Demographics and clinical characteristics of the OUH cohort
| Total cohort (n=262) | Cyc (n=21) | MMF (n=14) | p-value | |
|---|---|---|---|---|
| Age at diagnosis, yrs (SD) | 51.4 (15.5) | 52.6 (12.1) | 43.5 (18.5) | – |
| Follow-up period, yrs (SD) | 6.4 (4.0) | 5.5 (3.9) | 4.1 (2.3) | – |
| Male, n (%) | 42 (16.0) | 6 (28.6) | 2 (14.3) | – |
| dcSSc, n (%) | 68 (26.0) | 8 (38.1) | 10 (71.4) | – |
| Deceased, n (%) | 54 (20.6) | 8 (38.1) | 4 (28.6) | – |
| PH, n (%) | 35 (14) | 8 (42.1) | 1 (7.7) | 0.038 |
| BL FVC%,(SD) | 96.4 (19.5) | 83.8 (16.5) | 81.5 (13.3) | – |
| BL DLCO%,(SD) | 70.4 (19.5) | 54.2 (16.2) | 61.8 (14.9) | – |
| BL fibrosis%,(SD) | 6.1 (11.3) | 19.1 (15.8) | 10.8 (10.3) | – |
| FVC% decline,(SD) | -0.9 (14.4) | -3.3 (14.3) | 2.6 (11.3) | – |
| Annual FVC% decline,(SD) | -0.02 (6.1) | -0.3 (9.1) | 2.3 (4.1) | – |
| DLCO% decline,(SD) | -9.1 (13.5) | -9 (14.1) | 0.7 (12.4) | – |
| Annual DLCO% decline,(SD) | -2 (5.1) | -2.1 (3.5) | 1.8 (4.6) | 0.014 |
| Fibrosis% progression,(SD) | 1.2 (5.7) | -1.3 (9.3) | 7.5 (11.1) | 0.024 |
| Annual fibrosis% progression,(SD) | 0.5 (2.8) | -0.3 (4.9) | 2.3 (4.7) | – |
n: number; BL:baseline, SD: standard deviation; FVC: Forced vital capacity; DLCO: diffusing lung capacity for carbon monoxide; dcSSc: diffuse cutaneous SSc; PH: pulmonary hypertension.