Table 5.

Progression of HRCT findings (n = 37) and changes in PFT (n = 38) in patients with JMCTD. Values are mean (SD) or median (range) unless otherwise specified.

VariablesTime 1Time 2p
Disease duration at time of HRCT, yrs8.8 (0.6–33.6)17.8 (3.1–40.2)NA
Disease duration at time of PFT, yrs11.6 (3.3–30.9)17.7 (2.3–41.2)NA
Age at PFT examination, yrs21.9 (6.5)32.2 (6.7)NA
Age at HRCT, yrs21.7 (7.3)29.3 (8.6)NA
Time between HRCT scans8.3 (2.7)NA
Time between PFT10.3 (1.0)NA
FVC%92.7 (11.4)85.6 (15.6)0.004
FEV1%94.6 (12.0)85.9 (10.3)0.001
FEV1/FVC ratio0.87 (0.05)0.84 (0.05)NS
DLCO%72.2 (14.0)70.5 (11.3)NS
DLCO/VA%88.2 (15.7)88.2 (15.1)NS
ILD present, n (%)9 (24)10 (27)NS
Ground-glass attenuation, n (%)3 (8)0NS
Reticular pattern, n (%)7 (19)10 (27)NS
  Grade 18 (22)9 (24)NS
  Grade 24 (11)6 (16)NS
  Grade 301 (3)NS
Lung parenchyma with ILD, %2.0 (1–18)4.0 (1–18)NS
  • HRCT: high-resolution computed tomography; PFT: pulmonary function tests; JMCTD: juvenile mixed connective tissue disease; ILD: interstitial lung disease; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusion capacity of carbon monoxide; VA: alveolar volume; NA: not assessed; NS: not significant.