Interstitial lung disease: new challenges and evolving phenotypes

Cottin, Vincent

doi:10.1183/09059180.00003910

Cited by 4 publications

(2 citation statements)

References 34 publications

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“…[ 2 ] Subsequently, the same disease characteristics were described as an atypical phenotype of idiopathic interstitial pneumonias. [ 3 ] Cases of CPFE are usually tobacco smokers. Hence, CPFE has been proposed as a new clinical entity in smokers or a different clinical phenotype in smokers developing interstitial pulmonary fibrosis (IPF).…”

Section: Introductionmentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema in a tyre industry worker

Karkhanis

Joshi

2012

Lung India

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We report a case of combined pulmonary fibrosis and emphysema (CPFE) with severe pulmonary hypertension in a 46-year-old man, nonsmoker, tyre industry worker. CPFE is commonly reported to be associated with tobacco smoking. This case highlights the possible role of environmental dust exposure (talc) in the pathogenesis of the disease and confirms the clinical characteristics of CPFE described in previous studies.

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Section: Introductionmentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema in a tyre industry worker

Karkhanis

Joshi

2012

Lung India

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“…In these patients, the diffusing capacity of the lungs for carbon monoxide (DLCO) is severely restricted (in most cases to 20-45% of the predicted reference range), while lung volumes are normal or slightly changed and severe airway obstructions are absent. The mortality rate for this disease is high and is also strongly associated with elevated pulmonary pressure [18,22,23].…”

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confidence: 99%

Pulmonary hypertension due to lung diseases: Updated recommendations from the Cologne Consensus Conference 2018

Olschewski

Behr

Bremer

et al. 2018

International Journal of Cardiology

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The 2015 European Guidelines on Pulmonary Hypertension did not only cover pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) associated with chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists, phosphodiesterase‑5 inhibitors, sGC stimulators) have not been sufficiently investigated in other forms of PH. Therefore, the European Guidelines do not recommend the use of these drugs in patients with chronic lung disease and PH. This recommendation, however, is not always in agreement with medical ethics as physicians sometimes feel inclined to treat other forms of PH which may affect quality of life and survival of these patients in a similar manner. To this end, it is crucial to consider the severity of both PH and the underlying lung disease. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Paediatric Cardiology (DGPK) was held in Cologne, Germany, to discuss open and controversial issues surrounding the practical implementation of the European Guidelines. Several working groups were created, one of which was dedicated to the diagnosis and treatment of PH in patients with chronic lung disease. The 2018 updated recommendations of this working group are summarized in the present paper.

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