Centromere B Antibody Positive: Clinical Significance
A positive centromere B antibody test primarily indicates limited cutaneous systemic sclerosis (lcSSc), occurring in 50-80% of these cases, and warrants immediate evaluation for scleroderma spectrum disorders. 1
Primary Disease Association
- Limited cutaneous systemic sclerosis (lcSSc) is the predominant diagnosis, with anticentromere antibodies (ACA) detected in 50-80% of cases and generally indicating a better prognosis compared to other scleroderma subtypes 1
- The antibody specifically targets centromere protein B (CENP-B), an alphoid DNA binding protein, and represents a pathogenic autoimmune response 2
Essential Clinical Evaluation
When anticentromere B antibody is detected, perform a systematic assessment for:
Scleroderma Spectrum Features
- Raynaud's phenomenon (strongly associated with positive ACA) 2, 3
- Skin thickening and sclerodactyly 1
- Digital ulcers and telangiectasias 1
- Esophageal dysmotility 1
Pulmonary Complications
- Screen for pulmonary arterial hypertension (PAH) using echocardiography, pulmonary function testing, electrocardiography, NT-proBNP, and 6-minute walking distance, as PAH represents a serious late complication in ACA-positive patients 1
- Evaluate for interstitial lung disease through chest radiography, pulmonary function testing, and high-resolution CT when clinically indicated 1
Hepatobiliary Disease
- Primary biliary cholangitis occurs in 8% of lcSSc cases, usually in those with anticentromere antibodies 1
- Check liver function tests, particularly alkaline phosphatase 1
- High titers of anti-CENP-B antibody (>400-800) show close relationship with primary biliary cholangitis 4
Cardiovascular Monitoring
- Monitor for scleroderma renal crisis with regular blood pressure checks and home monitoring 1
- Assess cardiovascular risk factors including hypertension, diabetes, hyperlipidemia, and smoking 1
Alternative Diagnoses to Consider
While lcSSc is the primary association, anticentromere antibodies can occur in other conditions:
- Systemic lupus erythematosus (SLE): Rare (1.9% prevalence), but genuine SLE can occur without concurrent scleroderma features 5
- Primary Sjögren's syndrome: ACA-positive pSS represents a distinct clinical subset, particularly when antibodies react with CENP-C and HP1α 6
- Seronegative polyarthritis and other rheumatic diseases with active digital vasculitis 3
Important caveat: The presence of anticentromere antibody without scleroderma or CREST often indicates another serious underlying rheumatic or connective tissue disease 3
Additional Autoimmune Testing
- Perform extractable nuclear antibody panel (anti-Smith, anti-SSA/Ro, anti-SSB/La, anti-RNP, anti-Jo-1) to identify potential overlap syndromes 1
- Consider anti-mitochondrial antibody testing given the association with primary biliary cholangitis 4
Management Priorities
- Treat gastrointestinal manifestations with proton pump inhibitors and prokinetic agents for esophageal dysmotility 1
- Screen for and manage nutritional deficiencies from malabsorption 1
- Perform bone density scanning for osteoporosis, which is increased in systemic sclerosis 1
- Screen for depression, elevated in patients with chronic diseases including systemic sclerosis 1