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. Author manuscript; available in PMC: 2017 Jan 1.
Published in final edited form as: Expert Opin Orphan Drugs. 2015 Nov 23;4(1):31–47. doi: 10.1517/21678707.2016.1114454

Table 1.

Association of auto-antibodies with different SSc clinical phenotypes

Antibody Type Prevalence % Disease subtype Clinical Phenotype
Anti-centromere 24–45 IcSSc CREST Syndrome
Pulmonary arterial hypertension
Anti-Scl-70 15–42 dcSSc Rapidly Progressive SSc-ILD
Cardiac Involvement
Anti-RNA polymerase III 5–31 dcSSc Scleroderma Renal Crisis
Tendon friction rubs, Joint contractures
Myositis
Anti-U3RNP (Fibrillarin) 2–16 dcSSc Scleroderma Renal Crisis
Cardiac Involvement
Anti-PM-Scl 4–11 IcSSc Myositis, arthritis, calcinosis
Sicca syndrome
SSc-ILD
Anti- Th/To 1–18 IcSSc SSc-ILD
Scleroderma Renal Crisis
Gastrointestinal Involvement
Anti-U11/U12 RNP 3.2 - SSc-ILD
Anti-U1-RNP 2–14 IcSSc MCTD features
Anti-Ku 2 dcSSc Myositis, arthritis, joint contractures
Anti-NOR 90 0.8–6 dSSc SSc-ILD
Anti-Ro52 20–27 - SSc-ILD

lcSSc: Limited cutaneous SSc.

dcSSc: Diffuse cutaneous SSc.

SSc-ILD: SSc-associated Interstitial Lung Disease.

MCTD: Mixed connective tissue disease.