Table 1.
Demographic and clinical characteristics of patients with systemic sclerosis according to ILD extent on HRCT.
| Variable | Extensive ILD (n=21) | Non-extensive ILD (n=62) | Total (n=83) | p |
|---|---|---|---|---|
| Age (years), mean (SD) | 41.90 (10.07) | 40.95 (11.16) | 41.19 (10.84) | 0.730 |
| Sex — Male, n (%) | 0 (0) | 5 (8) | 5 (6) | 0.323 |
| Sex — Female, n (%) | 21 (100) | 57 (92) | 78 (94) | 0.323 |
| Smoking history — Non-smoker, n (%) | 21 (100) | 57 (92) | 78 (94) | 0.323 |
| Smoking history — Moderate smoker*, n (%) | 0 (0) | 5 (8) | 5 (6) | 0.323 |
| SSc subtype — Diffuse cutaneous (dcSSc), n (%) | 13 (62) | 42 (68) | 55 (66) | 0.709 |
| SSc subtype — Limited cutaneous (lcSSc), n (%) | 8 (38) | 19 (31) | 27 (33) | 0.709 |
| SSc subtype — Sine scleroderma (ssSSc), n (%) | 0 (0) | 1 (2) | 1 (1) | 0.709 |
| Organ involvement — Musculoskeletal, n (%) | 8 (38) | 28 (45) | 36 (43) | 0.619 |
| Organ involvement — Renal, n (%) | 1 (5) | 0 (0) | 1 (1) | 0.253 |
| Organ involvement — Gastrointestinal, n (%) | 10 (48) | 14 (23) | 24 (29) | 0.049 |
| mRSS, median (IQR) | 8 (3–10) | 7 (3–15) | 7 (3–15) | 0.459 |
| Disease duration (months), median (IQR) | 70 (12–114) | 28 (10–90) | 38 (11–95) | 0.423 |
| Predominant HRCT pattern — NSIP, n (%) | 9 (43) | 42 (68) | 51 (61) | 0.068 |
| Predominant HRCT pattern — UIP, n (%) | 12 (57) | 20 (32) | 32 (39) | 0.068 |
Table 2.
Functional, laboratory, and treatment characteristics of patients with systemic sclerosis according to ILD extent on HRCT.
| Variable | Extensive ILD (n=21) | Non-extensive ILD (n=62) | Total (n=83) | p |
|---|---|---|---|---|
| 6MWT distance (m), median (IQR) | 330 (281–392) | 349 (307–390) | 348 (300–390) | 0.492 |
| Oxygen desaturation ≥4% during 6MWT, n (%) | 3 (14) | 0 (0) | 3 (4) | 0.015 |
| FVC (% predicted), mean (SD) | 47.0 (13.6) | 64.7 (15.2) | 60.2 (16.7) | 0.001 |
| FEV1/FVC (%), median (IQR) | 90.5 (83–93) | 87.8 (85–92) | 88.4 (84–92) | 0.480 |
| ESR (mm/h), median (IQR)† | 28 (12–59) | 41 (27–62) | 41 (25–61) | 0.242 |
| CRP (mg/L), median (IQR)‡ | 1.65 (0.77–5.42) | 1.65 (0.77–6.72) | 1.65 (0.77–6.07) | 0.950 |
| Anti-Scl-70 antibody, n (%) | 4 (19) | 12 (19) | 16 (19) | 1.000 |
| Anti-centromere antibody, n (%) | 0 (0) | 3 (5) | 3 (4) | 0.568 |
| Treatment — Treatmentnaïve, n (%) | 1 (5) | 7 (11) | 8 (10) | 0.423 |
| Treatment — Methotrexate, n (%) | 1 (5) | 7 (11) | 8 (10) | 0.423 |
| Treatment — Mycophenolate mofetil, n (%) | 19 (90) | 48 (77) | 67 (81) | 0.423 |
Table 3.
Univariate association between functional parameters and ILD extent on HRCT.
| Variable | Extensive ILD (n=21) | Non-extensive ILD (n=62) | PR (95% CI) | p |
|---|---|---|---|---|
| 6MWT distance ≤338.35 m, n (%) | 12 (57) | 26 (42) | 1.579 (0.743–3.353) | 0.311 |
| 6MWT distance >338.35 m, n (%) | 9 (43) | 36 (58) | Reference | — |
| Oxygen desaturation ≥4%, n (%) | 3 (14) | 0 (0) | 4.444 (2.95–6.69) | 0.015 |
| Oxygen desaturation <4%, n (%) | 18 (86) | 62 (100) | Reference | — |
| FVC <70% predicted, n (%) | 21 (100) | 38 (61) | 1.553 (1.284–1.877) | <0.001 |
| FVC ≥70% predicted, n (%) | 0 (0) | 24 (39) | Reference | — |
Table 4.
Multivariable analysis of functional parameters associated with extensive ILD.
| Variable | Crude PR (95% CI) | p | Adjusted PR (95% CI)* | p |
|---|---|---|---|---|
| FVC (% predicted) | 0.952 (0.936–0.970) | <0.001 | 0.948 (0.931–0.965) | <0.001 |
| Oxygen desaturation ≥4% | 4.444 (2.959–6.675) | <0.001 | 4.333 (2.528–7.427) | <0.001 |
| 6MWT distance (m) | 0.998 (0.995–1.002) | 0.376 | 0.997 (0.993–1.002) | 0.234 |