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Rheumatology (Oxford) 2019 Feb 15. Epub 2019 Feb 15.

Rheumatology Unit, Bnai-Zion Medical Center, Haifa, Israel.

Objective: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level. Read More

Transl Res 2019 Feb 5. Epub 2019 Feb 5.

Department of Medicine, University of Colorado School of Medicine, Aurora, Colorado.

Pulmonary fibrosis refers to the development of diffuse parenchymal abnormalities in the lung that cause dyspnea, cough, hypoxemia, and impair gas exchange, ultimately leading to respiratory failure. Though pulmonary fibrosis can be caused by a variety of underlying etiologies, ranging from genetic defects to autoimmune diseases to environmental exposures, once fibrosis develops it is irreversible and most often progressive, such that fibrosis of the lung is one of the leading indications for lung transplantation. This review aims to provide a concise summary of the recent advances in our understanding of the genetics and genomics of pulmonary fibrosis, idiopathic pulmonary fibrosis in particular, and how these recent discoveries may be changing the clinical approach to diagnosing and treating patients with fibrotic interstitial lung disease. Read More

Immunol Cell Biol 2019 Feb 15. Epub 2019 Feb 15.

Inflammation, Repair & Development, National Heart & Lung Institute, Imperial College London, London, UK.

The lungs present a challenging immunological dilemma for the host. Anatomically positioned at the environmental interface, they are constantly exposed to antigens, pollutants and microbes, while simultaneously facilitating vital gas exchange. Remarkably, the lungs maintain a functionally healthy state, ignoring harmless inhaled proteins, adapting to toxic environmental insults and limiting immune responses to allergens and pathogenic microbes. Read More

Respir Res 2019 Feb 15;20(1):35. Epub 2019 Feb 15.

Division of Pulmonary Medicine Department of Internal Medicine, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 120-752, Republic of Korea.

Background: Sarcopenia can contribute to negative outcomes in patients with various lung diseases. However, whether sarcopenia affects prognosis in patients with idiopathic pulmonary fibrosis (IPF) has not been reported. Simple measures of muscle mass, derived from chest computed tomography (CT), are increasingly being used to identify patients with sarcopenia. Read More

Fed Pract 2018 Feb;35(2):40-42

and are Pulmonary and Critical Care Medicine Fellow Physicians; and are Pulmonary and Critical Care Medicine Attending Physicians; and is a Research Study Coordinator, all at the VA Caribbean Healthcare System in San Juan, Puerto Rico.

Diagnosis of Sjogren syndrome should be based on consideration of the clinical presentation, a pulmonary function test, blood and rheumatology laboratory findings, radiographic imaging patterns, and biopsy results. Read More

Eur Respir J 2019 Feb 14. Epub 2019 Feb 14.

NIHR Respiratory Clinical Research Facility, Royal Brompton Hospital, London, UK.

PI3 Kinases (PI3Ks) and mammalian target of rapamycin (mTOR) play a role in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Omipalisib (GSK2126458) is a potent inhibitor of PI3K/mTOR.A randomised, placebo-controlled, double-blind, repeat dose escalation, experimental medicine study of omipalisib in subjects with IPF was conducted (NCT01725139) to test safety, tolerability, pharmacokinetics (PK) and pharmacodynamics (PD). Read More

Eur Respir J 2019 Feb 14;53(2). Epub 2019 Feb 14.

Division of Pulmonary, Allergy and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, AL, USA.

JCI Insight 2019 Feb 14. Epub 2019 Feb 14.

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with unremitting extracellular matrix deposition, leading to a distortion of pulmonary architecture and impaired gas exchange. Fibroblasts from IPF patients acquire an invasive phenotype that is essential for progressive fibrosis. Here, we performed RNA-seq analysis on invasive and non-invasive fibroblasts and found that the immune checkpoint ligand CD274 (PD-L1) was up-regulated on invasive lung fibroblasts and was required for the invasive phenotype of lung fibroblasts, is regulated by P53 and FAK, and drives lung fibrosis in a humanized IPF model in mice. Read More

Eur Respir J 2019 Feb 31;53(2). Epub 2019 Jan 31.

