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https://www.readbyqxmd.com/read/28935667/upregulation-of-herv-k-is-linked-to-immunity-and-inflammation-in-pulmonary-arterial-hypertension
#1
Toshie Saito, Kazuya Miyagawa, Shih-Yu Chen, Rasa Tamosiuniene, Lingli Wang, Orr Sharp, Erik Samayoa, Daisuke Harada, Jan-Renier A J Moonen, Aiqin Cao, Pin-I Chen, Jan K Hennigs, Mingxia Gu, Caiyun G Li, Ryan D Leib, Dan Li, Christopher M Adams, Patricia A Del Rosario, Matthew A Bill, Francois Haddad, Jose G Montoya, William Robinson, Wendy J Fantl, Garry P Nolan, Roham T Zamanian, Mark R Nicolls, Charles Y Chiu, Maria E Ariza, Marlene Rabinovitch
Background -Immune dysregulation has been linked to occlusive vascular remodeling in pulmonary arterial hypertension (PAH) that is hereditary, idiopathic or associated with other conditions. Circulating autoantibodies, lung perivascular lymphoid tissue and elevated cytokines have been related to PAH pathogenesis but without clear understanding of how these abnormalities are initiated, perpetuated and connected in the progression of disease. We therefore set out to identify specific target antigens in PAH lung immune complexes as a starting point toward resolving these issues to better inform future application of immunomodulatory therapies...
September 21, 2017: Circulation
https://www.readbyqxmd.com/read/28935460/myocardial-fibrosis-detected-by-magnetic-resonance-in-systemic-sclerosis-patients-relationship-with-biochemical-and-echocardiography-parameters
#2
Milan Hromádka, Jitka Seidlerová, David Suchý, Daniel Rajdl, Jan Lhotský, Jaroslav Ludvík, Richard Rokyta, Jan Baxa
OBJECTIVES: Systemic scleroderma (SSc) is a rare connective tissue disease presenting with fibrosis affecting skin and internal organs. Cardiovascular magnetic resonance (CMR) with quantification of extracellular volume (ECV) and T1 mapping might help to detect heart involvement. We aimed to evaluate whether myocardial involvement correlates with functional and laboratory parameters. METHODS: Thirty-three asymptomatic SSc patients (29 women, aged 56.6±12.2years) and 20 controls (10 women, 53...
September 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28926602/autoimmune-disease-mouse-model-exhibits-pulmonary-arterial-hypertension
#3
Koichi Sugimoto, Kazuhiko Nakazato, Akihiko Sato, Satoshi Suzuki, Akiomi Yoshihisa, Takeshi Machida, Shu-Ichi Saitoh, Hideharu Sekine, Yasuchika Takeishi
BACKGROUND: Pulmonary arterial hypertension is often associated with connective tissue disease. Although there are some animal models of pulmonary hypertension, an autoimmune disease-based model has not yet been reported. MRL/lpr mice, which have hypergammaglobulinemia, produce various autoimmune antibodies, and develop vasculitis and nephritis spontaneously. However, little is known about pulmonary circulation in these mice. In the present study, we examined the pulmonary arterial pressure in MRL/lpr mice...
2017: PloS One
https://www.readbyqxmd.com/read/28923440/interstitial-lung-disease-in-the-connective-tissue-diseases-a-paradigm-shift-in-diagnosis-and-treatment
#4
Paul F Dellaripa
Interstitial lung disease (ILD) in the connective tissue diseases (CTD) is amongst the most challenging aspect of care of patients with rheumatic diseases and is the source of significant morbidity and mortality. While there has been progress in our understanding of the natural history of these complications, we still suffer from a limited reservoir of data to confidently determine which patients are at highest risk for disease and those who are at highest risk for disease progression. Treatment options until recently have been limited to anti-inflammatory therapies but with the emerging availability of anti-fibrotic therapies, a shift in strategy is emerging to target therapies based on the specific radiographic, histopathologic features and biomarker profiles that are unique to patients with rheumatic diseases and ILD...
