Imaging of Lung Disease Associated with Connective Tissue Disease.

There is a well-known association between the connective tissue disorders (CTDs) and lung disease. In addition to interstitial lung disease, the CTDs may affect the air spaces and pulmonary vasculature. Imaging tests are important not only in diagnosis but also in management of these complex disorders.

Identifying causation in hypersensitivity pneumonitis: a British perspective.

Background: Establishing whether patients are exposed to a 'known cause' is a key element in both the diagnostic assessment and the subsequent management of hypersensitivity pneumonitis (HP).

Objective: This study surveyed British interstitial lung disease (ILD) specialists to document current practice and opinion in relation to establishing causation in HP.

Methods: British ILD consultants (pulmonologists) were invited by email to take part in a structured questionnaire survey, to provide estimates of demographic data relating to their service and to rate their level of agreement with a series of statements.

The King's Brief Interstitial Lung Disease (KBILD) questionnaire: an updated minimal clinically important difference.

Introduction: The King's Brief Interstitial Lung Disease (KBILD) is a 15-item validated health-related quality of life (HRQOL) questionnaire. The method of scoring the KBILD has recently changed to incorporate a logit-scale transformation from one that used raw item responses, as this is potentially a more linear scale. The aim of this study was to re-evaluate the KBILD minimal clinically important difference (MCID) using the new logit -transformed scoring.

Safety and tolerability of nintedanib for the treatment of idiopathic pulmonary fibrosis in routine UK clinical practice.

http://ow.ly/4HeM30lFS67.

Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia.

Rationale: Pulmonary hypertension (PH) associated with fibrotic idiopathic interstitial pneumonia (IIP; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) confers important additional morbidity and mortality.

Objectives: To evaluate the safety and clinical efficacy of the dual endothelin-1 receptor antagonist bosentan in this patient group.

Methods: In a randomized, double-blind, placebo-controlled study, 60 patients with fibrotic IIP and right heart catheter confirmed PH were randomized 2:1 to bosentan (n = 40) or placebo (n = 20).

Alveolar proteinosis with hypersensitivity pneumonitis: a new clinical phenotype.

Background And Objective: The aim of the present study was to report the features of five patients with concurrent histopathological features of pulmonary alveolar proteinosis (PAP) and hypersensitivity pneumonitis (HP) and their high-resolution CT (HRCT) appearances.

Methods: Patients with histopathological features of both HP and PAP on surgical lung biopsy referred for tertiary review were retrospectively identified. The pathology and HRCT images were semi-quantitatively scored to evaluate the relative contribution to HP and PAP.