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Table 5 Comparison of pulmonary function tests (PFTs) obtained at most recent follow-up 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

32/44 (72.7 %)

70.5 % +/−19.5 %

6/16 (37.5 %)

90.3 % +/−17.8 %

33/47 (70.2 %)

69.9 % +/− 18.6 %

TLC

20/29 (69.0 %)

69.9 % +/− 17.0 %

5/15 (33.3 %)

85.6 % +/−20.3 %

20/32 (62.5 %)

69.2 % +/− 17.2 %

FEV1

35/43 (81.3 %)

68.6 % +/− 18.6 %

7/16 (43.8 %)

83.2 % +/−15.6 %

32/47 (68.0 %)

71.8 % +/− 18.5 %

DLCO

34/35 (97.1 %)

47.6 % +/− 20.1 %

15/16 (93.8 %)

60.2 % +/−13.8 %

35/38 (92.1 %)

47.3 % +/− 26.4 %

Isolated reduction in DLCO (other PFTs normal)

4/25 (16.0 %)

N/C

6/15 (40.0 %)

N/C

2/29 (6.9 %)

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.