Hallmarks of the ageing lung

S Meiners, O Eickelberg… - European Respiratory …, 2015 - Eur Respiratory Soc
Ageing is the main risk factor for major non-communicable chronic lung diseases, including
chronic obstructive pulmonary disease, most forms of lung cancer and idiopathic pulmonary …

Diseases of pulmonary surfactant homeostasis

JA Whitsett, SE Wert, TE Weaver - Annual Review of Pathology …, 2015 - annualreviews.org
Advances in physiology and biochemistry have provided fundamental insights into the role
of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory …

Exome sequencing links mutations in PARN and RTEL1 with familial pulmonary fibrosis and telomere shortening

BD Stuart, J Choi, S Zaidi, C Xing, B Holohan, R Chen… - Nature …, 2015 - nature.com
Idiopathic pulmonary fibrosis (IPF) is an age-related disease featuring progressive lung
scarring. To elucidate the molecular basis of IPF, we performed exome sequencing of …

The genetic basis of idiopathic pulmonary fibrosis

JA Kropski, TS Blackwell… - European Respiratory …, 2015 - Eur Respiratory Soc
Throughout the past decade, there have been substantial advances in understanding the
pathogenesis of idiopathic pulmonary fibrosis (IPF). Recently, several large genome-wide …

Rare Variants in RTEL1 Are Associated with Familial Interstitial Pneumonia

JD Cogan, JA Kropski, M Zhao, DB Mitchell… - American journal of …, 2015 - atsjournals.org
Rationale: Up to 20% of cases of idiopathic interstitial pneumonia cluster in families,
comprising the syndrome of familial interstitial pneumonia (FIP); however, the genetic basis …

CT scan findings of probable usual interstitial pneumonitis have a high predictive value for histologic usual interstitial pneumonitis

JH Chung, A Chawla, AL Peljto, CD Cool… - Chest, 2015 - Elsevier
The current usual interstitial pneumonitis (UIP)/idiopathic pulmonary fibrosis CT scan
classification system excludes probable UIP as a diagnostic category. We sought to …

Extensive phenotyping of individuals at risk for familial interstitial pneumonia reveals clues to the pathogenesis of interstitial lung disease

JA Kropski, JM Pritchett, DF Zoz… - American journal of …, 2015 - atsjournals.org
Rationale: Asymptomatic relatives of patients with familial interstitial pneumonia (FIP), the
inherited form of idiopathic interstitial pneumonia, carry increased risk for developing …

Epigenetics of idiopathic pulmonary fibrosis

IV Yang, DA Schwartz - Translational Research, 2015 - Elsevier
Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology.
Development of IPF is influenced by both genetic and environmental factors. Recent work by …

Idiopathic pulmonary fibrosis: an update

P Spagnolo, N Sverzellati, G Rossi, A Cavazza… - Annals of …, 2015 - Taylor & Francis
Idiopathic pulmonary fibrosis (IPF) is the most common and lethal form of idiopathic
interstitial pneumonia. The disease, which occurs primarily in middle-aged and older adults …

Lost after translation: insights from pulmonary surfactant for understanding the role of alveolar epithelial dysfunction and cellular quality control in fibrotic lung disease

S Mulugeta, SI Nureki… - American Journal of …, 2015 - journals.physiology.org
Dating back nearly 35 years ago to the Witschi hypothesis, epithelial cell dysfunction and
abnormal wound healing have reemerged as central concepts in the pathophysiology of …