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Evaluating patients with signs and symptoms of scleroderma and
other connective tissue diseases in whom the test for antinuclear
antibodies (ANA) is positive
Scl 70 (topoisomerase 1) is a 100-kD nuclear and nucleolar enzyme.
Scl 70 antibodies are considered to be specific for scleroderma
(systemic sclerosis) and are found in up to 60% of patients with this
connective tissue disease. Scl 70 antibodies are more common in
patients with extensive cutaneous involvement and interstitial
pulmonary fibrosis, and are considered a poor prognostic sign.(1,2)
See "Connective Tissue Diseases Cascade Test-Ordering Algorithm"
in Special Instructions and "Optimized Laboratory Testing for
Connective Tissue Diseases in Primary Care: The Mayo Connective
Tissue Diseases Cascade" in Publications.
<1.0 U (negative)
> or =1.0 U (positive)
Reference values apply to all ages.
A positive test result for Scl 70 antibodies is consistent with a
diagnosis of scleroderma.
Testing for Scl 70 antibodies is not useful in patients without
demonstrable ANA.
1. Homburger H, Larsen S: Detection of specific antibodies. In
Clinical Immunology: Principles and Practice. 1st edition. Edited
by R Rich, T Fleisher, B Schwartz, et al. St. Louis, Mosby-Year
Book, Inc, 1996, pp 2096-2109
2. White B: Systemic sclerosis. In Clinical Immunology Principles
and Practice. 2nd edition. Edited by R Rich, T Fleisher, W Shearer,
et al. St. Louis, Mosby-Year Book, 2001, pp 64.1-64.10