457 results match your criteria Collagen-Vascular Disease Associated With Interstitial Lung Disease


Genetic Variation in CCL18 Gene Influences CCL18 Expression and Correlates with Survival in Idiopathic Pulmonary Fibrosis: Part A.

J Clin Med 2020 Jun 21;9(6). Epub 2020 Jun 21.

Centre for Interstitial Lung Diseases, Department of Pulmonology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands.

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disease, characterized by fibroblast proliferation and extracellular matrix deposition. CC-chemokine ligand 18 (CCL18) upregulates the production of collagen by lung fibroblasts and is associated with mortality. This study was designed to evaluate the influence of single nucleotide polymorphisms (SNPs) in the gene on CCL18 expression and survival in IPF. Read More

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http://dx.doi.org/10.3390/jcm9061940DOI Listing

Mitochondria dysfunction and metabolic reprogramming as drivers of idiopathic pulmonary fibrosis.

Redox Biol 2020 06 19;33:101509. Epub 2020 Mar 19.

Aging Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA; Division of Pulmonary Allergy and Critical Care Medicine, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA; Vascular Medicine Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA. Electronic address:

Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown etiology. It is characterized by deposition of extracellular matrix proteins, like collagen and fibronectin in the lung interstitium leading to respiratory failure. Our understanding of the pathobiology underlying IPF is still incomplete; however, it is accepted that aging is a major risk factor in the disease while growing evidence suggests that the mitochondria plays an important role in the initiation and progression of pulmonary fibrosis. Read More

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http://dx.doi.org/10.1016/j.redox.2020.101509DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7251240PMC

Thrombospondin-1 mediates muscle damage in brachio-cervical inflammatory myopathy and systemic sclerosis.

Neurol Neuroimmunol Neuroinflamm 2020 May 6;7(3). Epub 2020 Mar 6.

From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.

Objective: To describe the clinical, serologic and histologic features of a cohort of patients with brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc) and unravel disease-specific pathophysiologic mechanisms occurring in these patients.

Methods: We reviewed clinical, immunologic, muscle MRI, nailfold videocapillaroscopy, muscle biopsy, and response to treatment data from 8 patients with BCIM-SSc. We compared cytokine profiles between patients with BCIM-SSc and SSc without muscle involvement and controls. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000694DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7136050PMC

Aryl hydrocarbon receptor signals attenuate lung fibrosis in the bleomycin-induced mouse model for pulmonary fibrosis through increase of regulatory T cells.

Arthritis Res Ther 2020 02 7;22(1):20. Epub 2020 Feb 7.

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan.

Background: Interstitial lung disease (ILD) is a serious complication of connective tissue diseases (CTDs). Although immune dysregulation triggered by genetic and environmental factors is thought to provoke inflammation and subsequent fibrosis, precise mechanisms of these processes remain unclear. Recent reports suggest that activation of aryl hydrocarbon receptor (AhR) signals by various ligands such as tryptophan derivatives can induce hyper-immune responses and are involved in autoimmunity. Read More

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http://dx.doi.org/10.1186/s13075-020-2112-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7006193PMC
February 2020

The effect of human adipose-derived stem cells on lipopolysaccharide-induced acute respiratory distress syndrome in mice.

Ann Transl Med 2019 Nov;7(22):674

Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea; Asan Life Institute, Seoul, Korea.

Background: Acute respiratory distress syndrome (ARDS) is a type of acute respiratory failure in critically ill patients. Recently, several treatment modalities have been proposed for ARDS, but it still has a high mortality rate. In general, the role of mesenchymal stem cells (MSCs) in controlling inflammatory responses has been studied in various immune-associated diseases in humans and animals. Read More

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http://dx.doi.org/10.21037/atm.2019.10.48DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6944600PMC
November 2019

Antifibrotic efficacy of nintedanib in a cellular model of systemic sclerosis-associated interstitial lung disease.

Clin Exp Rheumatol 2019 Jul-Aug;37 Suppl 119(4):115-124. Epub 2019 Sep 17.

Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.

Objectives: Nintedanib is approved for the treatment of idiopathic pulmonary fibrosis (IPF) and was demonstrated to slow disease progression in patients with IPF by reducing decline in forced vital capacity by 50%. Recently, nintedanib has been reported to exert anti-fibrotic activity on systemic sclerosis (scleroderma, SSc) skin fibroblasts and to diminish skin and lung fibrosis in mouse models. The goal of the present study was to determine the effects of nintedanib on a cellular model of SSc-associated interstitial lung disease (ILD). Read More

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October 2019
13 Reads

The Interstitium in the Hypertrophied Heart.

JACC Cardiovasc Imaging 2019 11 18;12(11 Pt 2):2357-2368. Epub 2019 Sep 18.

Cardiovascular Research Centre and Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom; National Heart and Lung Institute, Imperial College, London, United Kingdom. Electronic address:

Pathological left ventricular hypertrophy is a common feature of many cardiac diseases. It results from both myocyte hypertrophy and interstitial expansion. Interstitial expansion is most commonly secondary to the accumulation of mature cross-linked collagen fibers due to dysregulated metabolism, known as interstitial fibrosis. Read More

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http://dx.doi.org/10.1016/j.jcmg.2019.05.033DOI Listing
November 2019
1 Read

Hyaluronic Acid-Decorated Liposomes as Innovative Targeted Delivery System for Lung Fibrotic Cells.

