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Table 2 Comparison of pulmonary function tests (PFTs) at diagnosis between clinically diagnosed FPF (FPF-DC), FPF diagnosed through screening (FPF-DS) and sporadic groups

From: A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features

PFT parameter

Number (%) of FPF-DC patients with abnormal findinga

Mean % of predicted value +/− SD

Number (%) of FPF-DS patients with abnormal findinga

Mean % of predicted value +/− SD

Number (%) of sporadic PF patients with abnormal findinga

Mean % of predicted value +/− SD

FVC

23/45 (51.1 %)

78.4 % +/− 15.4 %

6/16 (37.5 %)

92.7 % +/−16.8 %

24/46 (52.2 %)

80.1 % +/− 19.3 %

TLC

20/41 (48.7 %)

78.5 % +/− 15.4 %

6/16 (37.5 %)

86.6 +/−19.6 %

19/41 (46.3 %)

80.2 % +/− 17.9 %

FEV1

22/42 (52.3 %)

79.1 % +/− 15.1 %

5/16 (31.2 %)

87.1 % +/−12.9

21/46 (45.6 %)

81.5 % +/− 17.6 %

DLCO

39/40 (97.5 %)

56.4 % +/− 15.7 %

12/16 (75.0 %)

66.4 % +/−18.4

37/42 (88.0 %)

57.2 % +/− 23.2 %

Isolated reduction in DLCO (other PFTs normal)

12/36 (33.3 %)

N/C

6/16 (37.5 %)

N/C

14/39 (35.9 %)

N/C

  1. SD = standard deviation, FVC = forced vital capacity, TLC = total lung capacity, FEV1 = forced expiratory volume exhaled in the 1st second, DLCO = diffusing capacity of the lung for carbon monoxide.
  2. a Abnormal if: FVC, TLC, FEV1 or DLCO < 80 % predicted.