Baseline characteristics and treatment modalities of patients with PF-ILD at PF-ILD diagnosis
| PF-ILD | P value | |||||||
| Total (n=167) | IPF (n=64) | Non-IPF (n=103) | ||||||
| IIPs other than IPF (n=49), NSIP (n=19), PPFE (n=2), unclassified (n=26), COP (n=2) | CTD-ILD (n=31), SSc (n=12), RA (n=8), IM* (n=7), others† (n=4) | IPAF (n=7) | HP (n=10) | Others‡ (n=6) | ||||
| Characteristics | ||||||||
| Sex, male | 115 (68.86) § | 48 (75.0) | 34 (69.39) | 17 (54.84) | 5 (71.43) | 6 (60.0) | 5 (83.33) | 0.4330 |
| Age (years) | 71.0 (64.0–75.0) | 73.0 (68.25–77.0) | 70.0 (63.5–74.5) | 64.0 (52.0–71.0) | 73.0 (67.0–74.0) | 69.5 (63.75–74.5) | 71.5 (66.75–79.5) | 0.0009 |
| BMI | 22.75 (21.2–25.33) | 23.65 (21.77–26.16) | 21.93 (20.17–24.41) | 22.34 (20.7–25.11) | 22.84 (22.02–24.26) | 22.02 (20.98–25.56) | 22.29 (19.57–28.32) | 0.3754 |
| COPD | 24 (14.37) | 9 (14.06) | 8 (16.33) | 3 (9.68) | 0 (0.00) | 2 (20.00) | 2 (33.33) | 0.4411 |
| DM | 24 (14.37) | 11 (17.19) | 7 (14.29) | 4 (12.9) | 1 (14.29) | 0 (0.00) | 1 (16.67) | 0.5331 |
| Serological examination | ||||||||
| Monocyte count | 429.2 (331–545.8) | 411.9 (331.8–531.8) | 421.1 (312–556.2) | 432.3 (335.8–712.5) | 516.6 (354.5–557.4) | 408.3 (265.5–480.3) | 507.1 (417.2–610.2) | 0.4060 |
| LDH | 217 (191.8–251.3) | 215 (192-260) | 218 (188-258) | 233 (190-254) | 221 (187-232) | 213 (192.5–233) | 217 (203.5–239) | 0.9941 |
| KL-6 | 896 (584.8–1357.2) | 926.6 (681.5–1588.1) | 791.5 (479.8–1079.9) | 952.3 (618.3–1335) | 826 (450.5–1503) | 1425.2 (572.3–1898.5) | 562.5 (336.8–806) | 0.0037 |
| CRP | 0.16 (0.1–0.38) | 0.16 (0.1–0.44) | 0.11 (0.08–0.11) | 0.31 (0.11–0.73) | 0.1 (0.05–0.77) | 0.18 (0.11–0.29) | 0.18 (0.13–0.35) | 0.2253 |
| Pulmonary function | ||||||||
| FVC | 2.29 (1.8–2.84) | 2.32 (1.90–2.85) | 2.42 (1.85–2.86) | 2.19 (1.7–2.61) | 2.39 (1.79–3.48) | 1.99 (1.67–2.6) | 3.01 (1.93–3.47) | 0.5002 |
| %FVC | 82.2 (69.1–91.7) | 84.45 (72.45–93.63) | 79.5 (65.55–92) | 78.3 (63.9–88.6) | 87.9 (73.9–98.6) | 72.15 (63.88–92.38) | 81.2 (59.9–90.98) | 0.1520 |
| DLCO (n=125) | 10.07 (7.47–12.0) | 10.36 (7.88–12.20) | 10.16 (7.25–12.27) | 9.22 (6.36–11.74) | 11.06 (7.21–13.38) | 10.54 (7.74–12.66) | 7.33 (5.62–8.91) | 0.4235 |
| %DLCO (n=125) | 58.5 (44.6–75.27) | 60.42 (47.03–78.09) | 61.8 (43.5–80.99) | 54.2 (34.55–62.85) | 65.47 (50.65–76.30) | 62.37 (51.37–74.68) | 41 (39.4–49.18) | 0.2098 |
| Radiological findings | ||||||||
| Honeycombing | 65 (38.92) | 36 (56.25) | 15 (30.61) | 9 (29.03) | 2 (28.57) | 3 (30.0) | 0 (0.0) | 0.0084 |
| Traction bronchiectasis | 101 (60.48) | 41 (64.06) | 29 (59.18) | 21 (67.74) | 3 (42.86) | 4 (40.0) | 3 (50.0) | 0.5361 |
| Immunosuppressive agents | ||||||||
| Glucocorticoids (PSL<10 mg/day) | 12 (5.99) | 0 (0.0) | 1 (1.96) | 11 (35.48) | 0 (0.0) | 0 (0.0) | 0 (0.0) | <0.001 |
| Glucocorticoids (PSL≥10 mg/day) | 23 (13.77) | 7 (10.94) | 7 (14.29) | 7 (22.58) | 0 (0.0) | 2 (20.0) | 0 (0.0) | 0.5147 |
| Immunosuppressant | 16 (9.58) | 2 (3.13) | 5 (10.2) | 9 (29.03) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0.0077 |
*Dermatomyositis (n=4), polymyositis (n=3).
†Microscopic polyangiitis (n=2), mixed connective tissue disease (n=1), systemic lupus erythematosus (n=1).
‡Exposure-related ILD (n=5), sarcoidosis (n=1)
§Data are shown as n (%) or median (IQR).
COPD; chronic obstructive lung pulmonary disease; BMI, body mass index; COP, cryptogenic organising pneumonia; CRP, C-reactive protein; CTD-ILD, connective tissue disease-interstitial lung disease; DLCO, diffusing capacity for carbon monoxide; DM, diabetes mellitus; FVC, forced vital capacity; HP, hypersensitivity pneumonitis; IIPs, idiopathic interstitial pneumonias; IM, inflammatory myopathies; IPAF, interstitial pneumonia with autoimmune features; IPF, idiopathic pulmonary fibrosis; KL-6, sialylated carbohydrate antigen Krebs von den Lungen-6; LDH, lactate dehydrogenase; NSIP, non-specific interstitial pneumonia; PF-ILD, progressive fibrosing interstitial lung disease; PPFE, pleuroparenchymal fibroelastosis; PSL, prednisolone; RA, rheumatoid arthritis; SSc, systemic sclerosis.