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Respir Investig 2019 Apr 16. Epub 2019 Apr 16.

Department of Respiratory Medicine and Allergology, Kindai University, Faculty of Medicine, Osakasayama, Osaka, Japan. Electronic address:

Background: The characteristics and significance of respiratory-related hospitalization in patients with idiopathic pulmonary fibrosis (IPF) in Asian countries remain unknown. The purpose of this study was to define the characteristics of respiratory-related hospitalization and to inspect the relationship between respiratory-related hospitalization and subsequent survival in patients with IPF in Japanese general practice.

Methods: Patients with IPF who underwent clinical evaluation between February 2008 and August 2017 were screened. Read More

Respir Investig 2019 Apr 16. Epub 2019 Apr 16.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address:

Background: Some cases of idiopathic fibrotic nonspecific interstitial pneumonia (f-NSIP) show a progressive course that is similar to that of idiopathic pulmonary fibrosis. However, it is difficult to predict poor patient outcomes. This study aimed to evaluate whether serial changes in serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) can predict disease progression. Read More

J Biol Chem 2019 Apr 18. Epub 2019 Apr 18.

Department of Pathology, University of Michigan.

Mutations in the genes encoding telomerase reverse transcriptase (TERT) and telomerase's RNA components, as well as shortened telomeres, are risk factors for idiopathic pulmonary fibrosis (IPF), wherein repetitive injury to the alveolar epithelium is considered to be a key factor in pathogenesis. Given the importance of TERT in stem cells, we hypothesized that TERT plays an important role in epithelial repair and that its deficiency results in exacerbation of fibrosis by impairing this repair/regenerative process. To evaluate the role of TERT in epithelial cells, we generated type II alveolar epithelial cell (AECII) specific TERT conditional knockout mice (SPC-Tert cKO) by crossing floxed TERT mice with inducible SPC-driven Cre mice. Read More

BMC Pulm Med 2019 Apr 18;19(1):81. Epub 2019 Apr 18.

Division of Pulmonology, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.

Background: In many clinical disorders, there is a relationship between the ratio of the diameter of the main pulmonary artery (mPA) to that of the aorta (Ao) on chest computed tomography (CT). The aim of this study was to determine if the mPA/Ao ratio at diagnosis is associated with the clinical characteristics and outcomes in patients with idiopathic pulmonary fibrosis (IPF).

Methods: We retrospectively reviewed the diameters of the pulmonary artery and aorta on chest CT, clinical characteristics, and results of other examinations in 303 patients at the time of initial diagnosis of IPF at our tertiary care center between 2011 and 2015. Read More

Cell Signal 2019 Apr 15;60:65-80. Epub 2019 Apr 15.

Department of Biochemistry, Universities of Giessen and Marburg Lung Center, Giessen, Germany. Electronic address:

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive extracellular matrix deposition in the lung parenchyma leading to the destruction of lung structure, respiratory failure and premature death. Recent studies revealed that the pathogenesis of IPF is associated with alterations in the synthesis and the activity of lipids, lipid regulating proteins and cell membrane lipid transporters and receptors in different lung cells. Furthermore, deregulated lipid metabolism was found to contribute to the profibrotic phenotypes of lung fibroblasts and alveolar epithelial cells. Read More

PLoS One 2019 18;14(4):e0215303. Epub 2019 Apr 18.

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Background: The clinical course of IPF varies. This study sought to identify phenotyping with quantitative computed tomography (CT) fibrosis and emphysema features using a cluster analysis and to assess prognostic impact among identified clusters in patient with idiopathic pulmonary fibrosis (IPF). Furthermore, we evaluated the impact of fibrosis and emphysema on lung function with development of a descriptive formula. Read More

PLoS One 2019 18;14(4):e0215565. Epub 2019 Apr 18.

Translational Bioinformatics, Translational Medicine, Bristol-Myers Squibb, Princeton, NJ, United States of America.

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease affecting ~5 million people globally. We have constructed an accurate model of IPF disease status using elastic net regularized regression on clinical gene expression data. Leveraging whole transcriptome microarray data from 230 IPF and 89 control samples from Yang et al. Read More

Exp Biol Med (Maywood) 2019 Apr 18:1535370219843827. Epub 2019 Apr 18.

1 Department of Surgical Intensive Care Unit, Beijing An Zhen Hospital, Capital Medical University, Beijing 100029, China.