Molecules 2019 Sep 10;24(18). Epub 2019 Sep 10.

Research Laboratory of Lung Diseases, Section of Cell Biology, IRCCS Policlinico San Matteo Foundation, 27100 Pavia, Italy.

Collagen Tissue Disease-associated Interstitial Lung Fibrosis (CTD-ILDs) and Bronchiolitis Obliterans Syndrome (BOS) represent severe lung fibrogenic disorders, characterized by fibro-proliferation with uncontrolled extracellular matrix deposition. Hyaluronic acid (HA) plays a key role in fibrosis with its specific receptor, CD44, overexpressed by CTD-ILD and BOS cells. The aim is to use HA-liposomes to develop an inhalatory treatment for these diseases. Read More

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http://dx.doi.org/10.3390/molecules24183291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6766933PMC
September 2019
35 Reads

Single-cell analysis reveals fibroblast heterogeneity and myofibroblasts in systemic sclerosis-associated interstitial lung disease.

Ann Rheum Dis 2019 10 12;78(10):1379-1387. Epub 2019 Aug 12.

Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.

Objectives: Myofibroblasts are key effector cells in the extracellular matrix remodelling of systemic sclerosis-associated interstitial lung disease (SSc-ILD); however, the diversity of fibroblast populations present in the healthy and SSc-ILD lung is unknown and has prevented the specific study of the myofibroblast transcriptome. We sought to identify and define the transcriptomes of myofibroblasts and other mesenchymal cell populations in human healthy and SSc-ILD lungs to understand how alterations in fibroblast phenotypes lead to SSc-ILD fibrosis.

Methods: We performed droplet-based, single-cell RNA-sequencing with integrated canonical correlation analysis of 13 explanted lung tissue specimens (56 196 cells) from four healthy control and four patients with SSc-ILD, with findings confirmed by cellular indexing of transcriptomes and epitopes by sequencing in additional samples. Read More

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http://dx.doi.org/10.1136/annrheumdis-2018-214865DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7255436PMC
October 2019
8 Reads

Role of Deleterious Rare Alleles for Acute-Onset Diffuse Interstitial Lung Disease in Collagen Diseases.

Clin Med Insights Circ Respir Pulm Med 2019 30;13:1179548419866443. Epub 2019 Jul 30.

Clinical Research Center for Allergy and Rheumatology, National Hospital Organization Sagamihara National Hospital, Sagamihara, Japan.

Objective: Acute-onset diffuse interstitial lung disease (AoDILD) includes acute exacerbation of interstitial lung disease (ILD), drug-induced ILD, and pneumonia in collagen diseases patients. As AoDILD causes a poor prognosis in collagen disease patients, the pathogenesis of AoDILD should be investigated. Exome sequencing studies revealed that rare variants were detected to be causative in some diseases. Read More

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http://dx.doi.org/10.1177/1179548419866443DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6668171PMC
July 2019
3 Reads

Clinical features and outcome of acute exacerbation in connective tissue disease-associated interstitial lung disease: A single-center study from India.

Int J Rheum Dis 2019 Sep 22;22(9):1741-1745. Epub 2019 Jul 22.

Department of Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, India.

Background: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is associated with high mortality, but there is limited clinical data on AE of interstitial lung disease (ILD) in connective tissue disease-associated ILD (CTD ILD). The present study was conducted to provide prevalence and clinical features of AE, as well as various risk factors associated with mortality among AE CTD ILD patients.

Methods: Between May 2013 and April 2018, 15 patients who developed AE among 105 consecutive patients with CTD with chronic ILD were included. Read More

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http://dx.doi.org/10.1111/1756-185X.13666DOI Listing
September 2019
7 Reads

Inhibitory effects of pirfenidone on fibroblast to myofibroblast transition in rheumatoid arthritis-associated interstitial lung disease via the downregulation of activating transcription factor 3 (ATF3).

Int Immunopharmacol 2019 Sep 19;74:105700. Epub 2019 Jun 19.

Department of Rheumatology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, PR China. Electronic address:

Objective: Pirfenidone (PFD) is an oral anti-fibrotic drug used for idiopathic pulmonary fibrosis (IPF) therapy. We determined the role of activating transcription factor 3 (ATF3) and the effect of PFD on fibroblast to myofibroblast transition (FMT) in rheumatoid arthritis-associated interstitial lung disease (RA-ILD).

Methods: RA-ILD lung specimens were obtained by CT-guided percutaneous transthoracic biopsy. Read More

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http://dx.doi.org/10.1016/j.intimp.2019.105700DOI Listing
September 2019
3 Reads

Targeting the RAS axis alleviates silicotic fibrosis and Ang II-induced myofibroblast differentiation via inhibition of the hedgehog signaling pathway.

Toxicol Lett 2019 Oct 7;313:30-41. Epub 2019 Jun 7.

