Associations between AID and systemic sclerosis features.
| AID | Disease Phenotype Associations in Univariate Analysis | Patients with AID, n (%) vs Patients without AID, n (%) | p* | Multivariate Stepwise Logistic Regression Analysis OR (95% CI) | p** |
|---|---|---|---|---|---|
| Sjögren’s syndrome, (n = 137) | Female, n (%) | 127/137 (92) vs 862/995 (87) | 0.07 | NS | |
| Age, mean ± SD, yrs | 65 ± 15 vs 62 ± 13 | 0.5 | NI | ||
| Disease duration, yrs | 14 ± 10 vs 12 ± 8 | 0.02 | NS | ||
| Limited cutaneous subtype, n (%) | 115/137 (84) vs 690/995 (69) | 0.002 | 2.7 (1.4–5.3) | 0.003 | |
| History of digital ulcers, n (%) | 40/137 (29) vs 377/995 (38) | 0.06 | NS | ||
| PAH, n (%) | 9/137 (7) vs 110/995 (11) | 0.04 | NS | ||
| Pulmonary fibrosis on CT scan, n (%) | 38/137 (27) vs 357/995 (36) | 0.07 | NS | ||
| ANA (> 1/160), n (%) | 134/137 (98) vs 912/995 (92) | 0.008 | NS | ||
| Anti-topo, n (%) | 21/137 (15) vs 254/995 (26) | 0.02 | NS | ||
| ACA, n (%) | 79/137 (58) vs 390/995 (39) | 0.001 | NS | ||
| Decreased forced vital capacity, n (%) | 10/137 (7) vs 156/995 (16) | 0.001 | NS | ||
| Decreased DLCO/AV, n (%) | 64/137 (47) vs 409/995 (41) | 0.6 | NI | ||
| Immunosuppressive drug, n (%) | 33/137 (24) vs 239/995 (24) | 0.9 | NI | ||
| Autoimmune thyroiditis, (n = 70) | Female, n (%) | 68/70 (97) vs 921/1062 (87) | 0.04 | NS | |
| Age, mean ± SD, yrs | 62 ± 13 vs 62 ± 13 | 0.9 | NI | ||
| Disease duration, yrs | 11 ± 9 vs 12 ± 9 | 0.5 | NI | ||
| Limited cutaneous subtype, n (%) | 55/70 (78) vs 750/1062 (71) | 0.2 | NI | ||
| History of digital ulcers, n (%) | 15/70 (22) vs 402/1062 (38) | 0.03 | NS | ||
| PAH, n (%) | 7/70 (10) vs 112/1062 (10) | 0.25 | NI | ||
| Pulmonary fibrosis on CT scan, n (%) | 13/70 (18) vs 382/1062 (36) | 0.001 | 0.3 (0.1–0.7) | 0.004 | |
| ANA (> 1/160), n (%) | 66/70 (94) vs 980/1062 (92) | 0.06 | NS | ||
| Anti-topo, n (%) | 14/70 (20) vs 271/1062 (25) | 0.4 | NI | ||
| ACA, n (%) | 41/70 (58) vs 428/1062 (40) | 0.02 | NS | ||
| Decreased forced vital capacity, n (%) | 8/70 (11) vs 158/1062 (15) | 0.3 | NI | ||
| Decreased DLCO/AV, n (%) | 27/70 (39) vs 446/1062 (42) | 0.3 | NI | ||
| Immunosuppressive drug, n (%) | 7/70 (10) vs 265/1062 (25) | 0.03 | NS | ||
| Myositis, (n = 35) | Female, n (%) | 31/35 (88) vs 958/1097 (87) | 0.9 | NI | |
| Age, mean ± SD, yrs | 64 ± 16 vs 62 ± 13 | 0.8 | NI | ||
| Disease duration, yrs | 11 ± 7 vs 12 ± 8 | 0.4 | NI | ||
| Limited cutaneous subtype, n (%) | 25/35 (71) vs 780/1097 (71) | 0.9 | NI | ||
| History of digital ulcers, n (%) | 11/35 (31) vs 406/1097 (37) | 0.6 | NI | ||
| PAH, n (%) | 3/35 (10) vs 116/1097 (11) | 0.6 | NI | ||
| Pulmonary fibrosis on CT scan, n (%) | 17/35 (49) vs 378/1097 (35) | 0.09 | NS | ||
| ANA (> 1/160), n (%) | 32/35 (91) vs 1014/1097 (92) | 0.8 | NI | ||