Impact Statement: Our work focused on alveolar epithelial cells (AECs)-derived type-2 cytokine (interleukin [IL]-25) in the pathogenesis of idiopathic pulmonary fibrosis (IPF). We showed that IL-25 and IL-17BR (IL-25's receptor) is upregulated in lung tissues (especially in AECs and lung fibroblasts) of IPF patients and contributes to lung fibrosis by directly activating lung fibroblasts and modulating epithelial-mesenchymal transition (EMT) of AECs. We suggest that IL-25 may be one of the master switches hidden in the milieu of abnormal epithelial-mesenchymal crosstalk. Read More

Hum Mol Genet 2019 Apr 1. Epub 2019 Apr 1.

Channing Division of Network Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.

Chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) are two pathologically distinct chronic lung diseases that are associated with cigarette smoking. Genetic studies have identified shared loci for COPD and IPF, including several loci with opposite directions of effect. The existence of additional shared genetic loci, as well as potential shared pathobiological mechanisms between the two diseases at the molecular level, remains to be explored. Read More

Can J Respir Ther 2018 1;54(2):35-40. Epub 2018 Aug 1.

Pulmonology Department, Centro Hospitalar São João, Oporto, Portugal.

Introduction: Recent studies have reported a high prevalence of obstructive sleep apnoea (OSA) among patients with diffuse parenchymal lung disease (DPLD), mainly idiopathic pulmonary fibrosis (IPF). Effective OSA treatment appears to have a positive impact on morbidity and mortality in these patients. However, poor compliance to positive airway pressure (PAP) treatment in fibrotic DPLD patients has been reported. Read More

Orphanet J Rare Dis 2019 Apr 17;14(1):82. Epub 2019 Apr 17.

Instituto de Investigaciones Biomedicas CSIC/UAM, IDIPaz, Arturo Duperier, 4, 28029, Madrid, Spain.

Background: Telomeres are nucleoprotein structures present at the terminal region of the chromosomes. Mutations in genes coding for proteins involved in telomere maintenance are causative of a number of disorders known as telomeropathies. The genetic origin of these diseases is heterogeneous and has not been determined for a significant proportion of patients. Read More

Clin Gastroenterol Hepatol 2019 Apr 14. Epub 2019 Apr 14.

IBD Centre, Humanitas Clinical and Research Centre, Rozzano, Milan, Italy; Department of Biomedical Sciences, Humanitas University, Rozzano, Milan, Italy. Electronic address:

Fibrotic strictures occur in 30% of patients with Crohn's disease (CD). However, there are no therapeutic agents that prevent or reverse fibrotic strictures. Strictures are treated by endoscopic dilatation procedures and surgical procedures, but there are high rates of recurrence. Read More

Ann Am Thorac Soc 2019 Apr 16. Epub 2019 Apr 16.

Icahn School of Medicine at Mount Sinai, Medicine, New York, New York, United States.

Rationale: Characteristics and outcomes of lung cancer in patients with idiopathic pulmonary fibrosis (IPF) in the United States (US) remain understudied.

Objectives: To determine the tumor characteristics and survival of IPF patients with non-small cell lung cancer (NSCLC) using US population-based data.

Methods: We selected Medicare beneficiaries from the Surveillance, Epidemiology and End Results registry with histologically confirmed NSCLC diagnosed between 2007-2011. Read More

Sci Rep 2019 Apr 15;9(1):6020. Epub 2019 Apr 15.

Department of Pharmacy and Pharmacology, University of Bath, Claverton Down, Bath, BA2 7AY, United Kingdom.

Phenotypic changes in lung fibroblasts are believed to contribute to the development of Idiopathic Pulmonary Fibrosis (IPF), a progressive and fatal lung disease. Long intergenic non-coding RNAs (lincRNAs) have been identified as novel regulators of gene expression and protein activity. In non-stimulated cells, we observed reduced proliferation and inflammation but no difference in the fibrotic response of IPF fibroblasts. Read More

Mol Cell Biol 2019 Apr 15. Epub 2019 Apr 15.

Department of Respiration, The First Affiliated Hospital of Zhengzhou University, Zhengzhou.

Long non-coding RNAs (lncRNAs) are involved in various human diseases. Recently, H19 is reported to be upregulated in fibrotic rat lung and play a stimulative role in bleomycin (BLM)-induced pulmonary fibrosis in mice. However, its expression in human fibrotic lung tissues and action mechanism remain unclear. Read More

J Pharm Pract 2019 Apr 15:897190019841731. Epub 2019 Apr 15.

