Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis

Respiratory Research

Table 1 Characteristics and clinical diagnoses of the patients with familial pulmonary fibrosis

Characteristics	N=68 (%)
Age (y)	64.4 ± 9.6
Nonsmoker	37 (54.4)
Pack-years	9 ± 11.9
Pulmonary function test results
FVC (%)^a	79.4 ± 19.5
FEV1 (%)^a	86.2 ± 20.1
FEV1/FVC^b	84.9 ± 5.8
DLCO (%)^c	61.3 ± 18.4
BMI	27.9 ± 5.59
Clinical diagnosis
IPF	46 (67.6)
Unclassifiable	9 (13.2)
CTD-ILD	7 (10.3)
PPFE	2 (2.9)
NSIP	2 (2.9)
COP	1 (1.5)
CHP	1 (1.5)

BMI body mass index, CHP chronic hypersensitivity pneumonitis, COP cryptogenic organizing pneumonia, CTD-ILD connective tissue disease associated interstitial lung disease, DLCO diffusion capacity to carbon monoxide, FEV1 forced expiratory volume in one second, FVC forced vital capacity, IPF idiopathic pulmonary fibrosis, NSIP nonspecific interstitial pneumonia, PPFE pleuroparenchymal fibroelastosis
^aMissing two cases
^bMissing seven cases
^cMissing three cases

ISSN: 1465-993X