except red blood cells. The ACA test detects and measures the amount of anticentromere antibody found in blood to help diagnose a form of scleroderma.

Scleroderma (also known as systemic sclerosis) is a group of rare connective tissue disorders. (For more details, see the condition article on Scleroderma.) There are two distinct subtypes of systemic sclerosis that are defined based on how much of the skin is affected:

  • Diffuse cutaneous systemic sclerosis that affects the entire body
  • Limited cutaneous systemic sclerosis that is typically associated with one or more symptoms that are known collectively as CREST syndrome

CREST syndrome symptoms include:

  • Calcinosis – calcium deposits under the skin
  • Raynaud phenomenon – episodes of decreased blood flow to fingers and toes, causing them to turn white and blue
  • Esophageal dysfunction – difficulty swallowing, acid reflux, and heartburn
  • Sclerodactyly – tight, thick, shiny skin on the hands and fingers
  • Telangiectasia – red spots on skin due to swollen capillaries

Other signs that may be considered in scleroderma include fingertip lesions, abnormal capillaries in the skin just below the fingernails, high blood pressure in the lungs (pulmonary arterial hypertension) and/or scarring of the lungs (interstitial lung disease).

ACA is found in about 60-80% of people who have limited cutaneous scleroderma while only about 5% of patients with diffuse scleroderma have ACA. ACA can be present in up to 95% of people who have CREST syndrome.

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Common Questions
  • How is the test used?

    The anticentromere antibody (ACA) test is primarily ordered to help diagnose the autoimmune disorder limited cutaneous scleroderma, a form of systemic scleroderma, and CREST syndrome. The test may be used to distinguish between this and other conditions with similar symptoms.

    An ACA test may be ordered along with other tests for autoantibodies, including ANA (antinuclear antibody) testing. ACA testing may be used to provide your healthcare practitioner with additional information if an ANA test is positive, especially if the test produces a certain pattern characteristic of particular antibodies. (For more on this, see the "What does the test result mean?" section in the ANA article.)

    An ACA test may be ordered along with a Scl-70 (anti-topoisomerase I) test. Scl-70 is another autoantibody that may be present with scleroderma. An anti-RNA polymerase III autoantibody (ARA) test may also be ordered in the diagnosis of systemic sclerosis. These autoimmune antibody tests could be performed as part of an Extractable Nuclear Antigen (ENA) antibody panel, or other antibody panels for autoimmune disorders and/or systemic sclerosis.

  • When is it ordered?

    The ACA test may be ordered when you have a positive result on an ANA test and/or one or more of the symptoms associated with CREST. These symptoms include:

    • Calcinosis – calcium deposits under the skin
    • Raynaud phenomenon – episodes of decreased blood flow to fingers and toes, causing them to turn white and blue
    • Esophageal dysfunction – difficulty swallowing, acid reflux, and heartburn
    • Sclerodactyly – tight, thick, shiny skin on the hands and fingers
    • Telangiectasia – red spots on skin due to swollen capillaries
  • What does the test result mean?

    If a your ACA result is positive, indicating you have anti-centromere antibodies in your blood, and you have symptoms of CREST, then it is likely that you have limited cutaneous scleroderma. ACA is found in about 60-80% of people who have limited cutaneous scleroderma and can be present in up to 95% of those who have CREST syndrome. Around 5-7% of ACA positive patients develop diffuse cutaneous scleroderma.

    If your ACA result is negative, then it is likely that your symptoms are due to another condition. However, it is possible, though rare, that you have limited cutaneous scleroderma and do not produce anticentromere antibodies, resulting in a negative ACA test.

  • Is there anything else I should know?

    ACA can be positive in some other autoimmune disorders, such as lupus, rheumatoid arthritis, or primary biliary cirrhosis.

    Typically, only people who have CREST symptoms are tested for ACA. However, some people may be positive for ACA prior to the development of these symptoms and ACA may be performed in conjunction with testing for other autoimmune disorders such as ANA testing.

    The amount of ACA present does not, in general, correlate to the severity of signs and symptoms.

  • What other lab tests may be done?

    In addition to ACA testing, other laboratory tests may be ordered to monitor your health status, including:

  • Is there anything I can do to decrease my anticentromere antibody level?

    No, it does not respond to lifestyle changes.

  • Will my anticentromere antibody ever go away?

    Concentrations of ACA in the blood may vary over time, but once you have developed ACA, you will continue to produce them for life.

  • Can anticentromere testing be done at my healthcare practitioner's office?

    No. ACA testing requires collection of a blood sample by a trained medical professional. The testing itself requires specialized equipment and trained medical laboratory personnel. It is not offered by every laboratory and usually is performed by a reference laboratory.