Dept of Respiratory Medicine, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.

Stat Appl Genet Mol Biol 2019 Feb 13. Epub 2019 Feb 13.

Department of Biostatistics, Yale School of Public Health, 60 College Street, New Haven, CT 06510, USA.

Longitudinal genomics data and survival outcome are common in biomedical studies, where the genomics data are often of high dimension. It is of great interest to select informative longitudinal biomarkers (e.g. Read More

Ann Am Thorac Soc 2018 Dec;15(Supplement_4):S266-S272

1 Division of Pulmonary and Critical Care Medicine and.

The mechanisms underlying the pathogenesis of chronic lung diseases, including chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis, remain incompletely understood. Mitochondria are vital cellular organelles crucial for energy generation, the maintenance of cellular metabolism, calcium homeostasis, intracellular signaling, and the regulation of cell death programs. Emerging evidence suggests that mitochondrial dysfunction plays a cardinal role in the initiation and progression of many human diseases, including chronic lung diseases. Read More

Am J Physiol Lung Cell Mol Physiol 2019 Feb 13. Epub 2019 Feb 13.

Medicine, McMaster University, Canada.

We have shown that calcium (Ca) oscillations in human pulmonary fibroblasts (HPFs) contribute to pro-fibrotic effects of TGFβ and that disruption of these oscillations blunts features of pulmonary fibrosis. Prostaglandin E (PGE) exerts anti-fibrotic effects in the lung but the mechanisms for this action are not well defined. We thus sought to explore interactions between PGE and the pro-fibrotic agent transforming growth factor beta (TGFβ) in pulmonary fibroblasts (PFs) isolated from patients with or without idiopathic pulmonary fibrosis (IPF). Read More

Lung 2019 Feb 13. Epub 2019 Feb 13.

Respiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy.

Introduction: Familial pulmonary fibrosis (FPF) is defined as an idiopathic diffuse parenchymal lung disease affecting two or more members of the same primary biological family. The aim of this study was to compare disease progression and tolerance to pirfenidone in a population of FPF patients who presented with radiological and/or histological evidence of UIP, and a group of idiopathic pulmonary fibrosis (IPF) patients.

Methods: Seventy-three patients (19 with FPF and 54 with IPF) were enrolled and data were collected retrospectively at 6, 12 and 24 months follow-up. Read More

Am J Respir Cell Mol Biol 2019 Feb 13. Epub 2019 Feb 13.

University Medical Center Groningen, Center for Congenital Heart Diseases, Groningen, Netherlands.

Cellular senescence is recognized as a crucial contributor to the pathobiology of various degenerative and cardiovascular diseases, like idiopathic pulmonary fibrosis and atherosclerosis. We describe the potential link between cellular senescence and the degenerative character of neointimal pulmonary vascular disease in pulmonary arterial hypertension (PAH). Senescence markers have been described in remodeled pulmonary arteries, triggers that induce senescence also induce PAH, pathways known to be disturbed in PAH, such as TGFβ/BMP and TNFα, are also causally associated to senescence and interventions that target a senescence phenotype, also target pulmonary vascular remodeling in vivo. Read More

Sci Rep 2019 Feb 12;9(1):1861. Epub 2019 Feb 12.

Centre for Translational Pharmacology, Institute of Molecular, Cell and Systems Biology, University of Glasgow, Glasgow, G12 8QQ, United Kingdom.

Many members of the G protein-coupled receptor family, including examples with clear therapeutic potential, remain poorly characterised. This often reflects limited availability of suitable tool ligands with which to interrogate receptor function. In the case of GPR84, currently a target for the treatment of idiopathic pulmonary fibrosis, recent times have seen the description of novel orthosteric and allosteric agonists. Read More

Respirology 2019 Feb 12. Epub 2019 Feb 12.

