TABLE 1.
fILD (n = 566) | PF-ILD COTTIN definition (n = 232) (CO) | PF-ILD RELIEF definition (n = 183) (RE) | PF-ILD INBUILD definition (n = 274) (IN) | PF-ILD uILD study definition (n = 174) (UILD) | CO vs RE | CO vs IN | RE vs IN | CO vs UILD | RE vs UILD | IN vs UILD | |
---|---|---|---|---|---|---|---|---|---|---|---|
Age at diagnosis (years) | 66.02 ± 11.89 | 66.81 ± 10.88 | 66.38 ± 10.43 | 65.81 ± 11.35 | 67.12 ± 10.71 | 0.68 | 0.31 | 0.58 | 0.78 | 0.51 | 0.22 |
Male_% | 53.32 | 53.02 | 54.10 | 55.47 | 58.62 | 0.82 | 0.58 | 0.77 | 0.26 | 0.74 | 0.51 |
BMI | 28.32 ± 5.28 | 28.94 ± 5.42 | 29.08 ± 5.28 | 29.07 ± 5.38 | 29.13 ± 5.61 | 0.78 | 0.78 | 0.97 | 0.73 | 0.93 | 0.90 |
Never smoker_% | 41.6 | 41.7 | 36.57 | 38.11 | 37.06 | 0.37 | 0.72 | 0.58 | 0.64 | 0.77 | 0.97 |
Former smoker_% | 53.92 | 53.36 | 60 | 56.6 | 57.65 | 0.37 | 0.72 | 0.58 | 0.64 | 0.77 | 0.97 |
Current Smoker_% | 4.48 | 4.93 | 3.43 | 5.28 | 5.29 | 0.37 | 0.72 | 0.58 | 0.64 | 0.77 | 0.97 |
Biopsy_% | 259 (45.76) | 111 (47.84) | 84 (45.9) | 129 (47.08) | 75 (45.4) | — | — | — | — | — | — |
SLB (n, %) | 24 (4.24) | 10 (4.31) | 11 (6.01) | 13 (4.74) | 10 (5.75) | — | — | — | — | — | — |
Cryobiopsy (n, %) | 135 (23.85) | 57 (24.57) | 49 (26.78) | 69 (25.18) | 46 (26.44) | — | — | — | — | — | — |
TBB (n, %) | 100 (17.67) | 44 (18.97) | 24 (13.11) | 47 (17.15) | 23 (13.22) | — | — | — | — | — | — |
Bronchoalveolar lavage | |||||||||||
Macrophages % | 73 (56,81) | 72 (55,81) | 74 (56,81) | 73 (56,81) | 75 (55,82) | — | — | — | — | — | — |
Lymphocytes % | 12 (4,24) | 12 (4,24.5) | 12 (5,24) | 12 (4,24) | 12 (4,23) | — | — | — | — | — | — |
Neutrophils % | 5 (2,10) | 4 (2,10) | 5 (2,10) | 5 (2,10) | 4 (2,10) | — | — | — | — | — | — |
Eosinophils % | 3 (1,7) | 3 (1,7) | 3 (1,7) | 3 (1,7) | 3 (1,7) | — | — | — | — | — | — |
Mast-cells % | 0 (0,1) | 0 (0,1) | 0 (0,1) | 0 (0,1) | 0 (0,1) | — | — | — | — | — | — |
FVC% | 72.67 ± 21.10 | 76.93 ± 21.60 | 80.03 ± 22.33 | 76.41 ± 21.16 | 75.67 ± 22.81 | 0.15 | 0.78 | 0.08 | 0.57 | 0.06 | 0.72 |
DLCO% | 47.07 ± 16.99 | 48.06 ± 16.89 | 50.48 ± 16.72 | 48.86 ± 16.22 | 47.45 ± 16.07 | 0.15 | 0.59 | 0.31 | 0.71 | 0.08 | 0.38 |
6MWT distance | 364 ± 106 | 374 ± 101 | 379 ± 97 | 375 ± 101 | 368 ± 102 | 0.63 | 0.89 | 0.71 | 0.61 | 0.34 | 0.51 |
Median follow up time (months) | 29 (10,45) | 34 (21,50) | 32 (22,44.5) | 33 (22,50) | 29 (17,43) | 0.39 | 0.94 | 0.42 | 0.01 | 0.08 | 0.01 |
Acute exacerbations_% | 18 | 26.9 | 24.59 | 25.55 | 25.14 | — | — | — | — | — | — |
Background treatment | |||||||||||
corticosteroids (n, %) | 338 (59.71) | 162 (69.82) | 126 (68.85) | 191 (69.70) | 115 (66.09) | — | — | — | — | — | — |
azathioprine (n, %) | 180 (31.80) | 97 (41.81) | 79 (43.16) | 116 (42.33) | 63 (36.20) | — | — | — | — | — | — |
methotrexate (n, %) | 88 (15.54) | 49 (21.12) | 37 (20.21) | 49 (17.88) | 27 (15.51) | — | — | — | — | — | — |
cyclophosphamide (n, %) | 39 (6.89) | 18 (7.75) | 15 (8.19) | 20 (7.29) | 12 (6.89) | — | — | — | — | — | — |
mycophenolate mofetil (n, %) | 6 (1.06) | 3 (1.29) | 6 (3.27) | 4 (1.45) | 2 (1.14) | — | — | — | — | — | — |
sulfasalazine (n, %) | 8 (1.41) | 4 (1.72) | 1 (0.54) | 4 (1.45) | 2 (1.14) | — | — | — | — | — | — |
acetylcysteine (n, %) | 59 (10.42) | 28 (12.06) | 15 (8.19) | 32 (11.67) | 24 (13.79) | — | — | — | — | — | — |
pirfenidone (n, %) | 23 (4.06) | 19 (8.18) | 13 (7.10) | 18 (6.56) | 10 (5.74) | — | — | — | — | — | — |
nintedanib (n, %) | 14 (2.47) | 9 (3.87) | 9 (4.91) | 11 (4.01) | 4 (2.29) | — | — | — | — | — | — |
Underlying diagnosis | |||||||||||
cHP (n, %) | 201 (35.51) | 38.36 | 36.61 | 36.13 | 37.36 | 0.59 | 0.86 | 0.92 | 0.22 | 0.59 | 0.34 |
iNSIP (n, %) | 78 (13.78) | 11.64 | 15.30 | 14.6 | 16.67 | 0.59 | 0.86 | 0.92 | 0.22 | 0.59 | 0.34 |
uILD (n, %) | 111 (19.61) | 23.28 | 23.50 | 22.63 | 27.59 | 0.59 | 0.86 | 0.92 | 0.22 | 0.59 | 0.34 |
CTD-ILD (n, %) | 162 (28.62) | 25.43 | 21.86 | 24.82 | 17.24 | 0.59 | 0.86 | 0.92 | 0.22 | 0.59 | 0.34 |
Other fILD (n, %) | 14 (2.47) | 1.29 | 2.73 | 1.82 | 1.15 | 0.59 | 0.86 | 0.92 | 0.22 | 0.59 | 0.34 |