TY - JOUR
T1 - Advances in bronchiectasis
T2 - endotyping, genetics, microbiome, and disease heterogeneity
AU - Flume, Patrick A.
AU - Chalmers, James D.
AU - Olivier, Kenneth N.
N1 - This work has been supported by the South Carolina Clinical & Translational Research Institute, with an academic home at the Medical University of South Carolina through the US National Institutes of Health (NIH) grant (UL1 TR001450) and in part by the Intramural Research Program of the US National Heart, Lung, and Blood Institute, NIH. JDC is supported by the GlaxoSmithKline/British Lung Foundation Chair of Respiratory Research.
PY - 2018/9/8
Y1 - 2018/9/8
N2 - Bronchiectasis is characterised by pathological dilation of the airways. More specifically, the radiographic demonstration of airway enlargement is the common feature of a heterogeneous set of conditions and clinical presentations. No approved therapies exist for the condition other than for bronchiectasis caused by cystic fibrosis. The heterogeneity of bronchiectasis is a major challenge in clinical practice and the main reason for difficulty in achieving endpoints in clinical trials. Recent observations of the past 2 years have improved the understanding of physicians regarding bronchiectasis, and have indicated that it might be more effective to classify patients in a different way. Patients could be categorised according to a heterogeneous group of endotypes (defined by a distinct functional or pathobiological mechanism) or by clinical phenotypes (defined by relevant and common features of the disease). In doing so, more specific therapies needed to effectively treat patients might finally be developed. Here, we describe some of the recent advances in endotyping, genetics, and disease heterogeneity of bronchiectasis including observations related to the microbiome.
AB - Bronchiectasis is characterised by pathological dilation of the airways. More specifically, the radiographic demonstration of airway enlargement is the common feature of a heterogeneous set of conditions and clinical presentations. No approved therapies exist for the condition other than for bronchiectasis caused by cystic fibrosis. The heterogeneity of bronchiectasis is a major challenge in clinical practice and the main reason for difficulty in achieving endpoints in clinical trials. Recent observations of the past 2 years have improved the understanding of physicians regarding bronchiectasis, and have indicated that it might be more effective to classify patients in a different way. Patients could be categorised according to a heterogeneous group of endotypes (defined by a distinct functional or pathobiological mechanism) or by clinical phenotypes (defined by relevant and common features of the disease). In doing so, more specific therapies needed to effectively treat patients might finally be developed. Here, we describe some of the recent advances in endotyping, genetics, and disease heterogeneity of bronchiectasis including observations related to the microbiome.
KW - Autoimmune Diseases/complications
KW - Bronchiectasis/etiology
KW - Cystic Fibrosis/complications
KW - Disease Progression
KW - Genetic Predisposition to Disease
KW - Humans
KW - Immunocompetence
KW - Microbiota
KW - Mutation
UR - http://www.scopus.com/inward/record.url?scp=85052868365&partnerID=8YFLogxK
U2 - 10.1016/S0140-6736(18)31767-7
DO - 10.1016/S0140-6736(18)31767-7
M3 - Review article
AN - SCOPUS:85052868365
SN - 0140-6736
VL - 392
SP - 880
EP - 890
JO - The Lancet
JF - The Lancet
IS - 10150
ER -