Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF)

Table 1 Comparison between idiopathic pleuroparenchymal fibroelastosis (IPPFE) and idiopathic pulmonary fibrosis (IPF): clinical characteristics

	IPPFE (n = 6)	IPF (n = 28)	p value
Sex, male/female	5/1	26/2	0.452*
Age at biopsy, yr	69.2 ± 3.9	61.4 ± 9.9	0.072
Smoking history, current/ex/never	0/3/3	13/13/2	0.011*
Pack-year of smoking	29.3 ± 33.1	66.8 ± 97.4	0.363
Detection by medical check-up, n (%)	3 (50)	21 (75.0)	0.328*
Respiratory symptoms at biopsy, n (%)	6 (100)	19 (67.9)	0.162*
Body mass index, kg/m²	17.9 ± 0.9	24.3 ± 2.8	<0.0001
Clubbing of finger, n (%)	1 (16.7)	6 (21.4)	0.999*
Fine crackles, n (%)	3 (50)	27 (96.4)	0.012*
Pneumothorax, n (%)	4 (66.7)	1 (3.6)	0.002*
Acute exacerbation, n (%)	1 (16.7)	6 (21.4)	0.999*
Therapeutic intervention, n (%)	4 (66.7)	15 (53.6)	0.672*
Period from detection of IP until acquisition of lung specimens	73.3 ± 46.1	29.7 ± 34.3	0.012
Observation period, mo	39.8 ± 29.2	67.8 ± 46.7	0.206
Death, n (%)	3 (50.0)	12 (42.9)	0.999*

Data are presented as n, mean ± SD or n (%).
Data were analyzed with Student’s t-test or *Fisher’s exact probability test.
Abbreviations: IPPFE idiopathic pleuroparenchymal fibroelastosis, IPF idiopathic pulmonary fibrosis, IP interstitial pneumonia.

ISSN: 1471-2466