RT Journal Article
SR Electronic
T1 Bronchiectasis in patients with alpha1-antitrypsin deficiency: prevalence and characteristics
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA737
DO 10.1183/13993003.congress-2016.PA737
VO 48
IS suppl 60
A1 Rosaria Cortese
A1 Maria Chiara Mennitti
A1 Francesca Mariani
A1 Davide Piloni
A1 Stefano Aliberti
A1 Stefania Ottaviani
A1 Ilaria Ferrarotti
A1 Angelo Corsico
YR 2016
UL http://erj.ersjournals.com/content/48/suppl_60/PA737.abstract
AB RATIONALE: Bronchiectasis is one of the pulmonary manifestations of a1-antitrypsin deficiency (AATD), although its prevalence along with patients' characteristics are not very well defined. The aim of our study was to evaluate prevalence of bronchiectasis in the Italian cohort of patients with severe AATD and to correlate their presence with clinical, radiological and functional aspects.MATERIAL AND METHODS: All AATD patients come from the Italian registry of AATD, established in 1993 Demographic data, smoking history, respiratory symptoms, genotype, as well as data on respiratory function were recorded. Chest HRCTs were revised to identified patients with and without bronchiectasis.RESULTS: Among the 475 patients enrolled in the Registry, 58 (12%) had bronchiectasis at HRCT. The demographic and clinical characteristics of the group of AATD patients with bronchiectasis were compared to AATD patients with clear exclusion of bronchiectasis after chest HRTC revision (n=135). The rest of AATD patients (n= 292) did not reported or performed HRTC. Patients with brochiectasis had more comorbidities (p=0,008) and were more frequently affected by pneumonia (p= 0,024) than those without bronchiectasis. No significative correlation was found between airway disease and sex, smoking history, or FEV1. AAT genotypes are reported in the Table.genotypeBronchiectasis - N(%)No Bronchiectasis - N(%)PI*ZZ43 (74%)79 (59%)PI*SZ4 (7%)25 (18%)Other11 (19%)31 (23%)Table - Distribution of genotypes in AATD patients with and without bronchiectasisCONCLUSIONS: Presence or absence of bronchiectasis should be ascertained in patients affected by severe AATD, because of its clinical consequences.