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Radiological and Clinical Features of COPD Patients with Discordant Pulmonary Physiology: Lessons from alpha-1-antitrypsin deficiency

¹University Hospital Birmingham NHS Foundation Trust, Birmingham, U.K. The University of Birmingham, Birmingham, U.K ²University Hospital Birmingham NHS Foundation Trust, Birmingham, U.K. The University of Birmingham, Birmingham, U.K

rob.stockley{at}uhb.nhs.uk

Abstract

INTRODUCTION: Subjects with COPD display heterogeneity in clinical, physiological and radiological characteristics, thought to result from different pathophysiological 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.

METHOD: CT scan densitometry, arterial oxygen tension and St Georges Respiratory Questionnaire scores were compared for 15 subjects with normal FEV1 and gas transfer corrected for alveolar volume (KCO), both defined as >80% predicted (group 1); 10 with abnormal FEV1 and normal KCO (group 2); 15 with normal FEV1 and abnormal KCO (group 3) and 10 with both an abnormal FEV1 and KCO (group 4).

RESULTS: Group 2 had the greatest predominance of basal emphysema and group 3 had the least. Upper zone voxel index (% voxels <-910 HU) 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 PaO2 (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.

CONCLUSION: Abnormality of FEV1 is associated with basal predominant emphysema and abnormality of KCO with relatively more upper zone emphysema but an isolated defect in KCO has a significant effect on health status.

Key Words: Pulmonary Disease • Chronic Obstructive • Alpha 1-Antitrypsin Deficiency • Respiratory Function Tests • Tomography Scanners • X-Ray Computed • Quality of Life