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https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#1
REVIEW
Russell Edward Pellar, Janet Elizabeth Pope
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted...
December 9, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28079848/high-resolution-computed-tomography-to-differentiate-chronic-diffuse-infiltrative-lung-diseases-with-chronic-multifocal-consolidation-patterns-using-logical-analysis-of-data
#2
Constance De Margerie-Mellon, Geneviève Dion, Julien Darlay, Imene Ridene, Marianne Kambouchner, Nadia Brauner, Michel Brauner, Dominique Valeyre, Pierre-Yves Brillet
BACKGROUND: Chronic lung consolidation has a limited number of differential diagnoses requiring distinct managements. The aim of the study was to investigate how logical analysis of data (LAD) can support their diagnosis at HRCT (high-resolution computed tomography). METHODS: One hundred twenty-four patients were retrospectively included and classified into 8 diagnosis categories: sarcoidosis (n=35), connective tissue disease (n=21), adenocarcinoma (n=17), lymphoma (n=13), cryptogenic organizing pneumonia (n=11), drug-induced lung disease (n=9), chronic eosinophilic pneumonia (n =7) and miscellaneous (n=11)...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079847/relationship-between-abnormalities-on-high-resolution-computerized-tomography-pulmonary-function-and-bronchoalveolar-lavage-in-progressive-systemic-sclerosis
#3
Gulfidan Cakmak, Tuba Selcuk Can, Sule Gundogdu, Canan Akman, Hande Ikitimur, Benan Musellim, Gul Ongen
:  Introduction and aim: Progressive systemic sclerosis (pSS) is a multisystemic connective tissue disease characterized by fibrosis of the skin and internal organs including lung. The mechanisms that leads to progressive lung fibrosis in scleroderma remain obscure. In this study, we aimed to investigate the correlation between HRCT findings and patients' clinical and functional status and the degree of alveolitis based on the BAL resultsMaterials and methods: 65 patients with pSS were evaluated...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28063071/interstitial-lung-disease-in-systemic-sclerosis-current-and-future-treatment
#4
REVIEW
Roberto Giacomelli, Vasiliki Liakouli, Onorina Berardicurti, Piero Ruscitti, Paola Di Benedetto, Francesco Carubbi, Giuliana Guggino, Salvatore Di Bartolomeo, Francesco Ciccia, Giovanni Triolo, Paola Cipriani
Systemic sclerosis (SSc) has the highest fatality rate among connective tissue diseases and is characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) frequently complicates SSc and can be a debilitating disorder with a poor prognosis. ILD is the most frequent cause of death in SSc, and the management of SSc-ILD patients is a great challenge. Early detection of pulmonary involvement based on a recent decline of lung function tests and on the extent of lung involvement at high-resolution computed tomography is critical for the best management of these patients...
January 6, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28043593/new-approaches-in-drug-treatment-for-tuberculosis-inhalation-using-liposomes-only-a-future-vision-or-soon-in-clinical-practice
#5
Lars-Olof Larsson
A major change of therapy in respiratory medicine has been the transition from oral or parenteral to inhalation therapy, for example, in asthma. Inhalation of anti-infectious drugs has however not a key-role in the treatment of pulmonary infections such as tuberculosis (TB). The inhalation therapy provides several benefits; the target is reached directly with evasion of first-pass metabolism, thereby resulting in reduced systemic side effects. Furthermore, the drug is delivered to an extensive surface area that is rich in lymphoid tissue...
December 2016: International Journal of Mycobacteriology
https://www.readbyqxmd.com/read/28026993/connective-tissue-growth-factor-promotes-pulmonary-epithelial-cell-senescence-and-is-associated-with-copd-severity
#6
Jun-Ho Jang, Hitendra S Chand, Shannon Bruse, Melanie Doyle-Eisele, Christopher Royer, Jacob McDonald, Clifford Qualls, Aloysius J Klingelhutz, Yong Lin, Rama Mallampalli, Yohannes Tesfaigzi, Toru Nyunoya
The purpose of this study was to determine whether expression of connective tissue growth factor (CTGF) protein in chronic obstructive pulmonary disease (COPD) is consistent in humans and animal models of COPD and to investigate the role of this protein in lung epithelial cells. CTGF in lung epithelial cells of ex-smokers with COPD was compared with ex-smokers without COPD by immunofluorescence. A total of twenty C57Bl/6 mice and sixteen non-human primates (NHPs) were exposed to cigarette smoke (CS) for 4 weeks...
