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May 20, 2007

Anticentromere Antibodies

Patients suspected of having an autoimmune disorder, particularly a systemic connective tissue disorder, are tested for antinuclear antibodies (ANA) as a first step in diagnosis. If the ANA test is positive, the particular ANA test pattern helps determine the specific condition that might be present. When a centromere pattern is present, other tests and clinical signs must be looked at before a diagnosis can be made.

Anticentromere antibodies are a type of antinuclear antibody that can occur in a number of different autoimmune disorders. However, due to their close association with limited scleroderma, a positive test result may be misinterpreted if other tests aren’t performed. In general, because anticentromere antibodies are seen in up to 90 percent with limited systemic sclerosis, a positive result may be mistakenly viewed as an early sign of systemic sclerosis. And because anticentromere antibodies are only seen in about 10 percent of patients with diffuse scleroderma, a negative test result for anticentromere antibodies may mistakenly be interpreted as a negative result for scleroderma in general.

Predictive Value of Anticentromere Antibodies

Patients with Raynaud’s phenomena who test positive for anticentromere antibodies are more likely than patients with negative anticentromere antibody test results to develop limited scleroderma. Patients with any features of CREST syndrome are also likely to have anticentromere antibodies before all of the features emerge. To assist with diagnosis, patients suspected of having scleroderma should also be tested for anti-Scl-70 antibodies. These antibodies are seen in up to 40 percent of patients with diffuse scleroderma although they are usually not seen in limited scleroderma.

Anticentromere Antibodies in Other Conditions

Besides systemic sclerosis, anticentromere antibodies are seen in a number of different conditions including drug-induced lupus, systemic lupus erythematosus (SLE), rheumatoid arthritis, Sjogren’s syndrome, and in overlap syndromes including lupus overlap syndromes, and conditions of systemic sclerosis/Sjogren’s syndrome overlap and systemic sclerosis/primary biliary cirrhosis overlap.

Anticentromere Antibodies with elevated CPK

Creatine phosphokinase (CPK) is an enzyme that’s elevated in muscle disorders, including diseases of heart muscle. In a study of patients with anticentromere antibodies nearly 30 percent of patients with positive test results and an elevated CPK level showed no clinical signs of muscle inflammation or skin lesions. These results suggest that another systemic autoimmune condition may exist which has an undifferentiated or mild disease course.

Resources:

Miyawaki S, Asanuma H, Nishiyama S, and Yoshinaga Y, Clinical and serological heterogeneity in patients with anticentromere antibodies, Journal of Rheumatology, Aug 2005; 32(8): 148-1494.

Elaine Moore, Autoimmune Diseases and Their Environmental Triggers, Jefferson, NC: McFarland Publishing, 2002.