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Related Content

On This Site

Tests
Antinuclear Antibody (ANA)
Autoantibodies
Extractable Nuclear Antigen Antibodies (ENA) Panel
Conditions
Scleroderma
Autoimmune Diseases

Elsewhere On The Web

National Institute of Arthritis and Musculoskeletal and Skin Diseases: Sclerode…
FamilyDoctor.org: Scleroderma
MedlinePlus Medical Encyclopedia: Scleroderma
Womenshealth.gov: Autoimmune diseases
Scleroderma Research Foundation
View Sources

Sources Used in Current Review

2020 review performed by Nadia Ayala-Lopez, MLS (ASCP), PhD, Clinical Chemistry Fellow, Department of Pathology, Johns Hopkins University School of Medicine. 

Nihtyanova, S., Denton, C. (2010). Autoantibodies as predictive tools in systemic sclerosis. Nat Rev Rheumatol 6, 112–116.

Van den Hoogen F, Khanna D, Fransen J, et al. (2013). 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Annals of the Rheumatic Diseases; 72:1747-1755.

Ho, K. T., & Reveille, J. D. (2003). The clinical relevance of autoantibodies in scleroderma. Arthritis Research & Therapy, 5(2), 80–93.

Sources Used in Previous Reviews

Yoon, J. and Raugi, G. (2012 January 13). CREST Syndrome. Medscape Reference [On-line information]. Available online at http://emedicine.medscape.com/article/1064663-overview. Accessed February 2012.

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Hill, H. and Tebo, A. (Updated 2011 May). Scleroderma - Systemic Sclerosis. ARUP Consult [On-line information]. Available online at http://www.arupconsult.com/Topics/SystemicSclerosis.html. Accessed February 2012.

(Updated 2010 May). Handout on Health: Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases [On-line information]. Available online at http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp. Accessed February 2012.

(©2012). Scleroderma, How is it Diagnosed? Arthritis Foundation [On-line information]. Available online at http://www.arthritis.org/disease-center.php?disease_id=26&df=diagnosed. Accessed February 2012.

Granito, A. (2012 January 31). Antinuclear Antibodies as Ancillary Markers in Primary Biliary Cirrhosis. Medscape News Today from Expert Rev Mol Diagn 12(1):65-74. [On-line information]. Available online at http://www.medscape.com/viewarticle/755110. Accessed February 2012.

Bellando-Randone, S. et. al. (2010 December 9). Patient Subgroups and Potential Risk Factors in Systemic Sclerosis: Is There a Possibility of an Early Diagnosis? Medscape Today from Int. J. Clin. Rheumatol 5(5):555-564. [On-line information]. Available online at http://www.medscape.com/viewarticle/731883. Accessed February 2012.

Assassi, S. et. al. (2009 October). Primary Biliary Cirrhosis (PBC), PBC Autoantibodies, and Hepatic Parameter Abnormalities in a Large Population of Systemic Sclerosis Patients. J Rheumatol. v36(10): 2250–2256. [On-line information]. PDF available for download at http://ukpmc.ac.uk/articles/PMC2885441/pdf/nihms201893.pdf. Accessed February 2012.

Magrey, M. and Abelson, A. (© 2000-2011). Laboratory Evaluation of Rheumatic Diseases. Cleveland Clinic [On-line information]. Available online at http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/laboratory-evaluation-rheumatic-diseases/. Accessed February 2012.

Makover, M. (Updated 2011 February 14). Scleroderma. MedlinePlus Medical Encyclopedia [On-line information]. Available online at http://www.nlm.nih.gov/medlineplus/ency/article/000429.htm. Accessed February 2012.

Hajj-ali, R. (Updated 2008 February). Systemic Sclerosis. Merck Manual for Healthcare Professionals [On-line information]. Available online through http://www.merckmanuals.com. Accessed February 2012.

Li, S. (Updated 2012 January 30). Pediatric Raynaud Phenomenon. Medscape Reference from [On-line information]. Available online http://emedicine.medscape.com/article/1355650-overview. Accessed February 2012.

Harris, M. and Rosen, A. (2003 November 18). Autoimmunity on Scleroderma: The Origin, Pathogenic Role, and Clinical Significance of Autoantibodies. Medscape Today from Curr Opin Rheumatol 15(6). [On-line information]. Available online http://www.medscape.com/viewarticle/463925. Accessed February 2012.

Pagana, K. D. & Pagana, T. J. (© 2011). Mosby's Diagnostic and Laboratory Test Reference 10th Edition: Mosby, Inc., Saint Louis, MO. Pp 73.

Reviewed January 2016 by Boris Calderon, MD, DABCC, FACB, NIH.

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(January 25, 2016). Varga, J. Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults. In: UpToDate., Axford, JS (Ed). UpToDate, Waltham, MA. Available online at http://www.uptodate.com/contents/diagnosis-and-differential-diagnosis-of-systemic-sclerosis-scleroderma-in-adults. Accessed on January 25, 2016.

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