September 15, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28923086/usefulness-of-lung-ultrasound-b-lines-in-connective-tissue-disease-associated-interstitial-lung-disease-a-literature-review
#5
REVIEW
YuKai Wang, Luna Gargani, Tatiana Barskova, Dan E Furst, Marco Matucci Cerinic
Interstitial lung disease (ILD) is a major pulmonary manifestation of connective tissue disease (CTD), leading to significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) is presently considered the diagnostic gold standard for pulmonary fibrosis diagnosis and quantification in the clinical arena. However, not negligible doses of ionizing radiation limit the use of HRCT, especially for serial follow-up in younger female patients. In the past decade, lung ultrasound (LUS) has been proposed to assess ILD by detecting and quantifying sonographic B-lines...
September 18, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28921478/interstitial-pneumonia-with-autoimmune-features-an-additional-risk-factor-for-ards
#6
Giacomo Grasselli, Beatrice Vergnano, Maria Rosa Pozzi, Vittoria Sala, Gabriele D'Andrea, Vittorio Scaravilli, Marco Mantero, Alberto Pesci, Antonio Pesenti
BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) identifies a recently recognized autoimmune syndrome characterized by interstitial lung disease and autoantibodies positivity, but absence of a specific connective tissue disease diagnosis or alternative etiology. We retrospectively reviewed the clinical presentation, diagnostic workup and management of seven critically ill patients who met diagnostic criteria for IPAF. We compared baseline characteristics and clinical outcome of IPAF patients with those of the population of ARDS patients admitted in the same period...
September 18, 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#7
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
September 15, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28904861/a-modified-graft-versus-host-induced-model-for-systemic-sclerosis-with-pulmonary-fibrosis-in-rag2-deficient-mice
#8
Xue Yang, Chi Liu, Masayuki Fujino, Ji Yang, Xiao-Kang Li, Hejian Zou
Systemic sclerosis (SSc) is a connective tissue disease that results in fibrosis in multiple organs. Various animal models for this disease have been developed, both genetic and induced. One of the induced models, sclerodermatous graft-versus-host disease (scl-GvHD), exhibits the main characteristics of SSc, but involves lethal γ-irradiation of recipients. We sought to develop a modified scl-GvHD model. Spleen cells from B10.D2 donor mice were transplanted into immunodeficient Rag-2 recipients on the BALB/c genetic background...
September 2017: FEBS Open Bio
https://www.readbyqxmd.com/read/28893914/stenotrophomonas-maltophilia-serine-protease-stmpr1-induces-matrilysis-anoikis-and-protease-activated-receptor-2-activation-in-human-lung-epithelial-cells
#9
Ashley L DuMont, Nicholas P Cianciotto
Stenotrophomonas maltophilia is an emerging, opportunistic nosocomial pathogen that can cause severe disease in immunocompromised individuals. We recently identified the StmPr1 and StmPr2 serine proteases as substrates of the Xps type II secretion system in S. maltophilia strain K279a. Here, we report that a third serine protease, StmPr3, is also secreted in an Xps-dependent manner. By constructing a panel of protease mutants in strain K279s, we were able to determine that StmPr3 contributes to the previously described Xps-mediated rounding and detachment of the A549 human lung epithelial cell line as well as Xps-mediated degradation of fibronectin, fibrinogen, and the cytokine interleukin-8...
September 11, 2017: Infection and Immunity
https://www.readbyqxmd.com/read/28886479/high-sensitivity-hplc-method-for-determination-of-the-allysine-concentration-in-tissue-by-use-of-a-naphthol-derivative
#10
Philip A Waghorn, Bruno L Oliveira, Chloe M Jones, Andrew M Tager, Peter Caravan
Common to all fibrotic and metastatic diseases is the uncontrollable remodeling of tissue that leads to the accumulation of fibrous connective tissue components such as collagen and elastin. Build-up of fibrous tissue occurs through the cross-linking of collagen or elastin monomers, which is initiated through the oxidation of lysine residues to form α-aminoadipic-δ-semialdehyde (allysine). To provide a measure of the extent of collagen oxidation in disease models of fibrosis or metastasis, a rapid, sensitive HPLC method was developed to quantify the amount of allysine present in tissue...