School of Public Health, North China University of Science and Technology, Tangshan, China; Hebei Key Laboratory for Organ Fibrosis, Medical Research Center, North China University of Science and Technology, Tangshan, China. Electronic address:

The hedgehog (HH) signaling pathway plays an important role in lung development, but its significance in silicosis is unclear. We showed that in human coal pneumoconiosis autopsy specimens, Sonic Hedgehog (SHH) and the Glioma-associated oncogene homolog transcription factors family (GLI) 1 proteins were up-regulated, whereas Patch-1 (PTC) was down-regulated. The protein levels of SHH, smoothened (SMO), GLI1, GLI2, α-smooth muscle actin (α-SMA) and collagen type Ⅰ (Col Ⅰ) were also elevated gradually in the bronchoalveolar lavage fluid (BALF) of different stages of coal pneumoconiosis patients, dynamic silica-inhalation rat lung tissue and MRC-5 cells induced by Ang II at different time points, whereas the PTC and GLI3 levels were diminished gradually. Read More

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http://dx.doi.org/10.1016/j.toxlet.2019.05.023DOI Listing
October 2019
11 Reads
3.262 Impact Factor

Management of Fibrosing Interstitial Lung Diseases.

Adv Ther 2019 07 22;36(7):1518-1531. Epub 2019 May 22.

Unit for Interstitial Lung Diseases, Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.

A proportion of patients with interstitial lung diseases (ILDs), including the ILDs that are commonly associated with autoimmune diseases, develop a progressive fibrosing phenotype characterised by worsening of lung function, dyspnoea and quality of life, and early mortality. No drugs are approved for the treatment of ILDs other than idiopathic pulmonary fibrosis (IPF). At present, immunomodulatory medications are the mainstay of treatment for non-IPF ILDs. Read More

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http://dx.doi.org/10.1007/s12325-019-00992-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6824393PMC
July 2019
7 Reads

Extracellular Matrix Component Remodeling in Respiratory Diseases: What Has Been Found in Clinical and Experimental Studies?

Cells 2019 04 11;8(4). Epub 2019 Apr 11.

Department of Clinical Medicine, Laboratory of Experimental Therapeutics/LIM-20, School of Medicine of University of Sao Paulo, Sao Paulo 01246-903, Brazil.

Changes in extracellular matrix (ECM) components in the lungs are associated with the progression of respiratory diseases, such as asthma, chronic obstructive pulmonary disease (COPD), and acute respiratory distress syndrome (ARDS). Experimental and clinical studies have revealed that structural changes in ECM components occur under chronic inflammatory conditions, and these changes are associated with impaired lung function. In bronchial asthma, elastic and collagen fiber remodeling, mostly in the airway walls, is associated with an increase in mucus secretion, leading to airway hyperreactivity. Read More

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https://www.mdpi.com/2073-4409/8/4/342
Publisher Site
http://dx.doi.org/10.3390/cells8040342DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6523091PMC
April 2019
15 Reads

Specific Features of Fibrotic Lung Fibroblasts Highly Sensitive to Fibrotic Processes Mediated via TGF-β-ERK5 Interaction.

Cell Physiol Biochem 2019 ;52(4):822-837

Division of Respiratory Medicine, Juntendo University Faculty of Medicine & Graduate School of Medicine, Tokyo, Japan.

Background/aims: Lung fibrosis is associated with lung tissue contraction due to abnormal accumulation of myofibroblasts, which aggressively promote the fibrotic process. Transforming growth factor (TGF)-β signaling in fibroblasts promotes extracellular matrix (ECM) synthesis and fibroblast migration and differentiation into myofibroblasts. Inhibition of extracellular signal-regulated kinase (ERK)5 blocks lung fibroblast activation by suppressing TGF-β signaling. Read More

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http://dx.doi.org/10.33594/000000057DOI Listing
April 2019
17 Reads

Malondialdehyde-Acetaldehyde Adducts and Antibody Responses in Rheumatoid Arthritis-Associated Interstitial Lung Disease.

Arthritis Rheumatol 2019 09 22;71(9):1483-1493. Epub 2019 Jul 22.

VA Nebraska-Western Iowa Health Care System and University of Nebraska Medical Center, Omaha.

Objective: To compare serum anti-malondialdehyde-acetaldehyde (anti-MAA) antibody levels and MAA expression in lung tissue from patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD) to those found in controls.

Methods: Anti-MAA antibody (IgA, IgM, IgG) concentrations were measured in patients with RA-ILD and compared to those of RA patients with chronic obstructive pulmonary disease (COPD) and RA patients without lung disease. Associations between anti-MAA antibody with RA-ILD were assessed using multivariable logistic regression. Read More

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http://dx.doi.org/10.1002/art.40900DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6717041PMC
September 2019
5 Reads

Pleuroparenchymal fibroelastosis secondary to autologous hematopoietic stem cell transplantation: A case report.

Exp Ther Med 2019 Apr 13;17(4):2557-2560. Epub 2019 Feb 13.

Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing 100029, P.R. China.

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease. Although an increased number of PPFE cases have been reported recently, the characteristics of this condition have not been well described. The present study reports on the case of a 34-year-old male patient who presented with unilateral lung abnormalities. Read More

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http://www.spandidos-publications.com/10.3892/etm.2019.7275
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http://dx.doi.org/10.3892/etm.2019.7275DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425129PMC
April 2019
33 Reads

Dust induces lung fibrosis through dysregulated DNA methylation.