3 Pharmacy Practice and Medicine, Creighton University School of Pharmacy and Health Professions, Omaha, NE, USA.

Introduction: Diffuse alveolar hemorrhage (DAH) is bleeding into the alveolar space of the lungs. Pirfenidone is an antifibrotic agent that is approved for the treatment of idiopathic pulmonary fibrosis (IPF). The most commonly reported side effects include gastrointestinal and skin-related events. Read More

Am J Respir Crit Care Med 2019 Apr 15. Epub 2019 Apr 15.

Cornell Medical College, New York, New York, United States.

The approval of nintedanib and pirfenidone for treatment of idiopathic pulmonary fibrosis has introduced complexity into the design of clinical trials for new drugs on many levels. Placebo-controlled trials excluding background therapy may have issues with feasibility. Those that allow background therapy must consider the possibility of drug-drug interactions with existing therapies, as well as the narrower therapeutic window available to demonstrate incremental slowing of lung function decline with a new agent. Read More

Biomed Res Int 2019 7;2019:1345402. Epub 2019 Mar 7.

Pulmonary Cell Research, Department of Biomedicine and Pneumology, Department of Internal Medicine, University Hospital and University of Basel, Switzerland.

Remodelling of the peripheral lung tissue and fibrotic foci are the main pathologies of idiopathic pulmonary fibrosis (IPF), a disease that is difficult to treat. TGF- activation of peripheral lung fibroblasts is indicated as the major cause of tissue remodelling in IPF and is resulting in fibroblast hyperplasia and deposition of extracellular matrix. Soluble guanylate cyclase (sGC) stimulators combined with cyclic AMP (cAMP) activators have been reported to reduce proliferation and matrix deposition in other conditions than IPF. Read More

Transl Res 2019 Mar 26. Epub 2019 Mar 26.

Department of Inflammation and Immunity, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio; Department of Gastroenterology, Hepatology and Nutrition, Digestive Diseases and Surgery Institute, Cleveland Clinic Foundation, Cleveland, Ohio. Electronic address:

Crohn's disease (CD) is a chronic inflammatory disease, which could affect any part of the gastrointestinal tract. A severe complication of CD is fibrosis-associated strictures, which can cause bowel obstruction. Unfortunately, there is no specific antifibrotic therapy available. Read More

Respir Investig 2019 Apr 10. Epub 2019 Apr 10.

Department of Respiratory Medicine, Toho University Omori Medical Center, Japan.

Background: Physicians have few opportunities to perform surgical lung biopsy (SLB) to diagnose idiopathic pleuroparenchymal fibroelastosis (IPPFE). Therefore, diagnostic criteria for IPPFE that do not require SLB must be established. Herein, we propose diagnostic criteria for IPPFE with and without SLB. Read More

Respir Med 2019 Apr 1. Epub 2019 Apr 1.

Center for Interstitial Lung Diseases, Division of Pulmonary and Critical Care and Sleep Medicine, University of Washington Medical Center, Seattle, WA, 98195, USA. Electronic address:

BMC Pulm Med 2019 Apr 11;19(1):78. Epub 2019 Apr 11.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Tomioka-Higashi 6-16-1, Kanazawa-ku, Yokohama, 236-0051, Japan.

Background: Current clinical practice guidelines for idiopathic pulmonary fibrosis (IPF) conditionally recommend use of pirfenidone and nintedanib. However, an optimal treatment sequence has not been established, and the data of treatment sequence from pirfenidone to nintedanib are limited. This study aimed to evaluate safety, tolerability and efficacy of nintedanib switched from pirfenidone in patients with IPF. Read More

Int Immunopharmacol 2019 Apr 8;72:112-123. Epub 2019 Apr 8.

Univ Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail) - UMR_S 1085, F-35000 Rennes, France. Electronic address:

The tyrosine kinase inhibitor, Nintedanib (NTD), has been approved for the treatment of idiopathic pulmonary fibrosis (IPF). In cell-free systems, NTD was recently shown to inhibit kinase activity of the human recombinant colony-stimulating factor 1 (CSF1) receptor (CSF1R) which mediates major functions of pulmonary macrophages. In the present study, we have investigated the effects of NTD on the phenotype of human monocyte-derived macrophages controlled by CSF1 in order to identify its anti-inflammatory properties via CSF1R inhibition. Read More

Chronic Obstr Pulm Dis 2019 Apr 9;6(2). Epub 2019 Apr 9.

Oregon Health Sciences University Hospital, Portland.