August 26, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28886193/estimating-the-incidence-of-interstitial-lung-diseases-in-the-cree-of-eeyou-istchee-northern-qu%C3%A3-bec
#11
Matthieu Storme, Alexandre Semionov, Deborah Assayag, Michael Lefson, Darlene Kitty, David Dannenbaum, Jill Torrie, Pierre Lejeune, Elizabeth Robinson, Faiz Ahmad Khan
BACKGROUND: Little is known about the epidemiology of interstitial lung disease (ILD) amongst Canada's Indigenous populations. Clinicians working in Eeyou Istchee (the Cree territory of the James Bay region of Québec, population 17, 956) suspected that ILD was more common in this area. We sought to identify all prevalent and incident cases of ILD in Eeyou Istchee between 2006 and 2013, to describe characteristics of affected patients, distribution of subtypes, and estimate disease incidence...
2017: PloS One
https://www.readbyqxmd.com/read/28883249/pneumonitis-with-diffuse-alveolar-hemorrhage-induced-by-sho-seiryu-to
#12
Kazuo Tsuchiya, Mikio Toyoshima, Takafumi Suda
A 78-year-old man presented with acute-onset fever and dyspnea. He had been taking Sho-seiryu-to for allergic rhinitis. A chest radiograph showed diffuse bilateral ground-glass opacities with subpleural sparing, crazy-paving pattern, and traction bronchiectasis. The patient's bronchoalveolar lavage fluid was bloody and transbronchial lung biopsy specimens showed alveolitis, organizing pneumonia, and type 2 alveolar epithelial cell proliferation. There were no clinical and laboratory findings suggestive of respiratory tract infection or connective tissue disease...
September 6, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28878315/cholesterol-modified-hydroxychloroquine-loaded-nanocarriers-in-bleomycin-induced-pulmonary-fibrosis
#13
Li Liu, Jun Ren, Zhiyao He, Ke Men, Ye Mao, Tinghong Ye, Hua Chen, Ling Li, Bocheng Xu, Yuquan Wei, Xiawei Wei
An increasing number of reports have suggested the use of hydroxychloroquine (HCQ) as an adjunct anti-cancer treatment to enhance the chemotherapeutic response, as well as for the treatment of several fibrotic skin diseases and cystic fibrosis. In this study, we synthesized a cholesterol-modified HCQ (Chol-HCQ) and hypothesized that a systemic delivery system with Chol-HCQ nanocarriers could be effective for the treatment of bleomycin-induced pulmonary fibrosis. Chol-HCQ significantly inhibits the proliferation of rat lung fibroblasts, regulates inflammation and ameliorates bleomycin-induced pulmonary fibrosis in rats...
September 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28872085/-manifestations-of-the-connective-tissue-associated-interstitial-lung-disease-under-high-resolution-computed-tomography
#14
Hailan Li, Zeng Xiong, Jinkang Liu, Yisha Li, Bin Zhou
To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed.
 Results: The classifications for 127 patients were as follows: 36 cases of rheumatoid arthritis (28...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28872084/mortality-trend-of-inpatients-with-connective-tissue-diseases-2005-2014
#15
Liya Li, Xiaoxia Zuo, Hui Luo, Yisha Li, Yunhui You, Liping Duan, Weiru Zhang, Hongjun Zhao, Tong Li, Wangbin Ning, Yanli Xie, Sijia Liu, Xiaoyun Xie, Ying Jiang, Shiyao Wu, Honglin Zhu, Ya'ou Zhou
To analyze the trend relevant factors leading to death and their patterns over a 10-year period in inpatients with connective tissue diseases (CTDs).
 Methods: All clinical data about death in inpatients with CTDs were retrospectively reviewed between 2005 and 2014 at the Department of Rheumatology and Immunology in Xiangya Hospital of Central South University.