Firestone Institute for Respiratory Health, Research Institute at St Joseph's Healthcare, Department of Medicine, McMaster University, Hamilton, ON, Canada.

Front Bioeng Biotechnol 2019 22;7. Epub 2019 Jan 22.

ARTORG Center, Medical Faculty, University of Bern, Bern, Switzerland.

The lung alveolar region experiences remodeling during several acute and chronic lung diseases, as for instance idiopathic pulmonary fibrosis (IPF), a fatal disease, whose onset is correlated with repetitive microinjuries to the lung alveolar epithelium and abnormal alveolar wound repair. Although a high degree of mechanical stress (>20% linear strain) is thought to potentially induce IPF, the effect of lower, physiological levels of strain (5-12% linear strain) on IPF pathophysiology remains unknown. In this study, we examined the influence of mechanical strain on alveolar epithelial wound healing. Read More

J Thorac Dis 2018 Dec;10(12):6695-6701

Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan.

Background: We have sometimes observed interstitial pneumonia which had chronic course and unknown causes but not diagnosed as idiopathic pulmonary fibrosis (IPF). However, the treatment strategy was not established definitely. To clarify the usefulness of cyclosporin A (CsA) in idiopathic chronic fibrosing interstitial pneumonia (iCFIP) without IPF, we examined longitudinal changes in pulmonary physiology. Read More

J Thorac Dis 2018 Dec;10(12):6660-6669

University Hospital of Pulmonology, Clinical Center of Serbia, Belgrade, Serbia.

Background: Idiopathic pulmonary fibrosis (IPF) has common risk factors with cancer and significant similarities in the pathobiology process, both diseases having poor outcomes. Immune checkpoint PD-L1 has become the target of checkpoint inhibitory therapy that unleashes antitumor T cells and has revolutionized cancer treatment. This is a pilot study exploring membrane immune checkpoint PD-L1 expression in human IPF lung tissue samples and its soluble form, soluble PD-L1 (sPD-L1) plasma concentrations in IPF patients, in order to investigate potential role of PD-L1 as an IPF biomarker. Read More

Chest 2019 Feb 8. Epub 2019 Feb 8.

Department of Internal Medicine; Lung Clinic Waldhof-Elgershausen, 35753 Greifenstein, Germany; Member of the German Center for Lung Research; European IPF Registry and Biobank (eurIPFreg/bank). Electronic address:

Introduction: In this study, we retrospectively assessed the relation between physiological measurements, survival and quantitative computed tomographic (HRCT) indices in IPF patients.

Methods: 70 patients (48 male; mean age 66.3 years ± 9. Read More

Histopathology 2019 Feb 11. Epub 2019 Feb 11.

Department of Pathology, University of California San Francisco, San Francisco, California, USA.

Aims: To evaluate the clinical significance of bronchiolocentric fibrosis in patients with a histopathologic pattern of usual interstitial pneumonia.

Methods And Results: Two hundred fifty-two patients with pathological usual interstitial pneumonia pattern were identified. Two hundred fifteen of these patients (215/252) had the multidisciplinary diagnosis of idiopathic pulmonary fibrosis. Read More

Ann Glob Health 2019 Jan 22;85(1). Epub 2019 Jan 22.

Interstitial Lung Disease Unit, Pulmonology Service, Bellvitge University Hospital. Instituto de Investigación Biomédica de Bellvitge (IDIBELL).

More than 100 different conditions are grouped under the term interstitial lung disease (ILD). A diagnosis of an ILD primarily relies on a combination of clinical, radiological, and pathological criteria, which should be evaluated by a multidisciplinary team of specialists. Multiple factors, such as environmental and occupational exposures, infections, drugs, radiation, and genetic predisposition have been implicated in the pathogenesis of these conditions. Read More

Lancet Respir Med 2019 Feb 6. Epub 2019 Feb 6.

