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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