Environ Toxicol 2019 Jun 28;34(6):728-741. Epub 2019 Feb 28.

Department of Occupational and Environmental Health, School of Public Health and Management, Ningxia Medical University, Yinchuan, Ningxia, People's Republic of China.

Pneumoconiosis is a serious occupational disease that often occurs to coal workers with no early diagnosis and effective treatment at present. Diffuse pulmonary fibrosis is the major pathological change of pneumoconiosis, and its mechanism is still unclear. Epigenetics is involved in the development of many diseases, and it is closely associated with fibrosis. Read More

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http://dx.doi.org/10.1002/tox.22739DOI Listing
June 2019
13 Reads

The contribution of bronchoalveolar lavage in the diagnosis of welder's lung in a patient with pulmonary fibrosis.

Arch Environ Occup Health 2020 7;75(1):56-59. Epub 2019 Jan 7.

Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey.

Interstitial pulmonary fibrosis is rare clinical entity related to welding usually associated with long term and heavy exposure to welding fumes. A 56-year-old asymptomatic male patient was referred to our clinic due to abnormal chest X-ray findings that was requested for regular controls. He has been working as a welder both indoor and outdoor settings for the last 25 years. Read More

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http://dx.doi.org/10.1080/19338244.2018.1544113DOI Listing
March 2020
10 Reads

New risk scoring system for predicting acute exacerbation of interstitial pneumonia after chemotherapy for lung cancer associated with interstitial pneumonia.

Lung Cancer 2018 11 7;125:253-257. Epub 2018 Oct 7.

Division of Respiratory Medicine, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.

Background: Fatal acute exacerbation (AE) of interstitial pneumonia (IP) sometimes occurs after chemotherapy for lung cancer. We developed and evaluated a scoring system for assessing AE risk after chemotherapy in patients with lung cancer associated with IP.

Methods: A review of medical records identified 109 patients with primary lung cancer associated with IP who had received chemotherapy at our center during the period from June 2007 through September 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01695002183059
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http://dx.doi.org/10.1016/j.lungcan.2018.10.008DOI Listing
November 2018
30 Reads

Diffuse Alveolar Hemorrhage and Pulmonary Vasculitides: Histopathologic Findings.

Semin Respir Crit Care Med 2018 08 7;39(4):425-433. Epub 2018 Nov 7.

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.

Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1669412
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http://dx.doi.org/10.1055/s-0038-1669412DOI Listing
August 2018
48 Reads

Proposed method of histological separation between connective tissue disease-associated interstitial pneumonia and idiopathic interstitial pneumonias.

PLoS One 2018 5;13(11):e0206186. Epub 2018 Nov 5.

Nagasaki University Hospital, Nagasaki Educational and Diagnostic Center of Pathology (NEDCP), Nagasaki, Japan.

Objectives: Idiopathic interstitial pneumonia (IIP) and connective tissue disease -associated interstitial pneumonia (CTD-IP) are the two most common types of interstitial pneumonia. IIP and CTD-IP share common histological features, yet their clinical management is different. Separation of the two conditions based solely on histology can be challenging, and there are no established criteria. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0206186PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218032PMC
April 2019
7 Reads

Inhibitory Anti-Peroxidasin Antibodies in Pulmonary-Renal Syndromes.

J Am Soc Nephrol 2018 11 2;29(11):2619-2625. Epub 2018 Oct 2.

Department of Nephrology and Hypertension,

Background: Goodpasture syndrome (GP) is a pulmonary-renal syndrome characterized by autoantibodies directed against the NC1 domains of collagen IV in the glomerular and alveolar basement membranes. Exposure of the cryptic epitope is thought to occur disruption of sulfilimine crosslinks in the NC1 domain that are formed by peroxidasin-dependent production of hypobromous acid. Peroxidasin, a heme peroxidase, has significant structural overlap with myeloperoxidase (MPO), and MPO-ANCA is present both before and at GP diagnosis in some patients. Read More

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http://www.jasn.org/lookup/doi/10.1681/ASN.2018050519
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http://dx.doi.org/10.1681/ASN.2018050519DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218858PMC
November 2018
45 Reads

Necrobiosis lipoidica associated with sarcoidosis.

J Cutan Pathol 2018 Dec 9;45(12):944-948. Epub 2018 Oct 9.

Department of Dermatology, University of Virginia, Charlottesville, Virginia.

We report the case of a 40-year-old African-American female with biopsy-proven pulmonary sarcoidosis who developed atrophic plaques on her shins, trunk, and scalp that were clinically and histologically consistent with necrobiosis lipoidica (NL). The lesions appeared 3 years after her diagnosis of sarcoidosis, and progressed despite chronic prednisone. Sarcoidosis and NL are granulomatous skin disorders reported to coexist in the same patient only 10 times in the literature. Read More

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http://doi.wiley.com/10.1111/cup.13357
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http://dx.doi.org/10.1111/cup.13357DOI Listing
December 2018
28 Reads

Comorbidities of IPF: How do they impact on prognosis.

Pulm Pharmacol Ther 2018 12 5;53:6-11. Epub 2018 Sep 5.

Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of Catania, Catania, Italy. Electronic address:

Idiopathic Pulmonary Fibrosis (IPF) is a severe parenchymal lung disease characterized by an intense deposition of collagen in the interstitial spaces. The introduction of anti-fibrotic drugs increased patients' life expectancy highlighting the role of comorbidities in patients' management and prognosis. IPF is frequently associated with other diseases mainly because of its onset during middle age and sometimes because of the presence of common pathogenic pathways such as in the case of lung cancer. Read More

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http://dx.doi.org/10.1016/j.pupt.2018.09.003DOI Listing
December 2018
19 Reads

Transcriptome profiling reveals the complexity of pirfenidone effects in idiopathic pulmonary fibrosis.

Eur Respir J 2018 11 22;52(5). Epub 2018 Nov 22.

Dept of Biochemistry, Universities of Giessen and Marburg Lung Center, Giessen, Germany.

Despite the beneficial effects of pirfenidone in treating idiopathic pulmonary fibrosis (IPF), it remains unclear if lung fibroblasts (FB) are the main therapeutic target.To resolve this question, we employed a comparative transcriptomic approach and analysed lung homogenates (LH) and FB derived from IPF patients treated with or without pirfenidone.In FB, pirfenidone therapy predominantly affected growth and cell division pathways, indicating a major cellular metabolic shift. Read More

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http://erj.ersjournals.com/lookup/doi/10.1183/13993003.00564
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http://dx.doi.org/10.1183/13993003.00564-2018DOI Listing
November 2018
51 Reads

Computed tomography trachea volumetry in patients with scleroderma: Association with clinical and functional findings.

PLoS One 2018 1;13(8):e0200754. Epub 2018 Aug 1.

Postgraduate Programme in Medical Sciences, School of Medical Sciences, State University of Rio de Janeiro, Rio de Janeiro, Brazil.

Background: In scleroderma, excessive collagen production can alter tracheal geometry, and computed tomography (CT) volumetry of this structure may aid in detecting possible abnormalities. The objectives of this study were to quantify the morphological abnormalities in the tracheas of ​​patients with scleroderma and to correlate these findings with data on clinical and pulmonary function.

Methods: This was a cross-sectional study in which 28 adults with scleroderma and 27 controls matched by age, gender and body mass index underwent chest CT with posterior segmentation and skeletonization of the images. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0200754PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6070209PMC
January 2019
16 Reads

Platelet microparticles sustain autophagy-associated activation of neutrophils in systemic sclerosis.

Sci Transl Med 2018 07;10(451)

Università Vita-Salute San Raffaele and Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute, via Olgettina 58, 20132 Milano, Italy.

Endothelial cell damage and platelet activation contribute to sustained vasculopathy, which is a key clinical characteristic of systemic sclerosis (SSc), also known as scleroderma. Microparticles released from activated platelets in the blood of SSc patients (SSc-microparticles) are abundant and express the damage-associated molecular pattern (DAMP) HMGB1. SSc-microparticles interacted with neutrophils in vitro and in immunocompromised mice and promoted neutrophil autophagy, which was characterized by mobilization of their granule content, enhanced proteolytic activity, prolonged survival, and generation of neutrophil extracellular traps (NETs). Read More

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http://stm.sciencemag.org/lookup/doi/10.1126/scitranslmed.aa
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http://dx.doi.org/10.1126/scitranslmed.aao3089DOI Listing
July 2018
45 Reads
15.843 Impact Factor

Multidimensional improvement in connective tissue disease-associated interstitial lung disease: Two courses of pulse dose methylprednisolone followed by low-dose prednisone and tacrolimus.

Respirology 2018 11 16;23(11):1041-1048. Epub 2018 Jul 16.

Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Background And Objective: Corticosteroids and immunosuppressive agents are considered mainstays of therapy for connective tissue disease-related interstitial lung disease (CTD-ILD); however, tacrolimus with corticosteroid therapy has not been fully investigated. Our objectives were to examine the multidimensional therapeutic benefit and tolerability of the combined therapy for the initial treatment of patients with CTD-ILD.

Methods: In this retrospective case series, we identified consecutive CTD-ILD patients treated with tacrolimus plus intravenous (i. Read More

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http://dx.doi.org/10.1111/resp.13365DOI Listing
November 2018
44 Reads

Collagen biomarkers and subclinical interstitial lung disease: The Multi-Ethnic Study of Atherosclerosis.

Respir Med 2018 07 6;140:108-114. Epub 2018 Jun 6.

Department of Medicine, Columbia University Irving Medical Center, 161 Fort Washington Avenue, New York, NY, 10032, USA; Department of Epidemiology, Columbia University Irving Medical Center, 161 Fort Washington Avenue, New York, NY, 10032, USA. Electronic address:

Background: Lung fibrosis is attributed to derangements in extracellular matrix remodeling, a process driven by collagen turnover. We examined the association of two collagen biomarkers, carboxy-terminal telopeptide of collagen type I (ICTP) and amino-terminal propeptide of type III procollagen (PIIINP), with subclinical interstitial lung disease (ILD) in adults.