Department of Interstitial Lung Disease, Royal Papworth Hospital NHS Foundation Trust, Cambridge, UK; Department of Medicine, University of Cambridge, Cambridge, UK.

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis. Lung transplantation is the only intervention shown to increase life expectancy for patients with IPF, but it is associated with disease-specific challenges. In this Review, we discuss the importance of a proactive approach to the management of IPF comorbidities, including gastro-oesophageal reflux, pulmonary hypertension, coronary artery disease, and malignancy. Read More

Tissue Cell 2019 Feb 29;56:14-22. Epub 2018 Nov 29.

Department of Zoology, Maulana Azad College, 8, Rafi Ahmed Kidwai Road, Kolkata, 700013, West Bengal, India. Electronic address:

There is no effective therapy exists for Idiopathic pulmonary fibrosis (IPF) till now. Few studies have been done on protective effects of green tea in pulmonary fibrosis but there is no single report on black tea extract (BTE) in pulmonary fibrosis so far. This study aims to investigate the anti-fibrotic effect of BTE against experimental pulmonary fibrosis. Read More

Acta Cardiol 2019 Feb 8:1-2. Epub 2019 Feb 8.

a Department of Diagnostic Radiology , Iwata City Hospital , Iwata , Japan.

Pharmacology 2019 Feb 7;103(5-6):250-256. Epub 2019 Feb 7.

Department of Oncologic Pathology, Mie University, Tsu, Japan.

Background: Pirfenidone (PFD), which is an antifibrotic agent used for treatment of idiopathic pulmonary fibrosis, induces G0/G1 cell cycle arrest in fibroblasts. We hypothesized that PFD-induced G0/G1 cell cycle arrest might be achieved in other types of cells, including cancer cells. Here we investigated the effects of PFD on the proliferation of pancreatic cancer cells (PCCs) in vitro. Read More

Adv Ther 2019 Feb 7. Epub 2019 Feb 7.

Department of Respiratory Diseases, The First Hospital of China Medical University, Shenyang, China.

Purpose: To investigate the efficacy and safety of nintedanib versus placebo in Chinese patients with idiopathic pulmonary fibrosis (IPF).

Methods: The INPULSIS trials consisted of two replicate, randomized, placebo-controlled, double-blind trials comparing nintedanib 150 mg bid with placebo over a 52-week treatment period. The primary endpoint was annual rate of decline in forced vital capacity (FVC); key secondary endpoints were change from baseline in Saint George's Respiratory Questionnaire's total score and time to first investigator-reported acute exacerbation. Read More

Oxid Med Cell Longev 2019 8;2019:7090534. Epub 2019 Jan 8.

Institute of Translational Medicine, The First Hospital of Jilin University, Changchun 130001, China.

Transcription factor nuclear factor erythroid 2-related factor 2 (Nrf2) is a major regulator of antioxidant response element- (ARE-) driven cytoprotective protein expression. The activation of Nrf2 signaling plays an essential role in preventing cells and tissues from injury induced by oxidative stress. Under the unstressed conditions, natural inhibitor of Nrf2, Kelch-like ECH-associated protein 1 (Keap1), traps Nrf2 in the cytoplasm and promotes the degradation of Nrf2 by the 26S proteasome. Read More

Clinics (Sao Paulo) 2019 Feb 4;74:e225. Epub 2019 Feb 4.

Divisao Pulmonar, Instituto do Coracao (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR.

Idiopathic pulmonary fibrosis is a chronic disease of unknown etiology that usually has a progressive course and is commonly associated with a poor prognosis. The main symptoms of idiopathic pulmonary fibrosis, including progressive dyspnea and dry cough, are often nonspecific. Chest high-resolution computed tomography is the primary modality used in the initial assessment of patients with suspected idiopathic pulmonary fibrosis and may have considerable influence on subsequent management decisions. Read More

Rev Med Chil 2018 Sep;146(9):1033-1040

Division of Pulmonary and Critical Care Medicine, Mayo Clinic Jacksonville, Florida, United States.

