Éditeur(s) : HAL CCSD European Respiratory Society
Résumé : International audience
ISSN: 0903-1936

hal-01117958
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01117958
DOI : 10.1183/09031936.00111113
PUBMED : 24687678

Éditeur(s) : HAL CCSD European Respiratory Society
Résumé : IF 8.332
International audience
The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail. In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease. The study population included 157 patients (mean±SD age 49.4±14.1 years), with a mean±SD blood eosinophil count of 7.4±6.4×109 L-1 at diagnosis. There was a mean±SD of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corticosteroid treatment, the severity of asthma increased 3-6 months before the onset of the systemic manifestations. Asthma was severe in 57%, 48%, and 56% of patients at diagnosis, at 3 years, and at the final visit, respectively. Persistent airflow obstruction was present in 38%, 30%, and 46% at diagnosis, at 3 years, and at the final visit, respectively. In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long-term management and morbidity. Copyright © 2016 ERS.
ISSN: 0903-1936

hal-01406073
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01406073
DOI : 10.1183/13993003.00097-2016
PUBMED : 27587545

Éditeur(s) : HAL CCSD European Respiratory Society
Résumé : International audience
Telomerase reverse transcriptase (TERT) or telomerase RNA (TERC) gene mutation is a major monogenic cause of pulmonary fibrosis. Sequencing of TERT/TERC genes is proposed to patients with familial pulmonary fibrosis. Little is known about the possible predictors of this mutation and its impact on prognosis. We retrospectively analysed all the genetic diagnoses made between 2007-2014 in patients with pulmonary fibrosis. We evaluated the prevalence of TERT/TERC disease-Associated variant (DAV), factors associated with a DAV, and the impact of the DAV on survival. 237 patients with pulmonary fibrosis (153 with familial pulmonary fibrosis, 84 with telomere syndrome features without familial pulmonary fibrosis) were tested for TERT/TERC DAV. DAV was diagnosed in 40 patients (16.8%), including five with non-idiopathic interstitial pneumonia. Prevalence of TERT/TERC DAV did not significantly differ between patients with familial pulmonary fibrosis or with only telomere syndrome features (18.2% versus 16.4%). Young age, red blood cell macrocytosis, and low platelet count were associated with the presence of DAV; the probability of DAV was increased for patients 40-60 years. Transplant-free survival was lower with than without TERT/TERC DAV (4.2 versus 7.2 years; p=0.046). TERT/TERC DAV were associated with specific clinical and biological features and reduced transplantfree survival. ©ERS 2016.
ISSN: 0903-1936

hal-01467555
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01467555
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01467555/document
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01467555/file/Prevalence%20and%20characteristics%20of%20TERT.pdf
DOI : 10.1183/13993003.02115-2015

Éditeur(s) : HAL CCSD European Respiratory Society
Résumé : International audience
[No abstract available]
ISSN: 0903-1936

hal-01406071
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01406071
DOI : 10.1183/13993003.00252-2016
PUBMED : 27799394

Éditeur(s) : HAL CCSD European Respiratory Society
Résumé : International audience
There are conflicting data regarding the magnitude and determinants of chronic obstructive pulmonary disease (COPD) risk in farmers.In a cross-sectional study of 917 nonfarming working controls and 3787 farmers aged 40-75 years, we assessed respiratory symptoms, tobacco exposure, job history (without direct exposure measurement) and lung function. COPD was defined by the Global Initiative for Chronic Obstructive Lung Disease (GOLD) criterion (post-bronchodilator forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) extless0.70) and by the Quanjer reference equation (post-bronchodilator FEV1/FVC extlesslower limit of normal (LLN)).The prevalence (95% CI) of COPD according to the GOLD criterion was 5.1% (4.4-5.8%) and 2.9% (1.8-4.0%) in farmers and controls, respectively (p=0.005), and 3.1% (2.5-3.6%) and 1.5% (0.7-2.3%), respectively, for the LLN criterion (p extless0.01). For both COPD criteria after adjustment for age, sex and smoking status, COPD prevalence was similar in controls and crop farmers. Compared to controls, four job categories had a higher prevalence of COPD according to the GOLD criterion, namely, cattle breeders, swine breeders, poultry breeders and breeders of two or more livestock types. Among cattle breeders, only those from Franche-Comté had higher prevalence of COPD according to both GOLD and LLN criteria.The prevalence of COPD in farmers is higher than in nonfarming working controls, and depends on the farming activity, the region and the criterion used to define COPD
ISSN: 0903-1936

hal-01216204
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01216204
DOI : 10.1183/13993003.00153-2015
PUBMED : 26453630