Significance of Elevated Centromere Protein B Antibody
Elevated anti-centromere protein B (CENP-B) antibody is most strongly associated with limited systemic sclerosis (formerly CREST syndrome) and indicates a high risk for developing Raynaud's phenomenon and other features of limited scleroderma. 1, 2
Clinical Significance and Disease Associations
- Anti-CENP-B antibodies are a specific type of anti-nuclear antibody (ANA) that target centromere protein B, which is an alphoid DNA binding protein 3
- These antibodies are primarily associated with limited cutaneous systemic sclerosis (lcSSc), formerly known as CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) 2, 4
- The presence of anti-CENP-B antibodies is significantly more common in limited systemic sclerosis (48%) compared to diffuse systemic sclerosis (9-11%) 5
- Anti-CENP-B antibodies have high disease specificity (95-96.5%) for systemic sclerosis when compared to other autoimmune conditions 6, 5
Diagnostic Value
- Anti-CENP-B antibody testing has approximately 98% sensitivity and 95% specificity for detecting anticentromere antibodies in patients at risk for systemic sclerosis 6
- These antibodies serve as a useful prognostic indicator in early scleroderma, helping to predict the pattern of disease that will develop 2
- Anti-CENP-B antibodies are rarely found in patients with rapidly advancing or diffuse scleroderma, making their presence a marker for a more limited disease course 2
- The antibody levels correlate with the presence and severity of Raynaud's phenomenon, suggesting a potential pathogenic role 3
Clinical Overlap Syndromes
- Anti-CENP-B antibodies can identify patients with Sjögren's syndrome who have autoimmune overlap syndromes 4
- Patients with Sjögren's syndrome who are positive for anti-CENP-B show greater frequency of:
- Raynaud's phenomenon
- Objective xerophthalmia (dry eyes)
- Peripheral neuropathy
- Additional autoimmune disorders, especially primary biliary cirrhosis 4
- These patients often have fewer traditional Sjögren's antibodies (anti-SSA/SSB) but a greater prevalence of other autoantibodies 4
Laboratory Testing Considerations
- Anti-CENP-B antibodies are typically detected as part of an ANA panel with a characteristic centromere pattern on immunofluorescence 1
- ELISA tests for CENP-B yield results with similar sensitivity and specificity as immunofluorescence for the diagnosis of systemic sclerosis 5
- The optimal screening dilution for ANA testing should be defined locally, with 1:160 often being the most suitable for adult patients 1
- In patients with systemic sclerosis, the autoantibody response is typically directed simultaneously against both CENP-A and CENP-B components of the centromere structure 5
Clinical Management Implications
- Screening for anti-CENP-B antibodies should be conducted in all patients with Raynaud's phenomenon, primary biliary cirrhosis, and scleroderma 2
- The presence of these antibodies in a patient with early symptoms should prompt close monitoring for the development of limited systemic sclerosis 2, 4
- Patients with positive anti-CENP-B antibodies should be evaluated for associated conditions, particularly primary biliary cirrhosis and other autoimmune disorders 4
- Regular assessment for progression of symptoms related to limited systemic sclerosis is warranted in antibody-positive individuals 3, 2
Understanding the significance of elevated anti-CENP-B antibodies allows for early identification of patients at risk for limited systemic sclerosis and associated overlap syndromes, enabling appropriate monitoring and management strategies.