TY - JOUR T1 - Why do some adults with PiMZ α<sub>1</sub>-antitrypsin develop bronchiectasis? JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00021-2016 VL - 2 IS - 2 SP - 00021-2016 AU - Nupur Aggarwal AU - Beatriz Martinez Delgado AU - Sandeep Salipalli AU - Nerea Matamala AU - Jessica Rademacher AU - Nicolaus Schwerk AU - Tobias Welte AU - Sabina Janciauskiene AU - Felix C. Ringshausen Y1 - 2016/04/01 UR - http://openres.ersjournals.com/content/2/2/00021-2016.abstract N2 - Bronchiectasis is an aetiologically heterogeneous, chronic and often progressive disease resulting in the permanent dilatation of one or more bronchi or bronchioli. Several reports indicated an association among inherited α1-antitrypsin deficiency (α1-ATD), pulmonary infections and bronchiectasis, with a frequency up to 10% [1, 2]. It has been postulated that in α1-ATD individuals repeated episodes of ordinary bronchitis, of whatever cause, may lead to the development of bronchiectasis [3]. Most reported α1-ATD cases with bronchiectasis are elderly homozygous PiZZ (Glu342Lys) smokers with emphysema. There are only a few historical case reports with bronchiectasis and α1-ATD in the absence of emphysema [4, 5]. Whether there is an increased risk of pulmonary diseases, including bronchiectasis, in heterozygous PiMZ α1-ATD carriers is a matter of debate [6, 7].Recurrent infections of the upper airways in early life may be a warning sign of inherited α1-antitrypsin deficiency http://ow.ly/iJsF300kbyV ER -