Methods: We performed a cross-sectional analysis of 3244 participants age 45-84 years in the Multi-Ethnic Study of Atherosclerosis. Read More

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http://dx.doi.org/10.1016/j.rmed.2018.06.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6310068PMC
July 2018
13 Reads

Lysyl oxidase like-2 contributes to renal fibrosis in Col4α3/Alport mice.

Kidney Int 2018 08 16;94(2):303-314. Epub 2018 May 16.

PharmAkea Inc., San Diego, California, USA.

Lysyl oxidase like-2 (LOXL2) is an amine oxidase with both intracellular and extracellular functions. Extracellularly, LOXL2 promotes collagen and elastin crosslinking, whereas intracellularly, LOXL2 has been reported to modify histone H3, stabilize SNAIL, and reduce cell polarity. Although LOXL2 promotes liver and lung fibrosis, little is known regarding its role in renal fibrosis. Read More

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http://dx.doi.org/10.1016/j.kint.2018.02.024DOI Listing
August 2018
6 Reads

Non-specific interstitial pneumonia associated with clinically amyopathic dermatomyositis showing "crazy paving" appearance on thin-section lung CT.

Respirol Case Rep 2018 Jul 3;6(5):e00326. Epub 2018 May 3.

Second Division, Department of Internal Medicine Hamamatsu University School of Medicine Hamamatsu Japan.

The "crazy paving" appearance consists of ground-glass opacity superimposed on a network of linear opacities on thin-section computed tomography (CT) images of the lung. This finding has been described in a variety of diseases but is extremely rare in patients with non-specific interstitial pneumonia (NSIP). We describe a 45-year-old woman with biopsy-proven NSIP associated with clinically amyopathic dermatomyositis that showed a "crazy paving" appearance on thin-section CT of the lung. Read More

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http://dx.doi.org/10.1002/rcr2.326DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5933249PMC
July 2018
28 Reads

Changes in the pattern of fibrosis in the rat lung with repetitive orotracheal instillations of gastric contents: evidence of persistent collagen accumulation.

Am J Physiol Lung Cell Mol Physiol 2018 09 10;315(3):L390-L403. Epub 2018 May 10.

Department of Respiratory Diseases and Medical Research Center, Pontificia Universidad Católica de Chile , Santiago , Chile.

Recurrent aspiration of gastric contents has been associated with several interstitial lung diseases. Despite this association, the pathogenic role of aspiration in these diseases has been poorly studied and little is known about extracellular matrix (ECM) changes in animal models of repetitive events of aspiration. Our aim was to study the repair phase of lung injury induced by each of several instillations of gastric fluid in Sprague-Dawley rats to evaluate changes in ECM and their reversibility. Read More

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https://www.physiology.org/doi/10.1152/ajplung.00559.2017
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http://dx.doi.org/10.1152/ajplung.00559.2017DOI Listing
September 2018
22 Reads

Leaf Extract Protects against Myocardial Injury via Attenuation of Endoplasmic Reticulum Stress in Streptozotocin-Induced Diabetic ApoE Mice.

Oxid Med Cell Longev 2018 25;2018:2370617. Epub 2018 Feb 25.

Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

Diabetes was induced in high-fat diet-fed ApoE mice via administration of low-dose streptozotocin (STZ) for five days. Mice were then treated with GBE (200 or 400 mg/kg) by gastric gavage daily for 12 weeks. Mice in the untreated diabetic group received saline instead, and nondiabetic C57BL/6J mice served as controls. Read More

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https://www.hindawi.com/journals/omcl/2018/2370617/
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http://dx.doi.org/10.1155/2018/2370617DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5845491PMC
September 2018
30 Reads

Performance of the St George's Respiratory Questionnaire in patients with connective tissue disease-associated interstitial lung disease.

Respirology 2018 Mar 25. Epub 2018 Mar 25.

Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Background And Objective: The St George's Respiratory Questionnaire (SGRQ) is a self-administered questionnaire used to assess health-related quality of life (HRQoL) in various chronic respiratory diseases. Few studies have assessed the performance of the SGRQ in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). We aimed to examine the SGRQ's performance characteristics and generate data to support its reliability and validity in patients with CTD-ILD. Read More

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http://dx.doi.org/10.1111/resp.13293DOI Listing
March 2018
21 Reads

miR-185 and miR-29a are similarly expressed in the bronchoalveolar lavage cells in IPF and lung cancer but common targets DNMT1 and COL1A1 show disease specific patterns.

Mol Med Rep 2018 May 19;17(5):7105-7112. Epub 2018 Mar 19.

Laboratory of Molecular and Cellular Pneumonology, Medical School, University of Crete, 71110 Heraklion, Crete, Greece.

Idiopathic pulmonary fibrosis (IPF) and lung cancer (LC) constitute two progressively devastating lung diseases with common risk factors including aging and smoking. There is an increasing interest in the investigation of common pathogenic mechanisms between IPF and LC with therapeutic implications. Several oncomirs, microRNAs associated with malignancy, are also linked with IPF. Read More

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http://dx.doi.org/10.3892/mmr.2018.8778DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5928671PMC
May 2018
21 Reads

Nintedanib reduces pulmonary fibrosis in a model of rheumatoid arthritis-associated interstitial lung disease.

