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Respir Res. 2018 Mar 20;19(1):45. doi: 10.1186/s12931-018-0747-6.

Increased circulating desmosine and age-dependent elastinolysis in idiopathic pulmonary fibrosis.

Author information

1
Department of Pulmonary Medicine, Canisius-Wilhelmina Hospital, Weg door Jonkerbos 100, 6532, SZ, Nijmegen, The Netherlands. b.debrouwer@cwz.nl.
2
Center of Interstitial Lung Diseases, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands.
3
Department of Pharmacology and Toxicology, FHML, Maastricht University, Maastricht, The Netherlands.
4
Department of Clinical Chemistry, Canisius-Wilhelmina Hospital, Nijmegen, The Netherlands.
5
Department of Clinical Chemistry, Central Diagnostic Laboratory, Maastricht University Medical Center+, Maastricht, The Netherlands.
6
Division of Pulmonary and Critical Care Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA.
7
Department of Pulmonary Medicine, Canisius-Wilhelmina Hospital, Weg door Jonkerbos 100, 6532, SZ, Nijmegen, The Netherlands.

Abstract

Although chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) seem to be opposite entities from a clinical perspective, common initial pathogenic steps have been suggested in both lung diseases. Emphysema is caused by an elastase/anti-elastase imbalance leading to accelerated elastin degradation. Elastinolysis is however, also accelerated in the IPF patients' lungs. The amino acids desmosine and isodesmosine (DES) are unique to elastin. During the degradation process, elastases liberate DES from elastin fibers. Blood DES levels consequently reflect the rate of systemic elastinolysis and are increased in COPD. This is the first report describing elevated DES levels in IPF patients. We also demonstrated that the age-related increment of DES concentrations is enhanced in IPF. Our current study suggests that elastinolysis is a shared pathogenic step in both COPD and IPF. Further investigation is required to establish the relevance of accelerated elastin degradation in IPF and to determine whether decelerating this process leads to slower progression of lung fibrosis and better survival for patients with IPF.

PMID:
29558926
PMCID:
PMC5859529
DOI:
10.1186/s12931-018-0747-6
[Indexed for MEDLINE]
Free PMC Article

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