Table 2.
KIF15-missense (N = 5) | Others (N = 130) | Sig | |
---|---|---|---|
Background and comorbidities | |||
Female sex | 0 (0%) | 43 (33%) | 0.177 |
Male sex | 5 (100%) | 87 (67%) | 0.177 |
Family history of pulmonary fibrosis | 0 (0%) | 8 (6%) | 1.000 |
Smoking history | 4 (80%) | 82 (63%) | 0.653 |
Cancers | 1 (20%) | 32 (25%) | 1.000 |
BMI, kg/m2 | 34.0 (28.1–36.0) | 27.8 (25.0–30.0) | 0.028* |
Comorbidities, N | 5.0 (2.0–7.5) | 4.0 (2.0–6.0) | 0.432 |
Treatment and prognosis | |||
Oxygen therapy | 4 (80%) | 39 (30%) | 0.036* |
Antifibrotic treatment | 4 (80%) | 75 (58%) | 0.402 |
Lung transplantation | 2 (40%) | 13 (10%) | 0.095 |
Deceased | 1 (20%) | 61 (47%) | 0.374 |
Age at diagnosis, years | 54.0 (36.5–69.5) | 72.0 (65.8–75.3) | 0.023* |
Age at death, years | 61.2 (61.2–61.2) | 79.0 (74.1–83.2) | 0.032* |
Age at transplant, years | 40.2 (30.9–40.2) | 61.8 (53.6–65.2) | 0.038* |
Age at death or transplant, years | 49.4 (30.9–49.4) | 76.8 (69.3–82.0) | < 0.001* |
Symptoms | |||
Dyspnoea | 3 (60%) | 65 (50%) | 1.000 |
Cough | 1 (20%) | 68 (52%) | 0.202 |
Acute exacerbations | 1 (20%) | 33 (25%) | 1.000 |
Laboratory findings | |||
Macrocytosis | 1 (20%) | 29 (23%) | 1.000 |
Thrombocytopenia | 1 (20%) | 17 (13%) | 0.520 |
Radiological findings | |||
Right ventricular strain | 1 (20%) | 16 (12%) | 0.495 |
Emphysema | 1 (20%) | 31 (24%) | 1.000 |
Honeycombing | 4 (80%) | 104 (80%) | 1.000 |
Traction bronchiectasis | 5 (100%) | 127 (98%) | 1.000 |
Ground-glass opacity | 2 (40%) | 23 (18%) | 0.231 |
Pulmonary function test findings | |||
FVC, L | 3.35 (2.77–3.58) | 2.95 (2.40–3.71) | 0.578 |
FVC, % predicted | 59.0 (51.5–90.0) | 81.0 (68.0–93.0) | 0.128 |
FEV1, L | 2.73 (2.27–2.76) | 2.36 (1.96–3.04) | 0.562 |
FEV1, % predicted | 66.0 (55.0–84.0) | 82.0 (73.0–95.0) | 0.086 |
DLCO, % predicted | 52.0 (37.0–64.5) | 59.0 (50.5–69.5) | 0.188 |
Bold values are statistically significant p < 0.05