Emphysema: looking beyond alpha-1 antitrypsin deficiency

Research output: Contribution to journalReview articlepeer-review

15 Citations (Web of Science)

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 acknowledgment 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 umbrella-term of COPD. Decelerating proteolysis and restoring damage should be main targets in emphysema. Vitamin A/K, hyaluronan, copper and roflumilast are promising candidates.

Original languageEnglish
Pages (from-to)381-397
Number of pages17
JournalExpert Review of Respiratory Medicine
Volume13
Issue number4
DOIs
Publication statusPublished - 3 Apr 2019

Keywords

  • Emphysema
  • centrilobular emphysema
  • panlobular emphysema
  • paraseptal emphysema
  • non-alpha1 antitrypsin emphysema
  • elastin degradation
  • elastin repair
  • proteases
  • antiproteases
  • COPD
  • OBSTRUCTIVE PULMONARY-DISEASE
  • LUNG-VOLUME-REDUCTION
  • AIR-FLOW OBSTRUCTION
  • CORONARY-ARTERY CALCIFICATION
  • SMOKE-INDUCED EMPHYSEMA
  • SMALL AIRWAYS DISEASE
  • TRANS-RETINOIC ACID
  • CIGARETTE-SMOKE
  • COMPUTED-TOMOGRAPHY
  • CENTRILOBULAR EMPHYSEMA

Cite this