December 27, 2016: COPD
https://www.readbyqxmd.com/read/28005084/-comorbidity-diseases-in-adults-with-diagnosed-interstitial-lung-diseases-among-inhabitants-of-the-silesian-voivodeship-poland
#7
Ewa Niewiadomska, Małgorzata Kowalska, Jan E Zejda
BACKGROUND: Interstitial lung diseases form a group of chronic diseases associated with a significant worsening of the quality of life. Proper management of these diseases involves the recognition and treatment of comorbidities, so it implies high direct and indirect costs of therapy. The lack of epidemiological data on the total incidence of interstitial diseases in Poland, as well as of information on their increasing incidence in other European countries justify investigations into epidemiological situation in the Silesian voivodeship (the southern region of Poland)...
December 22, 2016: Medycyna Pracy
https://www.readbyqxmd.com/read/27979657/undifferentiated-connective-tissue-disease-and-interstitial-lung-disease-trying-to-define-patterns
#8
María Laura Alberti, Francisco Paulin, Heidegger Mateos Toledo, Martín Eduardo Fernández, Fabián Matías Caro, Jorge Rojas-Serrano, Mayra Edith Mejía
OBJECTIVES: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. METHODS: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns)...
December 12, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27977621/lung-cancer-development-in-patients-with-connective-tissue-disease-related-interstitial-lung-disease-a-retrospective-observational-study
#9
Yasunori Enomoto, Naoki Inui, Katsuhiro Yoshimura, Koji Nishimoto, Kazutaka Mori, Masato Kono, Tomoyuki Fujisawa, Noriyuki Enomoto, Yutaro Nakamura, Toshihide Iwashita, Takafumi Suda
Previous studies have reported that patients with idiopathic pulmonary fibrosis occasionally develop lung cancer (LC). However, in connective tissue disease (CTD)-related interstitial lung disease (ILD), there are few data regarding the LC development. The aim of the present study was to evaluate the clinical significance of LC development in patients with CTD-ILD. A retrospective review of our database of 562 patients with ILD between 2000 and 2014 identified 127 patients diagnosed with CTD-ILD. The overall and cumulative incidences of LC were calculated...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27919567/splicing-factor-proline-glutamine-rich-is-a-novel-autoantigen-of-dermatomyositis-and-associated-with-anti-melanoma-differentiation-associated-gene-5-antibody
#10
Yuji Hosono, Ran Nakashima, Satoshi Serada, Kosaku Murakami, Yoshitaka Imura, Hajime Yoshifuji, Koichiro Ohmura, Tetsuji Naka, Tsuneyo Mimori
OBJECTIVE: Anti-MDA5 antibody positive dermatomyositis (DM) and clinically amyopathic DM (CADM) often develop into rapidly progressive interstitial lung disease, but their pathogenesis remains unclear. We observed that sera from DM/CADM patients immunoprecipitated a common 110 kDa polypeptide. We investigated this autoantigen and its clinical significance. METHODS: Autoantibodies were screened in 333 patients with various connective tissue diseases (CTDs) and 20 healthy controls (HCs) by immunoprecipitation with [(35)S]methionine-labeled HeLa cells...
December 2, 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27912147/inhibition-of-endocan-attenuates-monocrotaline-induced-connective-tissue-disease-related-pulmonary-arterial-hypertension
#11
Haiyan Zhao, Yunxin Xue, Yun Guo, Yue Sun, Dongmei Liu, Xiaofei Wang
Connective tissue disease related pulmonary arterial hypertension (CTD-PAH) is characterized by vascular remodeling, endothelial dysfunction and inflammation. Endocan is a novel endothelial dysfunction marker. The aim of the present study was to investigate the role of endocan in CTD-PAH. Monocrotaline (MCT)-induced PAH rats were used as the CTD-PAH model. Short hairpin RNA packed in a lentiviral vector used to inhibit endocan expression was intratracheally instilled in rats prior to the MCT injection. Endocan was found to be increased in the serum and lung of MCT-induced PAH rats...
