Table 2 Rare autoantibodies in systemic sclerosis and reported clinical associations

Anti-RNAP

6–31

dcSSc, renal crisis, malignancies

[85, 86]

Anti-Scl70

10–40

dcSSc, ILD, digital ulcers, cardiac involvement, malignancies

[87, 88]

Anti-U3RNP

5–8

dcSSc, cardiomyopathy, myopathy, PAH, ILD, severe small bowel involvement

[89]

Anti-U1RNP

4–14

lcSSc, PAH, overlap syndrome

[90]

Anti-Ku

1–3

Muscle and joint involvement

[91]

Anti-PM-Scl

2–10

myositis, arthritis, lung or kidney involvement and mechanic’s hands

[92]

Anti-Th/To

1–10

lcSSc, PAH, ILD, puffy fingers, small bowel disease, hypothyroidism

[93]

Anti-U11/U12RNP

ILD

[94]