Objective: This study compares and contrasts the clinical features of non-cystic fibrosis bronchiectasis with 3 uncommon disorders known to be associated with bronchiectasis but with distinctly different underlying defined pathophysiologic derangements, namely severe alpha-1 antitrypsin deficiency (AATD), common variable immunodeficiency (CVI) and primary ciliary dyskinesia (PCD).

Methods: The Bronchiectasis Research Registry provides a central database for studying patients with non-cystic fibrosis bronchiectasis. This report consists of information from 13 U. Read More

Am J Respir Crit Care Med 2019 Apr 11. Epub 2019 Apr 11.

Nottingham University, Nottingham, United Kingdom of Great Britain and Northern Ireland.

Am J Respir Cell Mol Biol 2019 Apr 11. Epub 2019 Apr 11.

University of Chicago, 2462, Chicago, Illinois, United States ;

Idiopathic pulmonary fibrosis is characterized by the TGF-β-dependent differentiation of lung fibroblasts into myofibroblasts, leading to excessive deposition of extracellular matrix proteins, which distort lung architecture and function. Metabolic reprogramming in myofibroblasts is emerging as an important mechanism in the pathogenesis IPF and recent evidence suggests that glutamine metabolism is required in myofibroblasts although the exact role of glutamine in myofibroblasts is unclear. Here we demonstrate that glutamine, and its conversion to glutamate by glutaminase is required for TGF-β-induced collagen protein production in lung fibroblasts. Read More

ERJ Open Res 2019 Apr 8;5(2). Epub 2019 Apr 8.

FormAction Santé, Pérenchies, France.

Background: Few studies have examined the benefits of pulmonary rehabilitation in patients with fibrotic idiopathic pulmonary pneumonia (f-IIP). Here, we report the results of an observational study in routine clinical practice of home-based pulmonary rehabilitation for f-IIP patients.

Methods: A total of 112 consecutive patients (61 with idiopathic pulmonary fibrosis and 51 with fibrotic nonspecific interstitial pneumonitis) were enrolled, of whom 65 had mild-to-moderate disease (forced vital capacity (FVC) ≥50% predicted and diffusing capacity of the lung for carbon monoxide () ≥30% predicted) and 47 had severe disease (FVC <50% predicted and/or <30% predicted). Read More

J Pharmacol Exp Ther 2019 Apr 10. Epub 2019 Apr 10.

Asahi Kasei Pharma Co. Ltd.

AK106-001616 is a novel, potent, and selective inhibitor of the cytosolic phospholipase A2 (cPLA2) enzyme. Unlike traditional non-steroidal anti-inflammatory drugs and selective cyclooxygenase-2 inhibitors, AK106-001616 reduced prostaglandin (PG) E2 and leukotriene (LT) B4 production by stimulated cells. The suppression of PGE2 and LTB4 production was also confirmed using an air pouch model in rats administered a single oral dose of AK106-001616. Read More

BMC Pulm Med 2019 Apr 11;19(1):75. Epub 2019 Apr 11.

Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Avicenne, Service de pneumologie, Site constitutif du centre de référence des maladies pulmonaires rares OrphaLung, EA2363, Université Paris, 13, Bobigny, France.

Background: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, with a median survival of 2-3 years and variable natural history, characterized by gradual and progressive deterioration. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication, associated with poor survival and a mortality > 50%. To date, no treatment has proven effective in AE-IPF, with cyclophosphamide (CYC) the only therapy suggested to be effective on survival, primarily based on retrospective series. Read More

Respir Res 2019 Apr 11;20(1):71. Epub 2019 Apr 11.

Università Cattolica del Sacro Cuore, Fondazione Policlinico A. Gemelli, Rome, Italy.

Background: Given the broad definition of an acute exacerbation of IPF, it is likely that acute exacerbations are heterogeneous in their aetiology, severity and clinical course. We used pooled data from the INPULSIS® trials of nintedanib versus placebo to investigate whether acute exacerbations reported as serious adverse events were associated with higher mortality than those reported as non-serious adverse events and to assess the effect of nintedanib on these types of events.

Methods: Adverse events considered by an investigator to be an acute exacerbation were adjudicated as a confirmed acute exacerbation, suspected acute exacerbation, or not an acute exacerbation. Read More

Am J Physiol Lung Cell Mol Physiol 2019 Apr 10. Epub 2019 Apr 10.

Molecular Pharmacology, University of Groningen, Netherlands.