January 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/27894999/matched-comparative-modeling-of-normal-and-diseased-human-airway-responses-using-a-microengineered-breathing-lung-chip
#12
Kambez H Benam, Richard Novak, Janna Nawroth, Mariko Hirano-Kobayashi, Thomas C Ferrante, Youngjae Choe, Rachelle Prantil-Baun, James C Weaver, Anthony Bahinski, Kevin K Parker, Donald E Ingber
Smoking represents a major risk factor for chronic obstructive pulmonary disease (COPD), but it is difficult to characterize smoke-induced injury responses under physiological breathing conditions in humans due to patient-to-patient variability. Here, we show that a small airway-on-a-chip device lined by living human bronchiolar epithelium from normal or COPD patients can be connected to an instrument that "breathes" whole cigarette smoke in and out of the chips to study smoke-induced pathophysiology in vitro...
November 23, 2016: Cell Systems
https://www.readbyqxmd.com/read/27888985/azathioprine-response-in-patients-with-fibrotic-connective-tissue-disease-associated-interstitial-lung-disease
#13
Justin M Oldham, Cathryn Lee, Eleanor Valenzi, Leah J Witt, Ayodeji Adegunsoye, Scully Hsu, Lena Chen, Steven Montner, Jonathan H Chung, Imre Noth, Rekha Vij, Mary E Strek
BACKGROUND: Azathioprine is a commonly prescribed therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). Combination therapy that included azathioprine was recently shown to increase the risk of death and hospitalization in patients with idiopathic pulmonary fibrosis. Whether azathioprine increases the risk of adverse outcomes in patients with fibrotic CTD-ILD, including those with CTD-associated usual interstitial pneumonia (UIP), remains unknown. METHODS: A retrospective cohort analysis was performed to determine the combined incidence rate of death, transplant and respiratory hospitalization associated with azathioprine exposure...
December 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27878317/-what-is-currently-available-for-sport-medicine-therapy
#14
U Tegtbur
After 30 years of age physical capacity decreases with increasing age by 5-20% per decade. High physical activity in daily life as well as exercise training of endurance, strength, coordination and mobility can delay the functional and anatomical loss of muscle, bone, cartilage and connective tissue by more than 10 years. In recent years, numerous concepts have scientifically been proven in the exercise therapy of internal diseases; therefore, similar to drug treatment, cellular mechanisms of exercise training adaptation are known in detail...
December 2016: Der Internist
https://www.readbyqxmd.com/read/27876024/evaluation-of-computer-based-computer-tomography-stratification-against-outcome-models-in-connective-tissue-disease-related-interstitial-lung-disease-a-patient-outcome-study
#15
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Anne Laure Brun, Ryoko Egashira, Ronald Karwoski, Maria Kokosi, Athol U Wells, David M Hansell
BACKGROUND: To evaluate computer-based computer tomography (CT) analysis (CALIPER) against visual CT scoring and pulmonary function tests (PFTs) when predicting mortality in patients with connective tissue disease-related interstitial lung disease (CTD-ILD). To identify outcome differences between distinct CTD-ILD groups derived following automated stratification of CALIPER variables. METHODS: A total of 203 consecutive patients with assorted CTD-ILDs had CT parenchymal patterns evaluated by CALIPER and visual CT scoring: honeycombing, reticular pattern, ground glass opacities, pulmonary vessel volume, emphysema, and traction bronchiectasis...
