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Table 3 Antinuclear antibodies associated with systemic sclerosis (SSc)

From: French recommendations for the management of systemic sclerosis

Antinuclear antibody specificities Appearance of fluorescence % of patients Form of the disease Clinical phenotype
Anticentromere antibodies Centromeric 20–40% Limited cutaneous SSc PAH
Severe peripheral vascular disease
Anti-Scl-70 or anti-topoisomerase antibodies Homogeneous and nucleolar 20–30% Diffuse cutaneous SSc Severe ILD
Severe peripheral vascular disease
Anti-RNA polymerase III Speckled
(±nucleolar)
20–25% in Caucasians of North America and the UK and about 5% in France, Germany, Italy, and Japan Diffuse cutaneous SSc Rapidly progressive skin damage
Scleroderma renal crisis
Cancer
Anti-U3-RNP (antifibrillarin) Nucleolar In 4–10% Diffuse cutaneous SSc
 > 
Limited cutaneous SSc
ILD
PAH
Renal crisis
Digestive damage of small intestine
Anti-Th/To Nucleolar 2–5% Limited cutaneous SSc ILD
PAH
Anti-Pm-Scl Speckled and nucleolar 2% Limited cutaneous SSc (overlap with myositis) Myositis
Anti-U1 RNP Speckled   Limited cutaneous SSc (overlap with MCTD) Arthritis
Myositis
PAH
Anti-U11/U12 RNP Speckled 1–3% Diffuse cutaneous SSc/limited cutaneous SSc Severe ILD
Anti-Ku Speckled   Limited cutaneous SSc (overlap syndrome with myositis) Myositis
Anti-RuvBL 1/2 Speckled 1–3% Diffuse cutaneous SSc (overlap myositis) Myositis
  1. Ab antibody, PAH pulmonary arterial hypertension, ILD interstitial lung disease, RNA ribonucleic acid, RNP ribonucleoprotein, MCTD mixed connective tissue disease