Impact of genetic factors on fibrosing interstitial lung diseases. Incidence and clinical presentation in adults

R Borie, C Kannengiesser, C Dupin, MP Debray… - La Presse Médicale, 2020 - Elsevier
At least 10% of patients with pulmonary fibrosis, whether idiopathic or secondary, present
heritable pulmonary fibrosis suspected on familial aggregation of pulmonary fibrosis …

Management of suspected monogenic lung fibrosis in a specialised centre

R Borie, C Kannengiesser… - European …, 2017 - Eur Respiratory Soc
At least 10% of patients with interstitial lung disease present monogenic lung fibrosis
suspected on familial aggregation of pulmonary fibrosis, specific syndromes or early age of …

Syndromic genetic causes of pulmonary fibrosis

R Borie, I Ba, MP Debray, C Kannengiesser… - Current Opinion in … - journals.lww.com
The genetic causes of interstitial lung disease (ILD) are predominantly evidenced in
telomere related genes (TRG) and surfactant related genes (SRG). SRG are associated with …

Genetics of pulmonary fibrosis

TN Adams, CK Garcia - Idiopathic Pulmonary Fibrosis: A Comprehensive …, 2019 - Springer
Genetic sequence variants have been linked to familial pulmonary fibrosis (FPF). Many of
the same genetic mechanisms implicated in FPF are relevant to sporadic interstitial lung …

[HTML][HTML] Genetic susceptibility and interstitial lung diseases

SK Mathai, DA Schwartz, LA Warg - Current opinion in pulmonary …, 2014 - ncbi.nlm.nih.gov
The inherited risk for pulmonary fibrosis is substantial, and recent data suggests that genetic
risk for familial and sporadic forms of the disease are similar. Further characterization of the …

Familial pulmonary fibrosis: genetic features and clinical implications

D Zhang, CA Newton - Chest, 2021 - Elsevier
Pulmonary fibrosis comprises a wide range of fibrotic lung diseases with unknown
pathogenesis and poor prognosis. Familial pulmonary fibrosis (FPF) represents a unique …

Molecular mechanisms in progressive idiopathic pulmonary fibrosis

MP Steele, DA Schwartz - Annual review of medicine, 2013 - annualreviews.org
There is clear evidence that environmental exposures and genetic predisposition contribute
to the pathogenesis of idiopathic pulmonary fibrosis (IPF). Cigarette smoking increases the …

Genetic determinants of pulmonary fibrosis: evolving concepts

P Spagnolo, J Grunewald, RM Du Bois - The Lancet Respiratory …, 2014 - thelancet.com
Interstitial lung diseases encompass a wide range of diffuse lung disorders that are often
complicated by the development of pulmonary fibrosis and that can occur in isolation or in …

Familial pulmonary fibrosis

R Borie, C Kannengiesser, N Nathan, L Tabèze… - Revue des maladies …, 2015 - Elsevier
The occurrence of pulmonary fibrosis in numerous individuals from the same family
suggests a genetic cause for the disease. During the last 10 years, mutations involving …

[HTML][HTML] Familial idiopathic pulmonary fibrosis in a young female

SS Sritharan, ME Gajewska, ABS Skytte… - … medicine case reports, 2018 - Elsevier
Idiopathic pulmonary fibrosis is a chronic interstitial lung disease of unknown cause. In the
past years there have been observations of clustering of pulmonary fibrosis in families …