November 23, 2016: BMC Medicine
https://www.readbyqxmd.com/read/27875728/exercise-induced-pulmonary-hypertension-by-stress-echocardiography-prevalence-and-correlation-with-right-heart-hemodynamics
#16
Deepika Misra, Ante Kendes, Roxana Sulica, Blase Carabello
OBJECTIVES: The aim of this study was to determine the prevalence of exercise-induced pulmonary hypertension (EIPH) in consecutive subjects referred for stress echocardiography for chest pain or shortness of breath and correlate echocardiographic diagnosis of EIPH with hemodynamics at right heart catheterization (RHC). BACKGROUND: Elevated pulmonary pressure can lead to significant morbidity and mortality. EIPH by ehocardiography has been described in patients with connective tissue disease...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27865876/interstitial-lung-disease-in-the-elderly
#17
Karen C Patterson, Rupal J Shah, Mary K Porteous, Jason D Christie, Carly D'Errico, Matthew Chadwick, Matthew Triano, Charuhas Deshpande, Milton D Rossman, Leslie A Litzky, Maryl E Kreider, Wallace T Miller
BACKGROUND: Despite the relationship of idiopathic pulmonary fibrosis (IPF) with advancing age, little is known about the epidemiology of interstitial lung diseases (ILD) in the elderly. Here we describe the diagnoses, clinical characteristics, and outcomes of patients who were elderly at the time of ILD diagnosis. METHODS: Among subjects from a prospective cohort study of ILD, elderly was defined as age > 70. Diagnoses were derived from a multi-disciplinary review...
November 16, 2016: Chest
https://www.readbyqxmd.com/read/27815256/toll-like-receptor-4-activation-attenuates-profibrotic-response-in-control-lung-fibroblasts-but-not-in-fibroblasts-from-patients-with-ipf
#18
Simone Ebener, Sandra Barnowski, Carlos Wotzkow, Thomas M Marti, Elena Lopez-Rodriguez, Bruno Crestani, Fabian Blank, Ralph A Schmid, Thomas Geiser, Manuela Funke
Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease with a median survival of 3 yr. IPF deteriorates upon viral or bacterial lung infection although pulmonary infection (pneumonia) in healthy lungs rarely induces fibrosis. Bacterial lipopolysaccharide (LPS) activates Toll-like receptor 4 (TLR4), initiating proinflammatory pathways. As TLR4 has already been linked to hepatic fibrosis and scleroderma, we now investigated the role of TLR4 in IPF fibroblasts. Lung tissue sections from patients with IPF were analyzed for TLR4 expression...
January 1, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/27809827/acute-exacerbation-of-ipf-has-systemic-consequences-with-multiple-organ-injury-with-sra-and-tnf-%C3%AE-cells-in-the-systemic-circulation-playing-central-roles-in-multiple-organ-injury
#19
Iwao Emura, Hiroyuki Usuda
BACKGROUND: The pathophysiologic mechanisms underlying acute exacerbation of idiopathic pulmonary fibrosis (IPF) are not fully understood. Few studies have examined autopsy findings in patients who have died from an acute exacerbation of IPF. The pathologic findings in systemic organs have not been described. METHODS: We retrospectively reviewed the autopsy findings in 12 patients who had died from an acute exacerbation of IPF and two of connective tissue disease- associated interstitial lung disease between 2005 and 2015...
November 3, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27803128/stat-mutations-as-program-switchers-turning-primary-immunodeficiencies-into-autoimmune-diseases
#20
REVIEW
Tiziana Lorenzini, Laura Dotta, Mauro Giacomelli, Donatella Vairo, Raffaele Badolato
STAT proteins are a family of transcription factors that mediate cellular response to cytokines and growth factors. Study of patients with familial susceptibility to pathogens and/or autoimmune diseases has led to the identification of 7 inherited disorders that are caused by mutations of 4 STAT family genes. Homozygous or compound heterozygous mutations of STAT1 lead to complete or partial forms of STAT1 deficiency that are associated with susceptibility to intracellular pathogens and herpetic infections. Patients with heterozygous STAT1 gain-of-function (GOF) mutations usually present with chronic mucocutaneous candidiasis (CMC) but may also experience bacterial and viral infections, autoimmune manifestations, lymphopenia, cerebral aneurysms, and increased risk to develop tumors...
January 2017: Journal of Leukocyte Biology
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