Table 1

Clinical characteristics of patients

FeaturePiZZPiSZPiSZ lung index casesPiMMp Value
n=699n=126N=64N=316PiSZ vs PiZZPiSZ index vs PiMM
Male404 (57.8)71 (56.3)38 (59.4)182 (57.7)0.7170.810
Age50.8 ((16.4)53.5 ((20.0)55.4 ((1.5)68.4 ((13.6)0.021<0.001
Smoking status<0.0010.004
 Ex smoker510 (73.0)56 (44.4)37 (57.8)192 (60.0)
 Never smoked161 (23.0)43 (34.1)15 (23.4)3 (0.9)
 Current smoker28 (4.0)27 (21.4)12 (18.8)121 (39.0)
Pack-years smoked13.5 ((23.5)13.0 ((35.4)26.2 ((3.0)44.2 ((32.5)0.402<0.001
Reason for diagnosis0.007
 Lung disease532 (76.2)77 (61.0)
 Family screening118 (16.9)38 (30.5)
 Liver disease11 (1.6)1 (0.8)
 Other reason38 (5.4)10 (7.9)
COPD587 (84.0)66 (52.4)43 (67.2)316 (100)<0.001<0.001
Emphysema528 (75.5)46 (36.5)35 (54.7)257 (81.3)<0.001<0.001
Chronic bronchitis249 (35.6)31 (24.6)18 (28.1)198 (62.8)0.0340.037
Bronchiectasis189 (27.0)22 (17.4)11 (17.2)96 (30.3)0.041<0.001
AAT level4.0 ((2.1)14.4 (4.4)14.9 (3.7)<0.001
FEV1pp47.9(45.6)94.5 (51.3)76.5 (2.6)46.4 (26.0)<0.001<0.001
FEV1/FVC (%)38.2 (26.4)67.5 (37.5)55.0 (2.7)43.3 (20.0)<0.0010.004
TLCpp115.3 (21.9)107.4 (18.3)109.2 (1.9)103.0 (20.2)<0.001<0.001
RVpp119.3 (54.9)96.9 (46.3)107.9 (4.6)119.8 (53.8)<0.001<0.001
DLCOpp69.74(36.0)86.3 (34.2)76.5 (3.1)48.6 (23.9)<0.001<0.001
KCOpp64.3 (28.8)89.0 (29.5)78.1 (3.0)59.0 (29.6)<0.001<0.001
Follow-up time (years)10.4 (5.3)7.0 (8.5)7.8 (0.6)3.2 (3.0)0.021<0.001
Deceased172 (24.6)10 (7.9)7 (10.9)91 (28.8)<0.0010.003
  • Data are shown as n (%) or median (IQR). In PiSZ index cases, data are shown as mean (SE) due to normal distribution.

  • AAT, alpha-1 antitrypsin; DLCO, carbon monoxide diffusing capacity; KCO, carbon monoxide transfer coefficient; RV, residual volume; TLC, total lung capacity.