From the Guidelines
Patients with anticentromere B antibody positivity should be closely monitored for pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH), as they are at increased risk, particularly if they have limited cutaneous systemic sclerosis (lcSSc) 1. The presence of anticentromere B antibodies is associated with a higher risk of developing PAH, which is a significant cause of morbidity and mortality in patients with systemic sclerosis (SSc) 1. According to the state-of-the-art evidence in the treatment of systemic sclerosis, PAH occurs in approximately 8-15% of patients with SSc, with SSc-associated PAH (SSc-PAH) being the most common aetiology of PH in SSc 1. Key factors associated with an increased risk of SSc-PAH include older age, longer disease duration, positivity for anti-centromere antibodies, elevated erythrocyte sedimentation rate, and digital ulcers 1. Given the poor 5-year survival rate after diagnosis of PAH, early detection and management are crucial 1. Echocardiography is recommended as the first-line screening test for SSc-associated PH, and a very low DLCO (<46% with parenchymal lung disease and <73% without parenchymal lung disease) and decreasing DLCO are suggestive of PAH 1. Regular monitoring for potential complications affecting the lungs, heart, and kidneys is essential, as early intervention can significantly improve outcomes in this chronic autoimmune condition. Some of the key symptoms to monitor include Raynaud's phenomenon, skin thickening on fingers, acid reflux, and swallowing difficulties. Management focuses on symptom control with medications like calcium channel blockers for Raynaud's, proton pump inhibitors for reflux, and immunosuppressants if organ involvement occurs. It is essential to consult with a rheumatologist for a comprehensive evaluation and to discuss the best course of management and monitoring.
From the Research
Anticentromere B Positive
- Anticentromere B antibodies are associated with limited cutaneous involvement and lower frequency of pulmonary fibrosis and lower mortality in patients with systemic sclerosis, despite an increased risk for pulmonary hypertension 2.
- A case study reported a patient with limited cutaneous systemic sclerosis who developed scleroderma renal crisis, with anticentromere antibody being the only autoantibody detected 3.
- Anticentromere-A and anticentromere-B antibodies show high concordance and similar clinical associations in patients with systemic sclerosis, with both antibodies being associated with limited disease and rather mild skin sclerosis 4.
- Patients with systemic sclerosis who carry anti-centromere autoantibodies often have pulmonary function test abnormalities, even in the absence of clinical evidence of pulmonary disease 5.
- Anticentromere antibodies are associated with specific demographic, clinical, and organ system features, including digital tip ulcers and digital tuft resorption, and are seen more commonly in older, female Caucasians 6.