TABLE 1

Patient features at diagnosis

Subjects130
Age years69±7.8
Male105 (80.8)
BMI kg·m−2
 <18.52 (1.5)
 18.5–24.916 (12.3)
 25–29.969 (53.1)
 ≥3042 (32.3)
Smoking exposure pack-years
 <20 33 (25.4)
 ≥2060 (46.2)
Alcohol
 Active15 (11.5)
 Former6 (4.6)
Reason for consultation
 Respiratory symptoms85 (65.4)
 Familiar study10 (7.7)
 Radiological or primary care protocol (without a respiratory clinic)35 (26.9)
Cough85 (65.4)
Dyspnoea (mMRC)
 022 (16.9)
 155 (42.3)
 242 (32.3)
 311 (8.5)
Crackles118 (90.8)
Clubbing fingers65 (50.0)
Thorax HRCT pattern
 Definite UIP pattern60 (46.2)
 Probable UIP pattern55 (42.3)
 Pattern indeterminate for UIP15 (11.5)
Biopsy
 Cryobiopsy4 (3.1)
 Surgical biopsy48 (36.9)
Anatomopathological patterns
 Definite UIP35 (26.9)
 Probable UIP10 (7.7)
 Possible UIP6 (4.6)
 Nonrepresentative1 (0.8)
Severe physiological limitation at diagnosis
 FVC <50%2 (1.5)
DLCO <30%11 (8.4)
 6MWD <350 m20 (15.4)
Family aggregation36 (27.7)
Telomere shortening23 (17.7)
Antifibrotic treatment
 Pirfenidone63 (48.5)
 Nintedanib67 (51.5)

Data are presented as n, mean±sd or n (%). BMI: body mass index; mMRC: Modified Medical Research Council; HRCT: high-resolution computed tomography; UIP: usual interstitial pneumonia; FVC: forced vital capacity; DLCO: diffusing capacity for carbon monoxide; 6MWD: 6-min walk distance.