| Anti-topo, n (%) | 9/35 (26) vs 276/1097 (25) | 0.9 | NI | ||
| ACA, n (%) | 5/35 (14) vs 464/1097 (42) | 0.002 | NS | ||
| Decreased forced vital capacity, n (%) | 6/35 (17) vs 160/1097 (14) | 0.9 | NI | ||
| Decreased DLCO/AV, n (%) | 15/35 (43) vs 458/1097 (42) | 0.9 | NI | ||
| Immunosuppressive drug, n (%) | 27/35 (77) vs 245/1097 (22) | < 0.0001 | 11.5 (5.1–25.7) | < 0.0001 | |
| Primary biliary cirrhosis, (n = 31) | Female, n (%) | 28/31 (91) vs 961/1101 (87) | 0.7 | NI | |
| Age, mean ± SD, yrs | 65 ± 13 vs 62 ± 12 | 0.7 | NI | ||
| Disease duration, yrs | 16 ± 10 vs 12 ± 9 | 0.008 | NS | ||
| Limited cutaneous subtype, n (%) | 30/31 (97) vs 775/1101 (70) | 0.002 | 2.1 (1.5–2.8) | 0.02 | |
| History of digital ulcers, n (%) | 6/31 (21) vs 411/1101 (37) | 0.06 | NS | ||
| PAH, n (%) | 2/31 (7) vs 117/1101 (11) | 0.8 | NI | ||
| Pulmonary fibrosis on CT scan, n (%) | 5/31 (16) vs 390/1101 (35) | 0.04 | NS | ||
| ANA (> 1/160), n (%) | 28/31 (91) vs 1014/1101 (92) | 0.9 | NI | ||
| Anti-topo, n (%) | 1/31 (3) vs 284/1101 (26) | 0.008 | NS | ||
| ACA, n (%) | 23/31 (74) vs 446/1101 (40) | 0.0003 | 6.2 (2.3–16.5) | 0.003 | |
| Decreased forced vital capacity, n (%) | 1/31 (3) vs 165/1101 (15) | 0.08 | NS | ||
| Decreased DLCO/AV, n (%) | 12/31 (39) vs 461/1101 (42) | 0.2 | NI | ||
| Immunosuppressive drug, n (%) | 9/31 (29) vs 263/1101 (24) | 0.6 | NI | ||
| At least 1 AID, (n = 239) | Female, n (%) | 220/239 (92) vs 769/893 (86) | 0.02 | NS | |
| Age, mean ± SD, yrs | 63 ± 15 vs 59 ± 13 | 0.01 | NS | ||
| Disease duration, yrs | 13 ± 10 vs 12 ± 10 | 0.1 | NI | ||
| Limited cutaneous subtype, n (%) | 194/239 (81) vs 611/893 (68) | < 0.0001 | 3.3 (2.1–5.3) | < 0.0001 | |
| History of digital ulcers, n (%) | 64/239 (27) vs 353/893 (40) | 0.0007 | 0.6 (0.4–0.9) | 0.02 | |
| PAH, n (%) | 19/239 (8) vs 100/893 (11) | 0.3 | NI | ||
| Pulmonary fibrosis on CT scan, n (%) | 64/239 (27) vs 331/893 (37) | 0.006 | NS | ||
| ANA (> 1/160), n (%) | 229/239 (96) vs 817/893 (91) | 0.002 | 8.6 (2.1–35.9) | 0.004 | |
| Anti-topo, n (%) | 43/239 (18) vs 242/893 (27) | 0.003 | NS | ||
| ACA, n (%) | 117/239 (49) vs 372/893 (41) | 0.002 | NS | ||
| Decreased forced vital capacity, n (%) | 26/239 (11) vs 140/893 (16) | 0.006 | NS | ||
| Decreased DLCO/AV, n (%) | 105/239 (44) vs 367/893 (41) | 0.09 | NS | ||
| Immunosuppressive drug, n (%) | 26/239 (30) vs 246/893 (27) | 0.07 | NS |
AID: autoimmune diseases; PAH: pulmonary arterial hypertension; ANA: antinuclear antibodies; Anti-topo: antitopoIsomerase I antibodies; ACA: anticentromere antibodies; CT: computed tomography; DLCO/AV: carbon monoxide diffusion capacity divided by alveolar volume; NS: not significant. NI: not included.
↵* Significant if p < 0.004.
↵** Significant if p < 0.05.