What is the role of autoantibody testing in the workup of scleroderma?

Updated: Jun 24, 2020
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Owing to the almost universal involvement of the immune system, most patients have specific humoral and cell-mediated immunity abnormalities. [119, 120, 121, 122, 123] The value of serology testing is for initial diagnosis and assessment of associated conditions; it is of little use for monitoring disease activity.

Antinuclear antibodies are present in about 90%-95% of affected patients, usually with a speckled or centromere pattern. A nucleolar pattern, although less common, is more specific for systemic sclerosis.

Topoisomerase I antibodies (also known as Scl-70) are present in approximately 30% of patients with diffuse disease (absent in limited disease). Patients harboring Scl-70 antibodies are at higher risk of developing pulmonary parenchymal involvement, in contrast to patients harboring anti-centromere antibodies.

Anticentromere antibodies are present in about 45%-50% of patients with limited disease. They are rare in patients with diffuse disease.

Anti-RNA polymerase I and III antibodies are present in 15%-20% of patients with diffuse disease and correlate with rapid cutaneous involvement and high frequency of renal crisis. Anti-ThRNP is present mostly in limited disease and is associated with more extensive visceral disease. Anti-PM-Scl is present in patients with overlap connective tissue disease or with mixed connective tissue disease (MCTD) and is associated with myositis and renal involvement.

Fibrillarin antibodies and antibodies to ribonucleoprotein (RNP) may be present. Anti-RNP is present mostly in patients with diffuse disease with overlap syndromes and in patients with MCTD. These antibodies are more common in patients with skeletal muscle involvement and pulmonary disease.

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