Table 2.
Demographics, serum biomarkers, pulmonary function tests, six-minute walk test, arterial blood gas analysis, and treatment at the time of registration for lung transplantation (n = 118)
| With rPPFE | Without rPPFE | P | |
|---|---|---|---|
| Number | 30 | 88 | |
| Demographics | |||
| Age | 45.5 (37, 51) | 51 (43, 56) | 0.05 |
| Male | 13 (43.3) | 58 (65.9) | 0.03 |
| Ever-smoking | 8 (26.7) | 54 (61.4) | 0.001 |
| Previous pneumothorax | 24 (80.0) | 23 (26.1) | < 0.001 |
| Family history | 2 (6.7) | 21 (23.9) | 0.16 |
| mMRC [1] | 3 (3, 4) | 4 (3, 4) | 0.15 |
| BMI | 15.9 (14.8, 17.2) | 21.5 (18.8, 25.1) | < 0.001 |
| Serum biomarkers | |||
| KL-6, IU/L | 542 (402, 698) | 1460 (933, 2150) | < 0.001 |
| LDH, IU/L | 186.5 (172, 212) | 229 (202, 271) | < 0.001 |
| Pulmonary function tests | |||
| %FVC | 35.75 (22.6, 46.2) | 48.5 (37.6, 62.0) | < 0.001 |
| %DLCO | 45.5 (37, 51) | 24.4 (17.4, 31.9) | 0.002 |
| Six-minute walk test | |||
| Distance, meter | 375 (243, 500) | 333 (235, 454) | 0.36 |
| Distance < 250 m | 8 (26.7) | 26 (29.5) | 0.82 |
| Lowest SpO2, % | 87.5 (81, 93) | 81 (76, 87) | < 0.001 |
| Arterial blood gas analysis | |||
| PaO2, Torr | 77.8 (65.4, 86.6) | 74.05 (63.4, 87.9) | 0.73 |
| PaCO2, Torr | 49.55 (45.6, 56.9) | 43.55 (40.7, 47.55) | < 0.001 |
| Treatment | |||
| Corticosteroid | 11 (36.7) | 62 (70.5) | 0.002 |
| IS agent | 3(10.0) | 40 (45.5) | < 0.001 |
| Antifibrotic agent | 7 (23.3) | 39 (44.3) | 0.05 |
| LTOT | 15 (50.0) | 63 (71.6) | 0.04 |
Data are presented as number (percentage) or median (interquartile range)
Abbreviations: PPFE plueroparenchymal fibroelastosis, rPPFE radiological PPFE, mMRC modified Medical Research Council dyspnea scale, BMI body mass index, KL-6 Krebs von der Lungen-6, LDH lactate dehydrogenase, %FVC the percentage to predicted forced vital capacity, %DLCO the percentage to predicted diffusion capacity of carbon monoxide, SpO2 percutaneous oxygen saturation, PaO2 arterial partial pressure of oxygen, PaCO2 arterial partial pressure of carbon dioxide, IS immunosuppressive, LTOT long-term oxygen therapy