PMID- 30761929
OWN - NLM
STAT- MEDLINE
DCOM- 20200610
LR  - 20200610
IS  - 1747-6356 (Electronic)
IS  - 1747-6348 (Linking)
VI  - 13
IP  - 4
DP  - 2019 Apr
TI  - Emphysema: looking beyond alpha-1 antitrypsin deficiency.
PG  - 381-397
LID - 10.1080/17476348.2019.1580575 [doi]
AB  - 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.
FAU - Janssen, Rob
AU  - Janssen R
AD  - a Department of Pulmonary Medicine , Canisius-Wilhelmina Hospital , Nijmegen , 
      The Netherlands.
FAU - Piscaer, Ianthe
AU  - Piscaer I
AD  - b Department of Respiratory Medicine , Maastricht University Medical Center , 
      Maastricht , The Netherlands.
AD  - c NUTRIM School of Nutrition and Translational Research in Metabolism , 
      Maastricht University Medical Center , Maastricht , The Netherlands.
FAU - Franssen, Frits M E
AU  - Franssen FME
AD  - b Department of Respiratory Medicine , Maastricht University Medical Center , 
      Maastricht , The Netherlands.
AD  - c NUTRIM School of Nutrition and Translational Research in Metabolism , 
      Maastricht University Medical Center , Maastricht , The Netherlands.
AD  - d CIRO , Center of Expertise for Chronic Organ Failure , Horn , The Netherlands.
FAU - Wouters, Emiel F M
AU  - Wouters EFM
AD  - b Department of Respiratory Medicine , Maastricht University Medical Center , 
      Maastricht , The Netherlands.
AD  - c NUTRIM School of Nutrition and Translational Research in Metabolism , 
      Maastricht University Medical Center , Maastricht , The Netherlands.
AD  - d CIRO , Center of Expertise for Chronic Organ Failure , Horn , The Netherlands.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20190222
PL  - England
TA  - Expert Rev Respir Med
JT  - Expert review of respiratory medicine
JID - 101278196
SB  - IM
MH  - Animals
MH  - Female
MH  - Humans
MH  - Male
MH  - Pneumonectomy
MH  - Pulmonary Disease, Chronic Obstructive/*etiology
MH  - Pulmonary Emphysema/complications/diagnosis/pathology/*therapy
MH  - Smoking/adverse effects
MH  - Smoking Cessation
MH  - alpha 1-Antitrypsin Deficiency/complications
OTO - NOTNLM
OT  - COPD
OT  - Emphysema
OT  - antiproteases
OT  - centrilobular emphysema
OT  - elastin degradation
OT  - elastin repair
OT  - non-alpha1 antitrypsin emphysema
OT  - panlobular emphysema
OT  - paraseptal emphysema
OT  - proteases
EDAT- 2019/02/15 06:00
MHDA- 2020/06/11 06:00
CRDT- 2019/02/15 06:00
PHST- 2019/02/15 06:00 [pubmed]
PHST- 2020/06/11 06:00 [medline]
PHST- 2019/02/15 06:00 [entrez]
AID - 10.1080/17476348.2019.1580575 [doi]
PST - ppublish
SO  - Expert Rev Respir Med. 2019 Apr;13(4):381-397. doi: 
      10.1080/17476348.2019.1580575. Epub 2019 Feb 22.