Can anti-centromere antibodies be positive without systemic sclerosis?

Yes. Low-titre positivity is occasionally found in asymptomatic older adults and does not by itself establish disease. Anti-centromere antibodies are also seen in a subset of primary biliary cholangitis patients without overt scleroderma, where they may be associated with portal hypertension.

How does anti-centromere differ from anti-Scl-70?

Anti-centromere antibodies are associated with limited cutaneous disease, lower interstitial lung disease risk, and isolated pulmonary arterial hypertension. Anti-Scl-70 (topoisomerase I) antibodies mark diffuse cutaneous systemic sclerosis with higher rates of lung fibrosis. The two are mutually exclusive in most patients and define distinct disease subsets.

Is fasting required for the test?

No. Anti-centromere antibody testing does not require fasting or any special preparation before the blood draw.

Should the test be repeated to monitor disease activity?

No. Antibody titres do not reliably track disease activity or treatment response, so routine serial testing is not recommended. Repeat testing may be useful if an initial result was equivocal and clinical suspicion persists.

What is the typical pattern on immunofluorescence?

Anti-centromere antibodies produce a discrete punctate or speckled nuclear pattern on HEp-2 cells, reflecting the localisation of centromeres within interphase nuclei. This pattern prompts reflex or confirmatory testing for centromere-specific antibodies.

Can the result be positive in overlap syndromes?

Anti-centromere antibodies typically occur in isolation, but rare coexistence with other autoantibodies such as anti-Ro or anti-U1RNP can occur and suggests possible overlap connective-tissue disease. Clinical and serological correlation is required in such cases.

How long do results take?

Results are typically available within three to five business days from specimen receipt at Quest. Turnaround may be longer if reflex or confirmatory immunoblot is performed.

BML-INF-083autoimmune inflammationUSA

Centromere B Antibody

The Centromere B Antibody test detects serum autoantibodies against centromere protein B, highly specific for limited cutaneous systemic sclerosis. Positivity supports classification of lcSSc, predicts lower interstitial lung disease risk but increased pulmonary arterial hypertension risk, and informs prognostic stratification when interpreted with clinical features and other…

Biomarkers

Blood (serum)

Sample

3–5 business days

Turnaround

Fasting

What is the Centromere B Ab test?

The Centromere B Antibody test detects serum autoantibodies against centromere protein B, a structural component of the chromosome kinetochore. It is ordered principally to support the diagnosis and classification of systemic sclerosis and to distinguish limited cutaneous disease — which carries a distinct natural history and complication profile — from diffuse cutaneous and overlap variants. Anti-centromere positivity does not establish a diagnosis in isolation, but when present in a patient with Raynaud phenomenon, sclerodactyly, or telangiectasia it lends strong serological support to lcSSc. The test also informs prognosis: anti-centromere-positive patients face lower rates of interstitial lung disease but warrant closer surveillance for pulmonary arterial hypertension and digital ischaemia. Practitioners order it as part of an autoimmune panel when systemic sclerosis is clinically suspected and when differentiating among the scleroderma-spectrum disorders.

What does the Centromere B Ab test measure?

CENP-B Ab(Centromere Protein B Antibody)

Range: Negative <20 AU; equivocal 20–40 AU; positive >40 AU (assay-dependent).Optimal: Not applicable; this is a qualitative or semi-quantitative result reported as negative, equivocal, or positive.Antibody Index / Units (AU)

Centromere protein B is an 80-kDa component of the kinetochore that binds the centromeric alpha-satellite DNA at the primary constriction of mitotic chromosomes. Anti-centromere antibodies recognise a complex of centromeric proteins (CENP-A, -B, and -C), but CENP-B is the dominant target and the most commonly measured antigen in routine assays. These autoantibodies arise when immune tolerance to nuclear self-antigens breaks down, producing a characteristic punctate nuclear staining pattern on immunofluorescence that reflects the discrete localisation of centromeres within interphase nuclei.

Anti-centromere antibodies are highly specific for limited cutaneous systemic sclerosis (lcSSc), historically termed CREST syndrome (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia). They are found in approximately 50–90 per cent of lcSSc cases and far less commonly in diffuse cutaneous disease. Positivity predicts a lower risk of severe pulmonary fibrosis compared with anti-topoisomerase (Scl-70) antibodies but an increased risk of isolated pulmonary arterial hypertension. Practitioners use the result to support classification, inform prognosis, and guide surveillance for vascular complications.

Why is the Centromere B Ab test ordered?

