Emphysema: looking beyond alpha-1 antitrypsin deficiency.

Emphysema: looking beyond alpha-1 antitrypsin deficiency. Expert Rev Respir Med. 2019 Feb 14;: Authors: Janssen R, Piscaer I, Franssen FM, Wouters EF Abstract INTRODUCTION: Distinct pathologies can cause chronic obstructive pulmonary disease (COPD). Emphysema is a COPD-phenotype characterized by destruction of lung parenchyma. Alpha-1 antitrypsin deficiency (AATD) is a genetic cause of emphysema, whereas smoking is the most important risk factor of non-AATD emphysema. A general underappreciation of non-AATD emphysema has hampered progress in the field, and clinical guidelines have prohibited the use of emphysema as a diagnosis. Non-AATD emphysema, however, is far from irrelevant as it associates with dyspnea, reduced exercise capacity and relevant outcome measures. Areas covered: Mechanisms underlying enhanced tissue loss in emphysema are protease/antiprotease imbalance, increased oxidative stress, several fundamental cell biological processes such as programmed cell death and autophagy, and impaired repair mechanisms. Therapeutic options for emphysema vary from smoking cessation to lung volume reduction. Current pharmacological treatments have less favorable effects in emphysematous than in non-emphysematous COPD patients. Expert opinion: We advocate the acknowledgement of non-AATD emphysema as a clinical diagnosis and propose to end the era of bringing all pathologies that may lead to chronic airflow limitation together under the u...
Source: Expert Review of Respiratory Medicine - Category: Respiratory Medicine Tags: Expert Rev Respir Med Source Type: research