Bronchoscopy cryoprobes are used for palliative treatment of endobronchial obstructions caused by tumors and removal of granulation tissue or foreign bodies. Currently this technology is also used for diagnosis of diffuse interstitial lung disease (ILD). The multidisciplinary team that establishes the clinical, radiological and histopathological correlation in ILD, decides about performing a surgical lung biopsy when the characteristics of the interstitial disease are not similar to Idiopathic Pulmonary Fibrosis (IPF). Read More

ERJ Open Res 2019 Feb 1;5(1). Epub 2019 Feb 1.

Amsterdam UMC, University of Amsterdam, Center for Experimental and Molecular Medicine, Amsterdam, The Netherlands.

http://ow.ly/HjsV30nbcji. Read More

Thorax 2019 Feb 5. Epub 2019 Feb 5.

Interstitial lung abnormalities, when present in members of undiagnosed family members recruited on the basis of familial interstitial pneumonia, or in undiagnosed research participants, have been associated with a syndrome that includes distinct sets of imaging abnormalities, restrictive physiological and exercise impairments, and an increased prevalence of histopathological findings, and genetic predictors, that have been noted in patients with idiopathic pulmonary fibrosis. Recent longitudinal studies have demonstrated that qualitative and quantitative assessments of interstitial abnormalities are associated with accelerated lung function decline, an increased rate of clinical diagnoses of interstitial lung disease and an increased rate of mortality. In this perspective, in addition to reviewing the prior information, four major efforts that could help the field of early pulmonary fibrosis detection move forward are discussed. Read More

Int J Mol Med 2019 Jan 31. Epub 2019 Jan 31.

Graduate Institute of Clinical Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan, R.O.C.

Idiopathic pulmonary fibrosis (IPF) is a lethal fibrotic lung disease with an increasing global burden. It is hypothesized that fibroblasts have a number of functions that may affect the development and progression of IPF. However, the present understanding of cellular and molecular mechanisms associated with fibroblasts in IPF remains limited. Read More

Mediators Inflamm 2019 3;2019:5160694. Epub 2019 Jan 3.

Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.

Background: Acute exacerbation of IPF (AE-IPF) is associated with high mortality. We studied changes in pathogen involvement during AE-IPF and explored a possible role of infection in AE-IPF.

Objectives: Our purpose is to investigate the role of infection in AE-IPF. Read More

BMC Pulm Med 2019 Feb 4;19(1):27. Epub 2019 Feb 4.

Department of Respiratory Medicine, Beijing Hospital of Traditional Chinese Medicine, Capital Medical University, No. 23rd Art Museum Backstreet, Dongcheng District, Beijing, 100010, China.

Background: Chronic cough is a difficult-to-treat comorbidity of idiopathic pulmonary fibrosis (IPF), and significantly impacts on the quality of life of patients with IPF. Transient receptor potential (TRP) channel proteins may play an important role in chronic cough. However, expression of these proteins in lung of IPF is largely unknown. Read More

Cells 2019 Feb 3;8(2). Epub 2019 Feb 3.

Department of Biomedical Sciences, University of North Dakota, Grand Forks, ND 58203, USA.

Autophagy is a highly conserved catabolic process involving autolysosomal degradation of cellular components, including protein aggregates, damaged organelles (such as mitochondria, endoplasmic reticulum, and others), as well as various pathogens. Thus, the autophagy pathway represents a major adaptive response for the maintenance of cellular and tissue homeostasis in response to numerous cellular stressors. A growing body of evidence suggests that autophagy is closely associated with diverse human diseases. Read More

Aging (Albany NY) 2019 Feb 4. Epub 2019 Feb 4.

Department of Medical Genetics, Nanjing University School of Medicine, Nanjing, China.

