The Relationship of Chronic Sputum Expectoration to Physiologic, Radiologic, and Health Status Characteristics in α1-Antitrypsin Deficiency (PiZ)

Study objectives First, to determine the relationships among chronic sputum expectoration (CSE), exacerbations, airflow obstruction, and emphysema in patients with α 1 -antitrypsin deficiency (α 1 -ATD) [PiZ]. Second, to use multivariate analysis to determine how these factors influence health status. Design Cross-sectional, single-center. Setting UK center for α 1 -ATD, university teaching hospital. Patients One hundred seventeen nonsmoking patients underwent lung function testing, high-resolution CT (HRCT) scanning with density mask analysis, and health status assessment using the St. George's Respiratory Questionnaire (SGRQ) and short form 36 (SF-36) health survey questionnaire. Results Patients with CSE (n = 50) had worse postbronchodilator airflow obstruction than those who did not (p = 0.03), with a median FEV 1 of 1.15 L (interquartile range [IQR], 0.76 to 1.82) vs 1.44 L (IQR, 0.99 to 2.93), respectively, and higher HRCT scan voxel index (VI) values indicating more extensive emphysema (patients with CSE: median lower zone VI, 50; IQR, 28 to 61; patients without CSE: median lower zone VI, 41; IQR, 5 to 53; p = 0.04). Patients with CSE also had worse health status, as assessed by the SGRQ (p 1 , exacerbation frequency, and lower zone VI to be the most important predictors of health status. Conclusions Among patients with α 1 -ATD, those with CSE expectoration exhibit greater physiologic impairment and more extensive emphysema than those without. This is reflected in an inferior health status, which is also influenced independently by an increased exacerbation frequency in those with CSE.