BACKGROUND

Alpha 1-antitrypsin deficiency (AATD) it is a genetic disorder whereby reduced levels or function of the protease inhibitor lead to increased risk of pulmonary (emphysema and bronchiectasis) and hepatic disease.

Exhaled NO (eNO) level have been proposed as an early marker of pulmonary function impairment. However, the role of eNO in AATD still remains uncertain.

AIMS AND OBJETIVES

To assess the relationship between eNO levels and clinical features and pulmonary function in  patients with severe AATD.

METHODS

Prospective study of patients with severe AATD  (<60 mg/dL; PiZZ or piSZ) treated in a AATD consultation between 2018-2022. Clinical, radiological and functional data were collected. 

RESULTS

A total of n=46 patients were included for analysis. A comparative statistical analysis was performed between clinical and Functional variables and eNO levels. Main results are shown in Table 1. 

CONCLUSION

Patients with higher eNO levels tended to have better FEV1 and less decline in the last five years but did not reach statistical significance.

We could not demonstrate relationship between eNO levels and the presence of emphysema in HRCT or exacebations

Larger studies are need