TY - JOUR
T1 - Increased circulating desmosine and age-dependent elastinolysis in idiopathic pulmonary fibrosis
AU - de Brouwer, Bart
AU - Drent, Marjolein
AU - van den Ouweland, Jody M.W.
AU - Wijnen, Petal A.
AU - van Moorsel, Coline H.M.
AU - Bekers, Otto
AU - Grutters, Jan C.
AU - White, Eric S.
AU - Janssen, Rob
N1 - Funding Information:
This study was supported by two grants from Roche.
Publisher Copyright:
© 2018 The Author(s).
PY - 2018/3/20
Y1 - 2018/3/20
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85044247802&partnerID=8YFLogxK
U2 - 10.1186/s12931-018-0747-6
DO - 10.1186/s12931-018-0747-6
M3 - Comment/Letter to the editor
C2 - 29558926
AN - SCOPUS:85044247802
SN - 1465-9921
VL - 19
SP - 45
JO - Respiratory research
JF - Respiratory research
IS - 1
M1 - 45
ER -