 Results: In the 10-year time period, the overall hospital mortality was 15.68‰. The disease itself accounted for 44.71% of the total causes of death, infection accounted for 42...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28855175/infections-and-respiratory-tract-disease-as-risk-factors-for-idiopathic-inflammatory-myopathies-a-population-based-case-control-study
#16
John Svensson, Marie Holmqvist, Ingrid E Lundberg, Elizabeth V Arkema
OBJECTIVES: To investigate the association between infection or respiratory tract disease and future risk of developing idiopathic inflammatory myopathy (IIM). METHODS: A case-control study was performed using Swedish nationwide registers. Adults with newly diagnosed IIM were identified (2002-2011) from the National Patient Register (NPR) and the Swedish Rheumatology Register (n=957). Controls were matched by age, sex and place of residence (n=9476). Outpatient visits and hospitalisations preceding IIM diagnosis indicating infection or respiratory disease were identified from NPR...
August 30, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28855174/the-euromyositis-registry-an-international-collaborative-tool-to-facilitate-myositis-research
#17
James B Lilleker, Jiri Vencovsky, Guochun Wang, Lucy R Wedderburn, Louise Pyndt Diederichsen, Jens Schmidt, Paula Oakley, Olivier Benveniste, Maria Giovanna Danieli, Katalin Danko, Nguyen Thi Phuong Thuy, Monica Vazquez-Del Mercado, Helena Andersson, Boel De Paepe, Jan L deBleecker, Britta Maurer, Liza J McCann, Nicolo Pipitone, Neil McHugh, Zoe E Betteridge, Paul New, Robert G Cooper, William E Ollier, Janine A Lamb, Niels Steen Krogh, Ingrid E Lundberg, Hector Chinoy
AIMS: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. METHODS: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. RESULTS: Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%)...
August 30, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28845373/first-case-report-of-metastatic-squamous-cell-carcinoma-of-lung-associated-with-mounier-kuhn-syndrome-and-review-of-literature
#18
Fatima Ayub, Muhammad W Saif
Mounier-Kuhn syndrome is a relatively rare condition, mostly involving the trachea and main stem bronchi. It is caused either by the atrophy of elastic fibers or faulty fetal development of cartilage and smooth muscles, hence leading to an overall increase in the diameter of lower respiratory tract. No certain etiology was found in the majority of cases reported previously, however, several other connective tissue diseases have also been implicated with the congenital tracheobronchomegaly. One anecdotal case report mentioned the development of lung malignancy in a patient who had previously received external beam radiotherapy...
June 26, 2017: Curēus
https://www.readbyqxmd.com/read/28830432/utility-of-autoimmune-serology-testing-in-the-assessment-of-uncharacterized-interstitial-lung-disease-a-large-retrospective-cohort-review
#19
Mohammad Alsumrain, Federica De Giacomi, Shireen Mirza, Teng Moua
BACKGROUND: Autoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD). Whether this practice is helpful in delineating connective-tissue disease related ILD (CTD-ILD) is not well known. We assessed the frequency of incident CTD-ILD as detected by autoimmune serology testing and presenting clinical signs and symptoms. METHODS: Consecutive patients seen at our institution over a four year period with newly diagnosed uncharacterized ILD and autoimmune serologic testing were included...
August 23, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28814849/tiotropium-bromide-exerts-anti-inflammatory-effects-during-resistive-breathing-an-experimental-model-of-severe-airway-obstruction
#20
Dimitrios Toumpanakis, Konstantinos Loverdos, Vassiliki Tzouda, Vyronia Vassilakopoulou, Eleni Litsiou, Christina Magkou, Vassiliki Karavana, Michael Pieper, Theodoros Vassilakopoulos
INTRODUCTION: Resistive breathing (RB), a hallmark of obstructive airway diseases, is characterized by strenuous contractions of the inspiratory muscles that impose increased mechanical stress on the lung. RB is shown to induce pulmonary inflammation in previous healthy animals. Tiotropium bromide, an anticholinergic bronchodilator, is also shown to exert anti-inflammatory effects. The effect of tiotropium on RB-induced pulmonary inflammation is unknown. METHODS: Adult rats were anesthetized, tracheostomized and breathed spontaneously through a two-way non-rebreathing valve...
2017: International Journal of Chronic Obstructive Pulmonary Disease
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