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Article
Open AccessPhenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease
Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports...
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Article
Open AccessRole of the occupational disease consultant in the multidisciplinary discussion of interstitial lung diseases
Diffuse interstitial lung diseases (ILD) constitute a heterogeneous group of conditions with complex etiological diagnoses requiring a multidisciplinary approach. Much is still unknown about them, particularly...
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Article
Open AccessAutomatic quantitative computed tomography measurement of longitudinal lung volume loss in interstitial lung diseases
To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and exp...
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Article
Open AccessNintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis
Pleuroparenchymal fibroelastosis (PPFE) has a variable disease course with dismal prognosis in the majority of patients with no validated drug therapy. This study is to evaluate the effect of nintedanib in pat...
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Article
Open AccessEstimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)
There is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-ILD) in France. ...
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Article
Open AccessEstimation of the Prevalence of Progressive Fibrosing Interstitial Lung Diseases: Systematic Literature Review and Data from a Physician Survey
Some patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis exhibit a progressive clinical phenotype. These chronic progressive fibrosing ILDs have a variety of underlying dis...