Background: Idiopathic pulmonary fibrosis (IPF) is an age-related fatal disease with an unknown etiology. Increased oxidative stress and mitochondrial dysfunction are thought to be involved in its pathogenesis. However, the effect of the polymorphism on IPF is not known. Read More

Histopathology 2019 Feb 4. Epub 2019 Feb 4.

Department of Pathology, Fukuoka University School of Medicine and Hospital.

Aims: Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome that results from tobacco smoking. Emphysema and fibrosis in CPFE patients have been considered to exist separately, with emphysema in the upper lobes and interstitial pneumonia in the lower lobes. The aim of this study was to examine the intrapulmonary distribution of fibrosis and emphysema in clinically diagnosed patients with idiopathic pulmonary fibrosis (IPF) having coexisting emphysema. Read More

J Biol Chem 2019 Feb 1. Epub 2019 Feb 1.

Medicine, University of Pittsburgh, United States.

The hormone relaxin is considered a potential therapy for idiopathic pulmonary fibrosis (IPF). We have previously shown that a potential limitation to relaxin-based IPF therapy is decreased expression of a relaxin receptor, relaxin/insulin-like family peptide receptor 1 (RXFP1), in IPF fibroblasts. The mechanism that down-regulates RXFP1 in IPF remains unclear. Read More

PLoS One 2019 1;14(2):e0211455. Epub 2019 Feb 1.

Department of Pulmonology, "Victor Babes" University of Medicine and Pharmacy, Timişoara, Romania.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease with a fatal prognosis to whose rapid evolution multiple comorbidities may contribute, one of the most common being obstructive sleep apnea (OSA). There are several potential factors and conditions for the emergence of a cognitive deficit in relation to IPF or associated morbidities.

Objectives: The goals of this study were to assess cognition in patients with IPF in stable phase and to identify clinical cognition modifiers. Read More

Clin Respir J 2019 Jan 31. Epub 2019 Jan 31.

3rd Department of Cardiology, School of Medicine with the Division of Dentistry in Zabrze, Medical University of Silesia, Katowice, Silesian Centre for Heart Diseasein Zabrze, Poland.

Introduction: Idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD) show poor prognosis. The importance of left (LV) and right (RV) ventricular morphology and function in patients with end-stage lung diseases referred for lung transplantation (LT) is not well established.

Objectives: To assess whether LV and RV echocardiographic parameters influence survival of patients with IPF, COPD and other interstitial lung diseases (ILD) awaiting LT. Read More

Respir Med 2019 Feb 9;147:79-91. Epub 2019 Jan 9.

Department of Pulmonary Medicine, Erasmus Medical Centre, Rotterdam, the Netherlands. Electronic address:

Idiopathic pulmonary fibrosis (IPF) is a progressive, and ultimately fatal, chronic interstitial lung disease characterized by enhanced extracellular matrix deposition. Repetitive alveolar epithelial injury triggers the early development of fibrosis. These injuries, in combination with dysregulated wound repair and fibroblast dysfunction, lead to ongoing tissue remodelling and fibrosis seen in end-stage pulmonary fibrosis. Read More

Respir Med 2019 Feb 10;147:51-57. Epub 2019 Jan 10.

Department of Diseases of the Thorax, GB Morgagni Hospital, Asl Romagna, Forlì, Italy; Department of Respiratory Diseases & Allerg, Aarhus University Hospital, Aarhus, Denmark.

Purpose: the study aims at describing the role of sleep disordered breathing (SDB) on daytime symptoms, quality of sleep and quality of life (QoL) in patients with moderate-severe IPF.

Methods: we enrolled 34 consecutive room air breathing IPF outpatients who received a full-night polysomnography. The following questionnaires were administered: Epworth Sleepiness Score (ESS), Pittsburg Sleep Quality Index (PSQI), StGeorge's Questionnaire (StGQ). Read More

Respir Med 2019 Feb 3;147:31-36. Epub 2019 Jan 3.

