Table 1.
Baseline Characteristics of SSc-ILD Cohort
| Baseline Characteristics | All Subjects (N=93) | SSc-ILD without PH (N=64) | SSc-ILD and PH (N=29) | P value |
|---|---|---|---|---|
| Age at Initial Non-RP Sign/Symptom, mean ± SD years | 46.9 ± 13.3 | 45.2 ± 13.2 | 50.9 ± 12.8 | 0.054 |
| Age at ILD Diagnosis, mean ± SD years | 51.6 ± 12.2 | 49.7 ± 11.8 | 55.8 ± 12.1 | 0.02 |
| Age at Study Enrollment, mean ± SD years | 54.9 ± 11.5 | 52.9 ± 11.4 | 59.3 ± 10.6 | 0.01 |
| Female, no. (%) | 71 (76.3) | 47 (73.4) | 24 (82.8) | 0.33 |
| Race, no. (%) | 0.02 | |||
| White | 79 (84.9) | 57 (89.1) | 22 (75.9) | |
| African American | 8 (8.6) | 2 (3.1) | 6 (20.7) | |
| Asian/Asian American | 3 (3.2) | 3 (4.7) | 0 | |
| Native American /Alaskan Native | 1 (1.1) | 0 | 1 (3.4) | |
| Other | 2 (2.2) | 2 (3.1) | 0 | |
| Ethnicity, no. (%) | 0.51 | |||
| Hispanic | 9 (9.7) | 7 (10.9) | 2 (6.9) | |
| Non-Hispanic | 82 (88.2) | 55 (85.9) | 27 (93.1) | |
| Other | 2 (2.2) | 2 (3.1) | 0 | |
| SSc Subtype, no. (%) | 0.34 | |||
| Diffuse | 61 (65.6) | 44 (68.8) | 17 (58.6) | |
| Limited | 32 (34.4) | 20 (31.3) | 12 (41.4) | |
| Disease Duration | ||||
| Time from Initial Non-RP Sign/Symptom to Study Enrollment, mean ± SD years | 7.9 ± 7.2 | 7.7 ± 7.6 | 8.5 ± 6.1 | 0.65 |
| Time from Initial Non-RP Sign/Symptom to ILD Diagnosis, mean ± SD years | 4.7 ± 6.4 | 4.6 ± 6.9 | 4.9 ± 5.4 | 0.80 |
| Time from ILD Diagnosis to Study Enrollment, mean ± SD years | 3.2 ± 3.6 | 3.2 ± 3.2 | 3.5 ± 4.3 | 0.65 |
| ILD Duration, mean ± SD years | 4.7 ± 3.6 | 4.6 ± 3.2 | 4.8 ± 4.3 | 0.74 |
| MRSS (At Enrollment) N=89 | 9.4 ± 9.5 | 9.9 ± 9.8 | 8.4 ± 8.9 | 0.51 |
| Autoantibodies, no. (%) | ||||
| ANA Positivity N=86 | 79 (91.9) | 57 (95) | 22 (84.6) | 0.19 |
| ANA Pattern N=76 | 0.10 | |||
| Nucleolar | 11 (14.5) | 9 (16.4) | 2 (9.5) | |
| Centromere | 6 (7.9) | 2 (3.6) | 4 (19) | |
| Other† | 59 (77.6) | 44 (80) | 15 (71.4) | |
| Scl-70 Positivity N=85 | 24 (28.2) | 20 (33.3) | 4 (16) | 0.12 |
| RNA Polymerase III Positivity N=51 | 11 (21.6) | 9 (25.7) | 2 (12.5) | 0.47 |
| PM-Scl Positivity N=35 | 2 (5.7) | 1 (4.2) | 1 (9.1) | 0.54 |
| ILD (HRCT), no. (%) | 0.13 | |||
| NSIP | 84 (90.3) | 60 (93.8) | 24 (82.8) | |
| UIP | 9 (9.7) | 4 (6.3) | 5 (17.2) | |
| PFTs (near ILD Diagnosis) | ||||
| FVC% Predicted, mean ± SD N=93 | 76.2 ± 15.7 | 77.2 ± 14.3 | 73.9 ± 18.5 | 0.35 |
| TLC % Predicted, mean ± SD N=66 | 83 ± 16.3 | 85.0 ± 14.8 | 78.7 ± 18.8 | 0.14 |
| DLco % Predicted, mean ± SD N=85 | 58.3 ± 20.3 | 63.4 ± 20.1 | 46.8 ± 15.6 | <0.001 |
| FVC % Predicted/DLco % Predicted, mean ± SD N=85 | 1.5 ± 0.8 | 1.3 ± 0.5 | 1.9 ± 1.2 | 0.007 |
| TTE‡ | ||||
| RV Function, no (%) N=92 | <0.001 | |||
| Normal | 84 (91.3) | 62 (98.4) | 22 (75.9) | |
| Abnormal | 8 (8.7) | 1 (3.0) | 7 (24.1) | |
| RV Enlargement, no (%) N=93 | 0.02 | |||
| No | 80 (86.0) | 59 (92.2) | 21 (72.4) | |
| Yes | 13 (14.0) | 5 (7.8) | 8 (27.6) | |
| RVSP (mmHg), mean ± SD N=63* | 37.8 ± 19.6 | 30.9 ± 8.2 | 49 ± 26.7 | <0.001 |
No tricuspid regurgitation jet observed in 30 subjects.
Any positive ANA by IF pattern other than nucleolar or centromere pattern.
TTE data was captured after enrollment in cohort.
SSc-systemic sclerosis; ILD-interstitial lung disease; SSc-ILD-systemic sclerosis associated interstitial lung disease; PH-pulmonary hypertension; RP-Raynaud’s phenomenon; MRSS-modified Rodnan skin score; ANA-antinuclear antibody; IF-immunofluorescence; HRCT-high resolution computed tomography; NSIP-non-specific interstitial pneumonitis; UIP-usual interstitial pneumonitis; PFTs-pulmonary function tests; FVC-forced vital capacity; TLC-total lung capacity; DLco-diffusing capacity for carbon monoxide; TTE-echocardiogram; RV-right ventricular; RVSP-right ventricular systolic pressure.