Transforming growth factor-β (TGF-β)-induced fibroblast-to-myofibroblast differentiation contributes to remodeling in chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis, but whether this impacts the ability of fibroblasts to support lung epithelial repair remains little explored. We pre-treated human lung fibroblasts (primary [phFB] or MRC5 cells) with recombinant human TGF-β to induce myofibroblast differentiation, then co-cultured them with adult mouse lung EpCAM cells to investigate their capacity to support epithelial organoid formation in vitro. While control phFB and MRC5 lung fibroblasts supported organoid formation of mouse EpCAM cells, TGF-β-pre-treatment of both phFB and MRC5 impaired organoid-supporting ability. Read More

Semin Immunopathol 2019 Apr 9. Epub 2019 Apr 9.

Department of Immunology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan.

Fibrosis is defined as excessive deposition of the extracellular matrix (ECM) in the parenchyma of various organs, and sometimes leads to irreversible organ malfunction such as idiopathic pulmonary fibrosis (IPF), a fatal disorder of the lung. Chronic inflammatory stimuli induce fibrotic responses in various organs. Various immune cells, including T helper (Th) cells in the lung, protect the host from different harmful particles, including pathogenic microorganisms. Read More

Respiration 2019 Apr 9:1-10. Epub 2019 Apr 9.

Pulmonology Department, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, University Claude Bernard Lyon 1, Lyon, France.

Background: The European observational, prospective PASSPORT study evaluated the long-term safety of pirfenidone under real-world conditions in idiopathic pulmonary fibrosis (IPF), over up to 2 years following its initiation.

Objectives: The FAS (French Ancillary Study) assessed the clinical outcomes of IPF patients participating in PASSPORT (n = 192).

Methods: Efficacy data were collected retrospectively and prospectively. Read More

Am J Respir Crit Care Med 2019 Apr 9. Epub 2019 Apr 9.

University of Lille 2, Lille, France.

Rationale: Given the paucity of effective treatments for Idiopathic Pulmonary Fibrosis (IPF), new insights into the deleterious mechanisms controlling lung fibroblast activation, the key cell type driving the fibrogenic process, are essential to develop new therapeutic strategies. Transforming growth factor β (TGF-β) is the main pro-fibrotic factor, but its inhibition is associated with severe side effects due to its pleiotropic role.

Objectives: We hypothesized that downstream non-coding effectors of TGF-β in fibroblasts may represent new effective therapeutic targets whose modulation may be well-tolerated. Read More

Clin Exp Dermatol 2019 Apr 8. Epub 2019 Apr 8.

Hosary Medical Charity Centre, 2192, 8th Neighbourhood, 2nd District, 6 October City, Giza, Egypt.

I read with interest the case report case by Affleck et al.1 and its related comment by Abdelmaksoud.2 The cause of death was considered to be idiopathic pulmonary fibrosis -the commonest form of interstitial lung disease but his family were concerned that the methotrexate he had been taking was to blame. Read More

J Thorac Dis 2019 Feb;11(2):E20-E23

Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.

J Thorac Dis 2019 Feb;11(2):357-360

Division of Pulmonary, Critical Care, and Sleep Medicine, University of California San Diego. San Diego, CA, USA.

J Thorac Dis 2019 Feb;11(2):339-340

North West Interstitial Lung Disease Unit, Manchester University NHS Foundation Trust, Wythenshawe, Manchester, UK.

Respir Med 2019 Apr 12;150:30-37. Epub 2019 Feb 12.

Department of Medicine, Division of Pulmonary, Allergy, Critical Care, and Sleep, University of Minnesota, Minneapolis, MN, United States. Electronic address:

Interstitial lung disease (ILD) is a category of diffuse parenchymal lung diseases characterized by inflammation and/or fibrosis. The best characterized ILD is idiopathic pulmonary fibrosis (IPF). Acute exacerbation of IPF is a dreaded occurrence with grim prognosis and suboptimal treatment options. Read More

Respir Med 2019 Apr 25;150:154-160. Epub 2019 Mar 25.

Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy.

Background: The term Interstitial Pneumonia with Autoimmune Features (IPAF) describes patients with Interstitial Lung Diseases (ILDs) and clinical or serological features of autoimmune diseases insufficient to reach a specific classification of a Connective Tissue Disease (CTD). Currently, retrospective studies on IPAF patients have proven to be heterogeneous in general characteristics, outcomes and High-Resolution Computed Tomography (HRCT) pattern. This study aims to describe for the first time the clinical, serological and radiological features of a prospective cohort of IPAF patients. Read More

Respir Med 2019 Apr 13;150:120-125. Epub 2019 Mar 13.

