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

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

Lung function tests at initial diagnosis f-IPF (n = 27) s-IPF (n = 143) p value
mean value ± SD mean value ± SD
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
  1. 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