From the Guidelines
Patients with anticentromere B positivity typically have a better prognosis than those with other forms of scleroderma, with less severe internal organ involvement, particularly the lungs, as indicated by the presence of anti-centromere antibodies being considered protective and decreasing the likelihood of ILD development in SSc 1.
Key Considerations
- Anticentromere B antibody positivity is a specific marker for limited cutaneous systemic sclerosis (formerly called CREST syndrome), a form of scleroderma.
- The presence of anti-centromere antibodies is associated with a lower risk of interstitial lung disease (ILD) development in systemic sclerosis (SSc) patients, as reported in a study published in Pulmonology in 2025 1.
- Management of patients with anticentromere B positivity focuses on treating specific symptoms, such as calcium channel blockers for Raynaud's phenomenon, proton pump inhibitors for esophageal issues, and immunosuppressive medications for more severe manifestations.
- Regular monitoring for pulmonary hypertension and other complications is essential, as patients with anticentromere B positivity have a higher risk of developing pulmonary hypertension over time.
Screening and Monitoring
- Screening for organ involvement in SSc should be performed according to the guidelines outlined in the Nature Reviews Rheumatology study published in 2023, which includes screening for pulmonary arterial hypertension, ILD, scleroderma renal crisis, and other organ involvement 1.
- Patients with anticentromere B positivity should be screened for primary biliary cholangitis, as it occurs in 8% of cases of limited cutaneous SSc, usually in those positive for anti-centromere antibodies.
- Regular monitoring for cardiovascular risk factors, such as hypertension, diabetes mellitus, hyperlipidaemia, and metabolic syndrome, is also recommended.
Treatment and Prognosis
- The treatment of patients with anticentromere B positivity should focus on managing specific symptoms and preventing complications.
- The prognosis for patients with anticentromere B positivity is generally better than for those with other forms of scleroderma, with less severe internal organ involvement, particularly the lungs.
- However, patients with anticentromere B positivity have a higher risk of developing pulmonary hypertension over time, which requires regular monitoring and management.
From the Research
Anticentromere Antibody Positive
- Anticentromere antibody is associated with limited cutaneous systemic sclerosis, as seen in a case study where a 68-year-old Japanese woman with limited cutaneous systemic sclerosis developed scleroderma renal crisis 2.
- The presence of anticentromere antibody is generally considered a serologic marker of the CREST syndrome, a more benign subset of systemic sclerosis 3, 4.
- Anticentromere antibody can also be found in patients with localized scleroderma, suggesting a relationship between localized scleroderma and systemic sclerosis 3.
- The antibody is primarily found in patients with scleroderma, CREST syndrome, and Raynaud's phenomenon, and is a useful prognostic indicator in patients with early scleroderma 5, 6.
- The presence of anticentromere antibody is rare in patients with rapidly advancing or diffuse scleroderma, and is not typically found in patients with other rheumatic diseases such as rheumatoid arthritis or systemic lupus erythematosus 5, 6, 4.
Clinical Associations
- Anticentromere antibody is often associated with less severe disease, whereas anti-topoisomerase I antibody is associated with skin and cardiac involvement and malignancies 5.
- The antibody is commonly found in patients with primary biliary cirrhosis and scleroderma, and is a useful marker for predicting the pattern of scleroderma that will evolve 6.
- Screening for anticentromere antibody should be conducted in all patients with Raynaud's phenomenon, primary biliary cirrhosis, and scleroderma 6.