Overall baseline demographic and clinical characteristics of all patients with SSc-ILD and characteristics stratified by ILD progression over the 12±3-month observation period
| Progression criteria: ∆FVC% predicted | Total (N=826) | Significant progression (n=100) | Moderate progression (n=123) | Stable (n=396) | Improvement (n=207) |
| <−10 | −10 to −5 | >−5 to <5 | ≥5 | ||
| Age, years (SD)* | 56 (13.1) | 59 (13.1) | 56 (12.4) | 55 (13.5) | 58 (12.4) |
| Male, n (%)* | 150 (18) | 17 (17) | 16 (13) | 81 (20) | 36 (17) |
| Disease characteristics at baseline | |||||
| Disease duration, years* (SD) | 9.7 (8.3) | 8.8 (7.7) | 10.2 (8.2) | 10.2 (8.5) | 8.9 (8.3) |
| Disease duration <3 years*, n (%) | 175 (21) | 26 (26) | 27 (22) | 68 (17) | 54 (26) |
| Diffuse cutaneous SSc, n (%) | 365/732 (50) | 44/96 (46) | 55/106 (52) | 182/357 (51) | 84/173 (49) |
| Limited cutaneous SSc, n (%) | 367/732 (50) | 52/96 (54) | 51/106 (48) | 175/357 (49) | 89/173 (51) |
| Anti-topoisomerase I Ab, n (%) | 421/789 (53) | 41/97 (42) | 64/117 (55) | 218/378 (58) | 98/197 (50) |
| Anti-centromere Ab, n (%) | 141/783 (18) | 19/97 (20) | 18/113 (16) | 59/376 (16) | 45/197 (23) |
| Anti-RNA polymerase III Ab, n (%) | 23/451 (5) | 3/54 (6) | 3/60 (5) | 10/217 (5) | 7/117 (3) |
| Follow-up period, years*, mean (SD) | 5.4 (2.0) | 5.8 (1.4) | 5.6 (2.0) | 4.8 (3.2) | 5.0 (3.2) |
| Lung characteristics | |||||
| FVC% predicted,* mean (SD) | 87 (21.1) | 95 (23.3) | 90 (21.8) | 85 (20.4) | 85 (19.7) |
| DLCO% predicted,* mean (SD) | 59 (18.3) | 61 (17.8) | 60 (17.9) | 58 (19.3) | 60 (16.8) |
| ∆FVC% predicted,† mean (SD) | –0.1 (10.2) | –18 (7.9) | –7 (1.3) | 0.3 (2.2) | 12 (7.0) |
| ∆DLCO% predicted,† mean (SD) | –0.7 (12.2) | –4 (15.4) | 2 (12.8) | –0.3 (10.9) | 0.9 (11.9) |
| NYHA class, n (%) | N=797 | n=99 | n=119 | n=377 | n=202 |
| 1 | 363 (44) | 44 (44) | 57 (46) | 167 (42) | 95 (46) |
| 2 | 317 (38) | 42 (42) | 44 (36) | 152 (38) | 79 (38) |
| 3 | 103 (13) | 10 (10) | 17 (14) | 50 (13) | 26 (13) |
| 4 | 14 (2) | 3 (3) | 1 (1) | 8 (2) | 2 (1) |
| Other characteristics | |||||
| mRSS, mean (SD) | N=747 10 (8.1) | n=96 11 (7.6) | n=112 10 (8.5) | n=352 10 (7.6) | n=187 10 (8.8) |
| ∆mRSS,† mean (SD) | N=698 –0.4 (4.6) | n=88 0.5 (4.3) | n=103 –0.4 (3.1) | n=337 –0.3 (4.4) | n=170 –1.2 (5.6) |
| Reflux/dysphagia symptoms, n (%) | 547/822 (67) | 76/100 (76) | 83/122 (68) | 261/393 (66) | 127/207 (61) |
| Digital ulcers, n (%) | 266/808 (32) | 35/100 (35) | 38/118 (31) | 141/386 (36) | 5/2042 (25) |
| Tendon friction rubs, n (%) | 73/804 (9) | 7/99 (7) | 10/119 (8) | 35/383 (9) | 21/203 (10) |
| Synovitis, n (%) | 117/810 (14) | 18/100 (18) | 15/120 (13) | 60/386 (16) | 24/204 (12) |
| Muscle weakness, n (%) | 182/814 (22) | 25/100 (25) | 31/120 (25) | 78/388 (20) | 48/206 (23) |
| Scleroderma renal crisis, n (%) | 11/818 (1) | 4/100 (4) | 3/120 (2) | 6/391 (2) | 1/206 (0.5) |
| ESR, mean (SD) | 766 (93) 26 (20.6) | 98 (98) 29 (23.9) | 115 (93) 25 (21.7) | 361 (91) 26 (19.5) | 192 (93) 25 (20.2) |
| Elevated CRP, n (%) | 217/797 (27) | 40/99 (30) | 25/120 (33) | 98/377 (26) | 49/201 (24) |
| Immunosuppressant use, n (%) | 89/244 (37) | 8/20 (40) | 8/31 (26) | 51/121 (42) | 22/72 (31) |
Significant progression (FVC decline of >10%); moderate progression (FVC decline of 5% to 10%); stable ILD (FVC decline or improvement of <5%); moderate improvement (FVC improvement of 5% to 10%). Definitions of organ manifestations were described previously.3 28 All characteristics were assessed before or on the index date. The following treatment options were received by the included patients at baseline, and for this study were defined as immunosuppressive: prednisone >10 mg/day, azathioprine, cyclophosphamide, mycophenolate, methotrexate or rituximab.
*Available for all 826 patients.
†Change from baseline to 12 months.
Ab, antibody; CRP, C-reactive protein; DLCO, diffusion capacity of the lungs for carbon monoxide; ESR, erythrocyte sedimentation rate; FVC, forced vital capacity; mRSS, modified Rodnan skin score; NYHA, New York Heart Association; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.