A new variant in the gene: diverse clinical phenotypes and expression in the lung.

Introduction: Pulmonary fibrosis is a severe disease which can be familial. A genetic cause can only be found in ∼40% of families. Searching for shared novel genetic variants may aid the discovery of new genetic causes of disease.

Outcomes in pulmonary sarcoidosis: results of a newly implemented prednisone protocol.

Background and aim Prednisone is used as first-line therapy for patients with pulmonary sarcoidosis. There is however no clear association between prednisone dose and FVC change in patients with pulmonary sarcoidosis. In order to improve our standard of care we introduced a more conservative prednisone protocol.

Patterns of healthcare resource utilization in patients with sarcoidosis: a cross-sectional study.

Background: Limited data are available on healthcare resource use and costs in patients with sarcoidosis.

Objectives: The primary aim of this study was to describe cost-drivers of the top 1% and top ≥1-5% high-cost patients with sarcoidosis. The secondary aim was to compare costs of patients with and without fatigue complaints and to compare comorbidities.

Results of the standard set for pulmonary sarcoidosis: feasibility and multicentre outcomes.

Our study presents findings on a previously developed standard set of clinical outcome data for pulmonary sarcoidosis patients. We aimed to assess whether changes in outcome varied between the different centres and to evaluate the feasibility of collecting the standard set retrospectively. This retrospective observational comparative benchmark study included six interstitial lung disease expert centres based in the Netherlands, Belgium, the UK and the USA.

Longitudinal prediction of outcome in idiopathic pulmonary fibrosis using automated CT analysis.

http://bit.ly/2M7DfKS

First patient-centred set of outcomes for pulmonary sarcoidosis: a multicentre initiative.

Introduction: Routine and international comparison of clinical outcomes enabling identification of best practices for patients with pulmonary sarcoidosis is lacking. The aim of this study was to develop a standard set of outcome measures for pulmonary sarcoidosis, using the value-based healthcare principles.

Methods: Six expert clinics for interstitial lung diseases in four countries participated in a consensus-driven RAND-modified Delphi study.

Predicting outcomes in rheumatoid arthritis related interstitial lung disease.

The aim of this study was to compare radiology-based prediction models in rheumatoid arthritis-related interstitial lung disease (RAILD) to identify patients with a progressive fibrosis phenotype.RAILD patients had computed tomography (CT) scans scored visually and using CALIPER and forced vital capacity (FVC) measurements. Outcomes were evaluated using three techniques, as follows.

Predicting Outcomes in Idiopathic Pulmonary Fibrosis Using Automated Computed Tomographic Analysis.

Rationale: Quantitative computed tomographic (CT) measures of baseline disease severity might identify patients with idiopathic pulmonary fibrosis (IPF) with an increased mortality risk. We evaluated whether quantitative CT variables could act as a cohort enrichment tool in future IPF drug trials.

Objectives: To determine whether computer-derived CT measures, specifically measures of pulmonary vessel-related structures (VRSs), can better predict functional decline and survival in IPF and reduce requisite sample sizes in drug trial populations.

Efficacy and safety of infliximab biosimilar Inflectra in severe sarcoidosis.

Background: Infliximab, a monoclonal antibody against tumor necrosis factor alpha (TNF-α) is effective third-line therapy in severe sarcoidosis. The originator product of Infliximab, Remicade, is expensive, limiting universal access. Recently, a less expensive biosimilar of infliximab, Inflectra, has become available, but the efficacy and tolerability has not been studied in sarcoidosis.

[Idiopathic pulmonary fibrosis: new insights].

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease of unknown cause. IPF has a poor prognosis with a mean survival of 2 to 5 years after diagnosis. The diagnostic process is often complex and demands a multidisciplinary approach.