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Table 2 Rare autoantibodies in systemic sclerosis and reported clinical associations

From: Personalized medicine in rheumatology: the paradigm of serum autoantibodies

Autoantibody Prevalence (%) Clinical associations References
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 3 ILD [94]
  1. dcSSc diffuse systemic sclerosis, lcSSc limited systemic sclerosis, PAH pulmonary arterial hypertension, PBC primary biliary cholangitis, ILD interstitial lung disease