Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF)

BMC Pulmonary Medicine

Table 4 Results of functional diagnostic in both sporadic and familial IPF cohorts

Lung function tests at initial diagnosis	f-IPF (n = 27)	s-IPF (n = 143)	p value
Lung function tests at initial diagnosis	mean value ± SD	mean value ± SD	p value
Spirometry/ Whole body plethysmography
VC [% predicted value]	74 ± 19	65 ± 18	0.027
FVC [% predicted value]	75 ± 20	62 ± 19	0.011
FEV1/ VC [% predicted value]	104 ± 13	108 ± 16	0.133
TLC [% predicted value]	73 ± 21	69 ± 18	0.293
RV [% predicted value]	87 ± 41	80 ± 30	0.547
RV/ TLC [% predicted value]	97 ± 26	108 ± 25	0.026
Gas exchange
DLCO/ SB [% predicted value]	55 ± 20	42 ± 18	0.006
Blood gas analysis at rest
pO2 [mmHg]	75 ± 13	70 ± 10	0.015
O2 Saturation [%]	95 ± 2	94 ± 2	0.259
pCO2 [mmHg]	38 ± 3	39 ± 6	0.262
6 min-walk test
SpO2 before test [%]	95 ± 2	95 ± 3	0.377
SpO2 after test [%]	96 ± 2	86 ± 8	0.0002
6 MWD [m]	376 ± 140	379 ± 125	0.834
O2 Substitution during the test [l/min]	1 ± 2	4 ± 3	0.003
Right heart echocardiography
PAPsys [mmHg]	32 ± 13	40 ± 16	0.055

Abbreviations: f-IPF familial idiopathic pulmonary fibrosis, s-IPF sporadic idiopathic pulmonary fibrosis, SD standard deviation, VC vital capacity, FVC forced vitality capacity, TLC total lung capacity, RV residual volume, TLCO/sb transfer factor in single breath method, pO2 partial pressure of oxygen, pCO2 partial pressure of carbon dioxide, SpO2 oxygen saturation, VO2max maximum oxygen absorption, PAPsys systolic pulmonal arterial pressure

ISSN: 1471-2466