Evaluation of Raynaud phenomenon with features suggestive of secondary rather than primary disease (digital pitting, abnormal nailfold capillaries, or systemic symptoms)
Suspected limited cutaneous systemic sclerosis in patients presenting with sclerodactyly, facial or digital telangiectasia, or oesophageal dysmotility
Classification and prognostic stratification of known or newly diagnosed systemic sclerosis, interpreted alongside anti-topoisomerase (Scl-70) and anti-RNA polymerase III antibodies
Work-up of unexplained pulmonary arterial hypertension, where a positive result may point to underlying lcSSc
Incidental antinuclear antibody (ANA) positivity with a speckled or punctate nuclear pattern on immunofluorescence, prompting reflex or follow-up testing for extractable nuclear antigens
Differentiation of systemic sclerosis from other connective-tissue diseases in overlap syndromes or when clinical features are not yet definitive
Monitoring is not routinely required, as antibody titres do not reliably track disease activity, but repeat testing may be ordered if the initial result was equivocal and clinical suspicion persists

Sample collection and turnaround

Sample type

Blood (serum)

Fasting required

Collection method

Venipuncture at patient service centre

Turnaround

3–5 business days

Collection notes

Phlebotomy via Quest at their patient service centre; no special preparation. Separate serum promptly and refrigerate if not processed immediately.

Specimen requirements

Minimum 0.5 mL serum (gel separator acceptable). Avoid haemolysis; reject grossly lipaemic or contaminated specimens. Autoantibodies are stable refrigerated for at least one week or frozen for longer-term storage.

What can affect Centromere B Ab results?

variableAssay methodology and threshold cut-offs

Anti-centromere antibodies are detected by enzyme immunoassay, immunoblot, or indirect immunofluorescence on HEp-2 cells; cut-off values and reporting formats differ between platforms, so results should be interpreted using the performing laboratory's reference interval.

variableLow-titre positivity in healthy individuals

Low-level anti-centromere reactivity is occasionally seen in asymptomatic individuals, particularly older adults, and does not by itself establish disease; clinical correlation is essential.

variableCoexisting autoantibodies

Anti-centromere antibodies typically occur in isolation, but rare overlap with other specificities (such as anti-Ro or anti-U1RNP) can complicate classification and suggests possible overlap connective-tissue disease.

elevatesPrimary biliary cholangitis

Anti-centromere antibodies are found in a subset of primary biliary cholangitis (PBC) without overt scleroderma, and their presence in PBC has been associated with portal hypertension; consider liver biochemistry if the result is positive in a patient without cutaneous features.

variableSpecimen handling and storage

Autoantibodies are generally stable in serum stored refrigerated or frozen, but prolonged room-temperature storage or repeated freeze–thaw cycles may reduce reactivity.

What do Centromere B Ab results mean?

Elevated

A positive result (titre above the laboratory threshold) is highly specific for limited cutaneous systemic sclerosis and supports that diagnosis in patients with compatible clinical features. It predicts lower risk of diffuse interstitial lung disease but increased risk of isolated pulmonary arterial hypertension and digital vascular complications. A positive result in the absence of skin or vascular findings may indicate subclinical or early lcSSc, primary biliary cholangitis with scleroderma overlap, or — rarely — asymptomatic seropositivity; clinical and biochemical correlation is required.

Normal

A negative result makes anti-centromere-associated lcSSc unlikely but does not exclude systemic sclerosis, as diffuse cutaneous disease is typically associated with anti-topoisomerase (Scl-70) or anti-RNA polymerase III antibodies instead. If clinical suspicion is high, test for those specificities and consider repeat or reflex ANA with extended autoantibody panel.

Reduced

Not applicable; the assay is qualitative or semi-quantitative, reported as negative rather than numerically low.

Note: Anti-centromere antibodies are a classification and prognostic tool, not a standalone diagnostic test. They should be ordered and interpreted in the context of Raynaud phenomenon, cutaneous findings, capillaroscopy, and pulmonary function. Equivocal results warrant repeat testing or correlation with immunofluorescence pattern. Serial titres do not reliably predict flares or response to treatment, so routine monitoring of antibody levels is not recommended.

Frequently asked questions

A positive result is highly specific for limited cutaneous systemic sclerosis and supports that diagnosis in patients with Raynaud phenomenon, sclerodactyly, or telangiectasia. It predicts lower risk of severe lung fibrosis but higher risk of pulmonary arterial hypertension and digital vascular complications.

How to access the Centromere B Ab test

The Centromere B Ab reference page is free and open to all practitioners. Create a free practitioner account to see pricing, save tests to your list, and generate requisition forms. Verification takes 1–2 business days.

Create Free Account

About this page

Published: October 7, 2026

Last reviewed: October 7, 2026

References:

Nihtyanova SI, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol. (2014)
Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum. (2005)
van den Hoogen F, et al. 2013 classification criteria for systemic sclerosis: an ACR/EULAR collaborative initiative. Arthritis Rheum. (2013)
Assassi S, et al. Clinical, immunologic, and genetic features of systemic sclerosis patients with anti-centromere antibody. Medicine (Baltimore). (2009)

This content is for licensed healthcare practitioners only and does not constitute medical advice. Clinical decisions should be based on the full clinical picture and not on any single test result.