Table 1 Characteristics of the patients with systemic sclerosis-associated interstitial lung disease included in the study
Total population N = 181 | Extensive ILD N = 53 | Limited ILD N = 128 | |
|---|---|---|---|
Female, n (%) | 144 (79.6) | 40 (75.5) | 104 (81.3) |
Smoking status | |||
Never smoker, n (%) | 113/172 (65.7) | 38/51 (74.5) | 75/121 (62.0) |
Former smoker, n (%) | 16/172 (9.3) | 2/51 (3.9) | 14/121 (11.6) |
Current smoker, n (%) | 43/172 (25.0) | 11/51 (21.6) | 32/121 (26.4) |
Age at SSc diagnosis (years), median (IQR) | 53 (42.5–64) | 54 (44.5–64) | 53 (42–64.8) |
Cutaneous phenotype | |||
Diffuse cutaneous, n (%) | 80 (44.2) | 26 (49.1) | 54 (42.2) |
Limited cutaneous, n (%) | 89 (46.4) | 25 (47.2) | 64 (50.0) |
Sine scleroderma, n (%) | 12 (6.6) | 2 (3.8) | 10 (7.8) |
ScS involvement | |||
Gastrointestinal, n (%) | 139 (76.8) | 42 (79.2) | 97 (75.8) |
Cardiac, n (%) | 19 (10.5) | 7 (13.2) | 12 (9.4) |
Muscular, n (%) | 14 (7.7) | 3 (5.7) | 11 (8.6) |
Renal, n (%) | 5 (2.8) | 1 (1.9) | 4 (3.1) |
Autoantibodies | |||
Anti-centromere, n (%) | 20 (11.0) | 1 (1.9) | 19 (14.8) |
Anti-topoisomerase I, n (%) | 101 (55.8) | 37 (69.8) | 64 (50.0) |
Anti-RNA polymerase III, n (%) | 11 (6.1) | 4 (7.5) | 7 (5.5) |
Time from first non-Raynaud symptom (years) to ILD diagnosis, median (IQR) | 2 (1–5) | 2 (1–4) | 2 (1–7) |
Pulmonary function | |||
FVC (% predicted), median (IQR) | 78.5 (63.7–93.8) | 61.7 (50.5–69.8) | 84.1 (74.5–98.6) |
TLC (% predicted), median (IQR) | 83.4 (71.8–99.2) | 70.6 (61.9–76.0) | 92.4 (78.5–102.2) |
FEV1 (% predicted), median (IQR) | 81.1 (67.5–94.9) | 67.9 (57.4–74.3) | 86.1 (73.0–97.6) |
DLCO (% predicted), median (IQR) | 55.0 (42.0–66.0) | 43.5 (33.8–51.0) | 61 (49.0–71.5) |
Composite physiological index, median (IQR) | 40.9 (28.8–50.0) | 52.0 (47.1–59.5) | 35.4 (25.8–45.1) |
Radiological pattern | |||
UIP | 13 (7.2) | 6 (12.1) | 7 (5.5) |
NSIP | 115 (63.5) | 43 (75.9) | 72 (56.2) |
Indeterminate ILD | 53 (29.3) | 4 (10.3) | 49 (38.3) |
Extent of ILD (%), median (IQR) | 10 (5–20) | 30 (16–35) | 5 (5–12) |
Emphysema association, n (%) | 34 (18.8) | 9 (17.0) | 25 (19.5) |
Extent of emphysema (%), median (IQR) | 5 (3–10) | 3 (3–7.5) | 5 (3–10) |
Hiatal hernia | 29 (16.0) | 9 (17.2) | 20 (15.6) |
Esophageal dilation | 92 (47.9) | 30 (51.7) | 62 (48.4) |
Immunosuppressive therapy initiationa, n (%) | 81 (44.8) | 31 (58.5) | 50 (39.1) |