Am J Physiol Lung Cell Mol Physiol 2018 06 15;314(6):L998-L1009. Epub 2018 Mar 15.

Program in Cell Biology, Department of Pediatrics, National Jewish Health , Denver, Colorado.

Rheumatoid arthritis (RA)-associated interstitial lung disease (RA-ILD) develops in ~20% of patients with RA. SKG mice, which are genetically prone to development of autoimmune arthritis, develop a pulmonary interstitial pneumonia that resembles human cellular and fibrotic nonspecific interstitial pneumonia. Nintedanib, a tyrosine kinase inhibitor approved for treatment of idiopathic pulmonary fibrosis, has been shown to reduce the decline in lung function. Read More

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http://dx.doi.org/10.1152/ajplung.00304.2017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335004PMC
June 2018
22 Reads

The Clinical and Immunologic Features of Patients With Combined Anti-GBM Disease and Castleman Disease.

Am J Kidney Dis 2018 06 3;71(6):904-908. Epub 2018 Mar 3.

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, China; Institute of Nephrology, Peking University, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China; Peking-Tsinghua Center for Life Sciences, Beijing, China.

Patients with both anti-glomerular basement membrane (anti-GBM) disease and Castleman disease have been rarely reported. In this study, we report 3 patients with this combination. They had immunologic features similar to patients with classic anti-GBM disease. Read More

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http://dx.doi.org/10.1053/j.ajkd.2018.01.029DOI Listing
June 2018
27 Reads

Interstitial lung disease in systemic autoimmune rheumatic diseases: a comprehensive review.

Expert Rev Clin Immunol 2018 01 11;14(1):69-82. Epub 2017 Dec 11.

b Rheumatology Unit, Department of Internal Medicine , ASST-Fatebenefratelli L. Sacco University Hospital , Milan , Italy.

Background: Interstitial lung diseases (ILDs) are among the most serious complications associated with systemic rheumatic diseases, and lead to significant morbidity and mortality; they may also be the first manifestation of connective tissue diseases (CTDs). The aim of this narrative review is to summarise the data concerning the pathogenesis of CTD/ILD and its distinguishing features in different rheumatic diseseas. Areas covered: The pathogenesis, clinical aspects and treatment of ILD associated with rheumatic systemic diseases and CTDs were reviewed by searching the PubMed, Medline, and Cochrane Library databases for papers published between 1995 and February 2017 using combinations of words or terms. Read More

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https://www.tandfonline.com/doi/full/10.1080/1744666X.2018.1
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http://dx.doi.org/10.1080/1744666X.2018.1411190DOI Listing
January 2018
116 Reads

Radiation toxicity in patients with collagen vascular disease and intrathoracic malignancy treated with modern radiation techniques.

Radiother Oncol 2017 11 5;125(2):301-309. Epub 2017 Nov 5.

Harvard Medical School, Boston, United States; Department of Radiation Oncology, Brigham and Women's Hospital/Dana-Farber Cancer Institute, Boston, United States. Electronic address:

Background And Purpose: There is concern that patients with collagen vascular disease (CVD) are at higher risk of developing radiation toxicity. We analyzed radiation toxicities in patients with intrathoracic malignancy and CVD treated using modern radiotherapy.

Materials And Methods: This single-institution retrospective study included 31 patients with CVD and 825 patients without CVD treated from 1998 to 2014. Read More

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http://dx.doi.org/10.1016/j.radonc.2017.10.002DOI Listing
November 2017
55 Reads

The role of periostin in lung fibrosis and airway remodeling.

Cell Mol Life Sci 2017 12 16;74(23):4305-4314. Epub 2017 Sep 16.

Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Michigan, 4053 BSRB, 109 Zina Pitcher Place, Ann Arbor, MI, 48109-2200, USA.

Periostin is a protein that plays a key role in development and repair within the biological matrix of the lung. As a matricellular protein that does not contribute to extracellular matrix structure, periostin interacts with other extracellular matrix proteins to regulate the composition of the matrix in the lung and other organs. In this review, we discuss the studies exploring the role of periostin to date in chronic respiratory diseases, namely asthma and idiopathic pulmonary fibrosis. Read More

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http://dx.doi.org/10.1007/s00018-017-2649-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5659879PMC
December 2017
49 Reads

Imipramine blocks acute silicosis in a mouse model.

Part Fibre Toxicol 2017 09 11;14(1):36. Epub 2017 Sep 11.

Center for Environmental Health Sciences, Department of Biomedical and Pharmaceutical Sciences, University of Montana, Missoula, MT, 59812, USA.

Background: Inhalation of crystalline silica is associated with pulmonary inflammation and silicosis. Although silicosis remains a prevalent health problem throughout the world, effective treatment choices are limited. Imipramine (IMP) is a FDA approved tricyclic antidepressant drug with lysosomotropic characteristics. Read More

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http://dx.doi.org/10.1186/s12989-017-0217-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5594487PMC
September 2017
106 Reads

Exploring efficacy and safety of oral Pirfenidone for progressive, non-IPF lung fibrosis (RELIEF) - a randomized, double-blind, placebo-controlled, parallel group, multi-center, phase II trial.

BMC Pulm Med 2017 Sep 6;17(1):122. Epub 2017 Sep 6.