Center for Interstitial Lung Diseases, Division of Pulmonary, Critical Care & Sleep Medicine, University of Washington (UW), Seattle, USA. Electronic address:

Background: The diagnostic algorithm for idiopathic pulmonary fibrosis (IPF) guidelines has some shortcomings. The aim of the present study was to develop a novel software, "IPFdatabase", that could readily apply the diagnostic criteria per IPF guidelines and make a 'virtual' diagnosis of IPF.

Methods: Software was developed as a step-by-step compilation of necessary information according to guidelines to enable a diagnosis of IPF. Read More

Int J Mol Sci 2019 Jan 30;20(3). Epub 2019 Jan 30.

Department of Pharmacology, Faculty of Medicine, University of Valencia, 46010 Valencia, Spain.

Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pulmonary disease with a median survival of 2⁻4 years after diagnosis. A significant number of IPF patients have risk factors, such as a history of smoking or concomitant emphysema, both of which can predispose the patient to lung cancer (LC) (mostly non-small cell lung cancer (NSCLC)). In fact, IPF itself increases the risk of LC development by 7% to 20%. Read More

Ter Arkh 2018 Apr;90(3):42-46

Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University, Moscow, Russia.

Aim: To study the clinical significance of SP-A, SP-D in assessing the activity of idiopathic pulmonary fibrosis and sarcoidosis. We examined 81 patients with morphologically confirmed diagnoses of idiopathic pulmonary fibrosis (ILF) and sarcoidosis, a control group of 20 healthy individuals. The MSCT of the thoracic organs of the chest was performed, the diffusivity of the lungs was examined, oxygen saturation was determined. Read More

Lab Invest 2019 Jan 30. Epub 2019 Jan 30.

Institute of Functional and Applied Anatomy, Hannover Medical School, Hannover, Germany.

In human idiopathic pulmonary fibrosis (IPF), collapse of distal airspaces occurs in areas of the lung not (yet) remodeled. Mice lungs overexpressing transforming growth factor-β1 (TGF-β1) recapitulate this abnormality: surfactant dysfunction results in alveolar collapse preceding fibrosis and loss of alveolar epithelial type II (AE2) cells' apical membrane surface area. Here we examined whether surfactant dysfunction-related alveolar collapse due to TGF-β1 overexpression is linked to septal wall remodeling and AE2 cell abnormalities. Read More

Hum Vaccin Immunother 2019 Jan 30:1-4. Epub 2019 Jan 30.

a Department of Respiratory Medicine and Allergology , Sapporo Medical University School of Medicine , Sapporo , Japan.

Streptococcus pneumoniae causes pneumonia, meningitis, otitis media, and bacteremia. The mortality and morbidity of invasive pneumococcal disease are high among adults aged >65 years or those with underlying chronic or immunosuppressive conditions. A recent systematic review showed that patients treated with immunosuppressive agents have impaired immune responses to pneumococcal conjugate vaccine (PCV) and pneumococcal polysaccharide vaccine compared with healthy subjects. Read More

J Aerosol Med Pulm Drug Deliv 2019 Jan 30. Epub 2019 Jan 30.

1 Alfred Hospital, Department of Medicine, Monash University, Melbourne, Australia.

Background: This clinical trial evaluated the pharmacokinetics and safety/tolerability of inhaled pirfenidone solution in volunteers and patients with idiopathic pulmonary fibrosis (IPF).

Methods: Forty-four adults in six cohorts consented to receive single doses of a 12.5 mg/mL pirfenidone solution or placebo to assess tolerability and pharmacokinetics. Read More

Gan To Kagaku Ryoho 2018 Dec;45(13):2384-2386

Dept. of Surgery, Tokyo Women's Medical University Medical Center East.

We report a case of sigmoid colon cancer that was incidentally found using CT that was performed for upper abdominal pain. An 83-year-old man with a long history of lung tuberculosis and idiopathic pulmonary fibrosis presented with upper abdominal pain. CT findings revealed free gas around the stomach. Read More