From the Guidelines
Elevated Centromere Protein B (CENP-B) antibody levels are significant as they are associated with a decreased likelihood of developing interstitial lung disease (ILD) in systemic sclerosis (SSc) patients, which is a major cause of morbidity and mortality in this population. The presence of anti-centromere antibodies, including CENP-B, is considered protective against ILD development in SSc, as reported in a 2025 study published in the journal Pulmonology 1. This is crucial because ILD is a prevalent pulmonary manifestation of SSc, detected in approximately 50% of patients, and is the leading cause of hospitalization, morbidity, and mortality.
Key points to consider regarding elevated CENP-B antibody levels include:
- They are primarily associated with limited cutaneous systemic sclerosis (lcSSc), also known as CREST syndrome
- These antibodies serve as important diagnostic markers for lcSSc, which typically presents with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia
- Patients with elevated CENP-B antibodies generally have a better prognosis than those with other forms of systemic sclerosis, particularly in terms of ILD risk
- The presence of these antibodies is relatively specific, occurring in approximately 70-90% of patients with limited cutaneous systemic sclerosis, while rarely appearing in other autoimmune conditions, as suggested by the context of the study 1
Testing for CENP-B antibodies is typically done as part of an autoimmune panel when systemic sclerosis is suspected, and positive results should prompt referral to a rheumatologist for comprehensive evaluation and management of the condition. Given the protective effect of anti-centromere antibodies against ILD, the detection of elevated CENP-B antibody levels can significantly influence the prognosis and management plan for patients with systemic sclerosis, emphasizing the importance of early detection and appropriate referral for specialized care 1.
From the Research
Elevated Centromere Protein B Antibody Significance
The significance of an elevated Centromere Protein B (CENP-B) antibody level is closely related to its association with systemic sclerosis (SSc), particularly the limited form of the disease.
- Elevated CENP-B antibody levels are often found in patients with limited systemic sclerosis, characterized by limited skin involvement and a lower risk of pulmonary complications 2.
- The presence of anti-CENP-B antibodies is highly concordant with anti-CENP-A antibodies, suggesting that testing for both may slightly increase the diagnostic sensitivity for SSc 2.
- Patients with elevated CENP-B antibody levels tend to have milder skin sclerosis and are less likely to develop pulmonary interstitial fibrosis or restrictive lung disease 3, 4.
- The antibody is also associated with a higher frequency of telangiectasiae of the digits and calcinosis in patients with the CREST syndrome variant of limited systemic sclerosis 3.
- In some cases, elevated CENP-B antibody levels may be found in patients without SSc or CREST, but with other rheumatic or connective tissue diseases, such as systemic lupus erythematosus or seronegative polyarthritis 5.
Clinical Associations
The clinical associations of elevated CENP-B antibody levels include:
- Limited skin involvement and lower risk of pulmonary complications in SSc patients 6, 2
- Higher frequency of telangiectasiae of the digits and calcinosis in CREST syndrome patients 3
- Association with active digital vasculitis, rheumatic, and connective tissue disease in patients without SSc or CREST 5
- Similar clinical manifestations and disease associations as anti-CENP-A antibodies, despite different amino acid sequences and lack of cross-reactivity 2