Department of Internal Medicine II, University of Giessen-Marburg Lung Center, Justus-Liebig University Giessen, Giessen, Germany.

Background: Pirfenidone is currently approved in the EU for the treatment of mild to moderate idiopathic pulmonary fibrosis (IPF) and offers a beneficial risk-benefit profile. However, there are several other, progressive fibrotic lung diseases, in which conventional anti-inflammatory therapy is not sufficiently effective and antifibrotic therapies may offer a novel treatment option.

Methods/design: We designed a study protocol for inclusion of patients with progressive fibrotic lung disease despite conventional anti-inflammatory therapy (EudraCT 2014-000861-32). Read More

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http://dx.doi.org/10.1186/s12890-017-0462-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5588600PMC
September 2017
69 Reads

IPF lung fibroblasts have a senescent phenotype.

Am J Physiol Lung Cell Mol Physiol 2017 Dec 31;313(6):L1164-L1173. Epub 2017 Aug 31.

The Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, Pittsburgh, Pennsylvania;

The mechanisms of aging that are involved in the development of idiopathic pulmonary fibrosis (IPF) are still unclear. Although it has been hypothesized that the proliferation and activation of human lung fibroblasts (hLFs) are essential in IPF, no studies have assessed how this process works in an aging lung. Our goal was to elucidate if there were age-related changes on primary hLFs isolated from IPF lungs compared with age-matched controls. Read More

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http://dx.doi.org/10.1152/ajplung.00220.2017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6148001PMC
December 2017
48 Reads

Gene expression profiling of idiopathic interstitial pneumonias (IIPs): identification of potential diagnostic markers and therapeutic targets.

BMC Med Genet 2017 08 18;18(1):88. Epub 2017 Aug 18.

Department of Molecular and Internal Medicine, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.

Background: Chronic fibrosing idiopathic interstitial pneumonia (IIP) is characterized by alveolar epithelial damage, activation of fibroblast proliferation, and loss of normal pulmonary architecture and function. This study aims to investigate the genetic backgrounds of IIP through gene expression profiling and pathway analysis, and to identify potential biomarkers that can aid in diagnosis and serve as novel therapeutic targets.

Methods: RNA extracted from lung specimens of 12 patients with chronic fibrosing IIP was profiled using Illumina Human WG-6 v3 BeadChips, and Ingenuity Pathway Analysis was performed to identify altered functional and canonical signaling pathways. Read More

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http://dx.doi.org/10.1186/s12881-017-0449-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5562997PMC
August 2017
96 Reads

Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features.

BMC Pulm Med 2017 Aug 14;17(1):111. Epub 2017 Aug 14.

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

Background: To identify the prognostic factors for survival in patients with interstitial pneumonia with autoimmune features (IPAF) who meet the serological domain of the IPAF criteria.

Methods: We retrospectively analysed 99 IPAF patients who met the serological domain and were hospitalised at the Respiratory Medicine Unit of Kurashiki Central Hospital from 1999 to 2015. The high-resolution computed tomography findings were usual interstitial pneumonia (UIP; n = 1), non-specific interstitial pneumonia (NSIP; n = 63), NSIP with organizing pneumonia (OP) overlap (n = 15), and OP (n = 20). Read More

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http://dx.doi.org/10.1186/s12890-017-0453-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5554971PMC
August 2017
52 Reads

Fibrocyte measurement in peripheral blood correlates with number of cultured mature fibrocytes in vitro and is a potential biomarker for interstitial lung disease in Rheumatoid Arthritis.

Respir Res 2017 07 18;18(1):141. Epub 2017 Jul 18.

Department Clinical Immunology, Odense University Hospital, Odense, Denmark.

Background: Interstitial lung disease (ILD) can be a severe extra-articular disease manifestation in Rheumatoid Arthritis (RA). A potential role of fibrocytes in RA associated ILD (RA-ILD) has not previously been described. We present a modified faster method for measuring circulating fibrocytes, without intracellular staining. Read More

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http://dx.doi.org/10.1186/s12931-017-0623-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5516315PMC
July 2017
11 Reads

Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view.

Korean J Intern Med 2017 Jul 30;32(4):600-610. Epub 2017 Jun 30.

Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soon Chun Hyang University Seoul Hospital, Seoul, Korea.

Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). Read More

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http://dx.doi.org/10.3904/kjim.2016.212DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5511941PMC
July 2017
86 Reads

Elevated serum D-dimer level is associated with an increased risk of acute exacerbation in interstitial lung disease.

Respir Med 2017 07 19;128:78-84. Epub 2017 May 19.

Division of Pulmonary, Critical Care and Sleep Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, United States.

Background: Early recognition of patients with interstitial lung disease (ILD) who have an increased risk of developing acute exacerbation (AE) or preclinical AE may be clinically useful, since AE is associated with poor outcome and preventive measures would be of interest to ILD researchers. This study evaluated the relationship between elevated serum D-dimer level (≥0.4 mcg/mL) and subsequent AE or preclinical AE in patients with ILD. Read More

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http://dx.doi.org/10.1016/j.rmed.2017.05.009DOI Listing
July 2017
10 Reads
3.090 Impact Factor