Table 1

Well-described and novel antigens associated with systemic sclerosis autoantibodies and associated clinical features9

AntigenClinical associations
CentromereLimited SSc, CREST phenotype, pHTN, protection from ILD
Topoisomerase-1 (Scl-70)Diffuse SSc, ILD, early organ involvement
RNA-polymerase IIIRenal, cutaneous, malignancy, increased mortality
Th/TopHTN, ILD, gastrointestinal
PM-SclMyositis overlap syndromes
U3-RNP (fibrillarin)pHTN, myositis, younger onset, cardiac involvement
U1-RNPMyositis, mixed connective tissue disease
KuMyositis and joint involvement, SLE overlap
U11/U12-RNPILD
Eukaryotic initiation factor 2BDiffuse SSc, ILD
RNA-binding region-containing protein 3Malignancy, ILD, gastrointestinal, myopathy
RuvBL1 and RuvsBL2Diffuse SSc, myositis overlap
Bicaudal D homolog 2Myositis, ILD
Interferon-inducible protein 16Digital ischaemia
Angiotensin II type 1 receptorDigital ischaemia, pHTN
Endothelin-1 type A receptorDigital ischaemia, pHTN
Muscarinic-3 receptorGastrointestinal
Platelet-derived growth factor receptorPossibly profibrotic

  • ILD, interstitial lung disease; pHTN, pulmonary hypertension; SLE, systemic lupus erythematosus; SSc, systemic sclerosis.