Table 2

Disease manifestations by antibody status

ANA (N=2085)Anti-Scl-70 (N=854)Anti-RNA Pol 3 (N=86)ACA (N=775)Anti-U1 RNP (N=120)Anti-U3 RNP (N=39)
Lung fibrosis, n/N (%) (95% CI)873/2085 (41.9) (39.7 to 44.0)531/854 (62.2) (58.8 to 65.4)36/86 (41.9) (31.3 to 53.0)173/775 (22.3) (19.4 to 25.4)50/120 (41.7) (32.7 to 51.0)16/39 (41.0) (25.6 to 57.9)
PAH, n/N (%) (95% CI)307/2085 (14.7) (13.2 to 16.3)133/854 (15.6) (13.2 to 18.2)12/86 (14.0) (7.4 to 23.1)120/775 (15.5) (13.0 to 18.2)19/120 (15.8) (9.8 to 23.6)9/39 (23.1) (11.1 to 39.3)
Kidney manifestations, n/N (%) (95% CI)105/2085 (5.0) (4.1 to 6.1)43/854 (5.0) (3.7 to 6.7)7/86 (8.1) (3.3 to 16.1)26/775 (3.4) (2.2 to 4.9)10/120 (8.3) (4.1 to 14.8)5/39 (12.8) (4.3 to 27.4)
Gastrointestinal manifestations, n/N (%) (95% CI)1192/2085 (57.2) (55.0 to 59.3)498/854 (58.3) (56.4 to 63.9)51/86 (59.3) (47.9 to 70.8)440/775 (56.8) (53.2 to 60.3)63/120 (52.5) (43.2 to 61.7)15/39 (38.5) (23.4 to 55.4)
Heart manifestations, n/N (%) (95% CI)220/2085 (10.6) (9.3 to 12.0)105/854 (12.3) (10.2 to 14.7)6/86 (7.0) (2.6 to 14.6)47/775 (6.1) (4.5 to 8.0)16/120 (13.3) (7.8 to 20.7)3/39 (7.7) (1.6 to 20.9)
  • The denominator N varies according to the number of available observations.

  • * Antibody subsets are not mutually exclusive: one patient can have several antibodies and therefore contribute to several groups.

  • ACA, anticentromere antibodies; ANA, antinuclear antibodies; anti-Scl-70, anti-scleroderma-70 antibodies; anti-RNA Pol 3, anti-RNA polymerase 3; anti-U1 RNP, anti-U1 ribonucleoprotein; anti-U3 RNP, anti-U3 ribonucleoprotein; PAH, pulmonary arterial hypertension.