Anticentromere Antibodies in Multiple Sclerosis

Clinical Significance

Anticentromere antibodies are primarily associated with limited cutaneous systemic sclerosis (lcSSc), present in 50-80% of these cases, and generally indicate a better prognosis compared to other scleroderma subtypes 1.
The presence of ACA in a patient with suspected MS should be considered a "red flag" that warrants investigation for alternative diagnoses 2, 4.
MS diagnosis relies on specific MRI findings and clinical presentation, not on ACA or other autoantibodies 2, 3.

ACA positivity strongly suggests limited cutaneous systemic sclerosis (lcSSc), especially when accompanied by Raynaud's phenomenon 5, 1.
Patients with ACA should be evaluated for other features of scleroderma spectrum disorders, including skin thickening, pulmonary arterial hypertension (PAH), and digital ulcers or telangiectasias 5, 6.
Primary biliary cholangitis is associated with ACA and occurs in 8% of lcSSc cases 5.

Re-evaluate MS diagnosis by reviewing MRI findings for typical MS lesions and checking CSF for oligoclonal bands 2, 3, 7, 8.
Screen for scleroderma and other connective tissue diseases by assessing for Raynaud's phenomenon, skin thickening, and telangiectasias, and consider pulmonary function testing and echocardiography to evaluate for PAH 5, 6.
Consider MOG-IgG testing, as MOG antibody-associated disease can mimic MS but has distinct treatment implications 4, 7, 9.

If ACA is positive and features of scleroderma are present, referral to rheumatology is warranted for appropriate management 5.
ACA-positive patients should be monitored for development of pulmonary arterial hypertension, which requires specific treatment approaches 6, 5.
MS-specific disease-modifying therapies may not be appropriate if the diagnosis is revised to another condition based on ACA positivity and clinical features 7, 4.