Abstract
Introduction: AATD is associated with emphysema. Chronic Obstructive Pulmonary Disease (COPD) has a prevalence of 2.74% in GG&C.
Aim: We aim to assess the prevalence of AATD in patients with COPD in GG&C and their management after diagnosis.
Method: We accessed electronic data records of patients diagnosed with Pi*ZZ, Pi*SZ, or Pi*SS AATD since 1999. We compared age of diagnosis, age of death (if relevant), sex, alpha-1 antitrypsin level, pulmonary function tests, smoking status, CT thorax, and frequency of liver disease monitoring between genetic phenotypes.
Results: There were 195 patients in total (62 Pi*ZZ, 110 Pi*SZ, 23 Pi*SS). 33 (54.8%) Pi*ZZ patients had clinically diagnosed COPD. The average age of diagnosis for Pi*ZZ patients was 49 and the average age of death was 61. 22 (35.4%) Pi*ZZ patients died within the audit period. 34 (54.8%) Pi*ZZ patients were current or ex-smokers. Of these, 23 (67.6%) developed COPD and 14 (41%) were offered smoking cessation. 41 (66.1%) Pi*ZZ patients had a CT thorax and 83% of these showed emphysema. 25 (37%) had annual liver function tests and 7 (11%) had annual spirometry. The prevalence of Pi*ZZ AATD in the GG&C COPD population was 0.11%. In the Pi*SZ and Pi*SS groups, 10 (9%) and 1 (4.3%) had emphysema respectively.
Conclusion: Our audit demonstrated a much lower prevalence of Pi*ZZ AATD than anticipated which may reflect a lack of structured screening services. It also suggests that those diagnosed with AATD are not followed up as per the COPD Foundation guidelines. Mortality was higher for Pi*ZZ patients compared to the overall COPD population. This audit suggests that greater recognition and follow up of this cohort is required.
Footnotes
Cite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 2431.
This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2020