Radiologic and clinical features of COPD patients with discordant pulmonary physiology: lessons from alpha1-antitrypsin deficiency.
BACKGROUND
Subjects with COPD display heterogeneity in clinical, physiologic, and radiologic characteristics, which are thought to result from different pathophysiologic mechanisms. It is important to identify and understand specific phenotypes for patient management. We investigated differences in emphysema distribution and health status in alpha(1)-antitrypsin deficient subjects (PiZ) with discordant lung function.


METHODS
CT scan densitometry, arterial oxygen tension, and St. George respiratory questionnaire scores were compared for 15 subjects with normal FEV1 and lung diffusion capacity corrected for alveolar ventilation (KCO), both defined as > 80% predicted (group 1), 10 subjects with abnormal FEV(1) and normal KCO (group 2), 15 subjects with normal FEV1 and abnormal KCO (group 3), and 10 subjects with both an abnormal FEV1 and KCO (group 4).


RESULTS
Group 2 subjects had the greatest predominance of basal emphysema, and group 3 subjects had the least. Upper zone voxel index (ie, the percentage of voxels < -910 Hounsfield units) was greater in all groups with abnormal lung function (p = 0.003, 0.044, and < 0.001, respectively), indicating more upper zone emphysema than in subjects with normal lung function. Lower zone voxel index was increased in groups 2 and 4 compared to groups 1 and 3. Groups 2 and 4 had a lower Pao(2) (p < 0.001) than the other groups. All groups with abnormal lung function had a worse quality of life than those with normal lung function.


CONCLUSIONS
Abnormality of FEV1 is associated with basal-predominant emphysema, and abnormality of KCO is associated with relatively more upper zone emphysema; but, an isolated defect in KCO has a significant effect on health status.
Holme
Jayne
J
University Hospital Birmingham, Birmingham, UK.
Stockley
Robert A
RA
D000328:Adult; D016022:Case-Control Studies; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006304:Health Status; D006801:Humans; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011650:Pulmonary Alveoli / Q000502:physiology; D011653:Pulmonary Diffusing Capacity / Q000502:physiology; D029424:Pulmonary Disease, Chronic Obstructive / Q000000981:diagnostic imaging* / Q000209:etiology / Q000503:physiopathology*; D011788:Quality of Life; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity / Q000502:physiology; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*