University of Utah, Salt Lake City, UT, USA.

Background: Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease characterized by irreversible loss of lung function and an unpredictable course of disease progression.

Methods: The safety data for patients with IPF who received placebo in 6 clinical trials were pooled to examine the categories and frequencies of serious adverse events (SAEs) in this population.

Results: In 1082 patients with IPF who received placebo, 673 SAEs were reported. Read More

Swiss Med Wkly 2019 Feb 24;149(07-08). Epub 2019 Feb 24.

Background: Lung transplantation (LTx) provides a viable option for the survival of end-stage lung diseases. Besides survival as a clinical outcome measure, health-related quality of life (HRQoL) and psychological distress have become important outcomes in studies investigating the effectiveness of LTx in the short- and long-term.

Objective: To assess and compare HRQoL trajectories of patients after LTx prior to and over a follow-up period of three years post-transplant, and to identify differences regarding distress, HRQoL and patient-related outcomes. Read More

BMJ Open Respir Res 2019 18;6(1):e000363. Epub 2019 Feb 18.

Centre for Human and Applied Physiological Sciences, School of Basic and Medical Biosciences, Faculty of Life Sciences and Medicine, King's College London, London, UK.

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. Read More

Chest 2019 Apr;155(4):e91-e96

Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padova, Padova, Italy. Electronic address:

A nonsmoker man in his 40s underwent bilateral lung transplantation with a referral diagnosis of genetic-related idiopathic pulmonary fibrosis (IPF). The patient had no medical history in childhood and early adulthood, nor was there a family history of IPF. His nonsmoker father presented with lung cancer at 59 years of age. Read More

Med Clin North Am 2019 May 13;103(3):425-433. Epub 2019 Mar 13.

Lung Transplantation Program, Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, 9039 Gates Building, 3400 Spruce Street, Philadelphia, PA 19104, USA.

Lung transplantation is an appropriate therapeutic option for select patients with end-stage lung diseases and offers the possibility of improved quality of life and longer survival. Unfortunately, the transplant recipient is at risk for numerous immunologic, infectious, and medical complications that threaten both of these goals. Median survival after lung transplantation is approximately 6 years. Read More

Biochim Biophys Acta Mol Cell Res 2019 Apr 5. Epub 2019 Apr 5.

Roche Pharma Research and Early Development, Roche Innovation Center Basel, Switzerland; School of Pharmaceutical Sciences, University of Geneva, Geneva, Switzerland. Electronic address:

Discoidin domain receptor1 (DDR1) is a collagen activated receptor tyrosine kinase and an attractive anti-fibrotic target. Its expression is mainly limited to epithelial cells located in several organs including skin, kidney, liver and lung. DDR1's biology is elusive, with unknown downstream activation pathways; however, it may act as a mediator of the stromal-epithelial interaction, potentially controlling the activation state of the resident quiescent fibroblasts. Read More

Clin Pharmacokinet 2019 Apr 6. Epub 2019 Apr 6.

Galapagos SASU, 102 avenue Gaston Roussel, 93230, Romainville, France.

Background And Objectives: GLPG1690 is an autotaxin inhibitor in development for the treatment of idiopathic pulmonary fibrosis. Several publications suggested a role of autotaxin in the control of disease-affected lung function and of lysophosphatidic acid in lung remodeling processes. The aim of the current article was to describe the exposure-response relationship of GLPG1690 and further develop a rational basis to support dose selection for clinical trials in patients with idiopathic pulmonary fibrosis. Read More

Nat Commun 2019 04 5;10(1):1561. Epub 2019 Apr 5.

Institute of Cardiovascular Science, Faculty of Population Health Sciences, University College London, London, WC1E 6BT, UK.

Following numerous genome-wide association studies of disease susceptibility, there is increasing interest in genetic associations with prognosis, survival or other subsequent events. Such associations are vulnerable to index event bias, by which selection of subjects according to disease status creates biased associations if common causes of incidence and prognosis are not accounted for. We propose an adjustment for index event bias using the residuals from the regression of genetic effects on prognosis on genetic effects on incidence. Read More

Ann Am Thorac Soc 2019 Apr 5. Epub 2019 Apr 5.

Cedars-Sinai Medical Center, Los Angeles, California, United States.

Mediators Inflamm 2019 4;2019:3192089. Epub 2019 Mar 4.

Department of Pathology, Tongji Hospital, Tongji University School of Medicine, Shanghai 200065, China.