Table 1– Characteristics of patients
Patient 1Patient 2
Age at diagnosis of emphysema yr7159
SexFemaleFemale
BMI kg·m-21818
SmokingNeverNever
Inhaled toxicsNoneNone
FEV1 L (% pred)#0.810 (39)1.640 (67)
FVC L (% pred)#2.540 (97)3.580 (125)
FEV1/FVC#31.945.8
TLC L (% pred)4.448 (94)6.800 (133)
RV L (% pred)1.910 (91)3.190 (165)
Pa,O2mmHg6879
Pa,CO2 mmHg3632
CT scanCentrilobular and panlobular emphysema mild bronchiectasisCentrilobular and bullous emphysema
Serum α1-antitrypsin1.35 g·L−11.98 g·L−1
Protease inhibitor phenotypePi MMPi MS
Duodenal biopsySubtotal villous atrophyTotal villous atrophy
IgG anti-gliadin U·mL−12362
IgA anti-gliadin U·mL−1>150114
IgG anti-transglutaminase+20
IgA anti-transglutaminase+>120
Haemoglobin g·dL−113.210.8
Mean corpuscular volume μm39374
Ferritin ng·mL−11543
25 hydroxy vitamin D3 ng·mL−11512
  • BMI: body mass index; FEV1: forced expiratory volume in 1 s; % pred: % predicted; FVC: forced vital capacity; TLC: total lung capacity; RV: residual volume; Pa,O2: arterial oxygen tension; Pa,CO2: arterial carbon dioxide tension; CT; computed tomography; Ig; immunoglobulin. #: results are presented as post-bronchodilator data.