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connective tissue disease and lung

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https://www.readbyqxmd.com/read/28642621/variants-of-the-abca3-gene-might-contribute-to-susceptibility-to-interstitial-lung-diseases-in-the-chinese-population
#1
Wei Zhou, Yi Zhuang, Jiapeng Sun, Xiaofen Wang, Qingya Zhao, Lizhi Xu, Yaping Wang
ATP-binding cassette A3 (ABCA3) is a phospholipid carrier that is mainly expressed in the alveolar epithelium. Biallelic mutations of ABCA3 has been associated with fatal respiratory distress syndrome and interstitial lung disease (ILD) in children. However, whether variations in ABCA3 have a role in the development of adult ILD, including idiopathic pulmonary fibrosis (IPF), remains to be addressed. In this study, we screened for germline variants of ABCA3 by exons-sequencing in 30 patients with sporadic IPF and in 30 matched healthy controls...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28631692/-multidisciplinary-problems-of-rheumatology-and-pulmonology
#2
L P Ananyeva
Damage to the respiratory system worsens prognosis and increases mortality in all systemic immunoinflammatory rheumatic diseases (SIRDs). Leading pulmonary manifestations in each disease are different, so the typical picture of each nosological entity is very peculiar. In all SIRDs, the disease starts with lung involvement in 10-20% of cases, preceding the expanded clinical presentation of a systemic disease. At a certain stage, lung damage may be the only manifestation of the disease or predominate in its clinical picture, determining the severity of a condition...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28619061/rituximab-versus-cyclophosphamide-for-the-treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-recital-study-protocol-for-a-randomised-controlled-trial
#3
Peter Saunders, Vicky Tsipouri, Gregory J Keir, Deborah Ashby, Marcus D Flather, Helen Parfrey, Daphne Babalis, Elisabetta A Renzoni, Christopher P Denton, Athol U Wells, Toby M Maher
BACKGROUND: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little...
June 15, 2017: Trials
https://www.readbyqxmd.com/read/28616281/clinicopathological-factors-influenced-the-prognosis-of-surgically-resected-pulmonary-pleomorphic-carcinoma
#4
Katsuhiro Okuda, Risa Oda, Ayumi Suzuki, Tadashi Sakane, Osamu Kawano, Hiroshi Haneda, Satoru Moriyama, Ryoichi Nakanishi
BACKGROUND: Pulmonary pleomorphic carcinoma has made an unfavorable prognosis because of its properties of resisting radiation and chemotherapy, and its aggressive growth. The correlation between clinicopathological factors and prognosis about pulmonary pleomorphic carcinoma patients who received its surgical resection has not been clearly identified. METHODS: We retrospectively investigated the clinical records of 24 pulmonary pleomorphic carcinoma patients who had a surgical resection from January 2004 to December 2013 at our institute...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28614301/microglia-dependent-synapse-loss-in-type-i-interferon-mediated-lupus
#5
Allison R Bialas, Jessy Presumey, Abhishek Das, Cees E van der Poel, Peter H Lapchak, Luka Mesin, Gabriel Victora, George C Tsokos, Christian Mawrin, Ronald Herbst, Michael C Carroll
Systemic lupus erythematosus (SLE) is an incurable autoimmune disease characterized by autoantibody deposition in tissues such as kidney, skin and lungs. Notably, up to 75% of patients with SLE experience neuropsychiatric symptoms that range from anxiety, depression and cognitive impairment to seizures and, in rare cases, psychosis-collectively this is referred to as central nervous system (CNS) lupus. In some cases, certain autoantibodies, such as anti-NMDAR or anti-phospholipid antibodies, promote CNS lupus...
June 14, 2017: Nature
https://www.readbyqxmd.com/read/28614213/pathologic-separation-of-chronic-hypersensitivity-pneumonitis-from-fibrotic-connective-tissue-disease-associated-interstitial-lung-disease
#6
Andrew Churg, Joanne L Wright, Christopher J Ryerson
Chronic (fibrotic) hypersensitivity pneumonitis (HP) and fibrosing interstitial pneumonias associated with connective tissue disease (CTD-ILD) can be difficult to distinguish in biopsy specimens. To investigate features that might separate these entities, 2 pathologists blinded to the diagnoses reviewed 16 cases of chronic HP and 12 cases of CTD-ILD. Fifteen predefined parameters were examined by morphometric point counting, analysis/cm of lung tissue, or presence/absence. Germinal centers were present in a minority of patients, but favored a diagnosis of CTD-ILD (7/12 CTD vs...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28597132/lung-involvement-in-stable-undifferentiated-connective-tissue-diseases-a-rheumatology-perspective
#7
Antonella Riccardi, Rosaria Irace, Ilaria Di Stefano, Michele Iudici, Serena Fasano, Marialuisa Bocchino, Annalisa Capaccio, Alessandro Sanduzzi, Gabriele Valentini
Previous studies of the occurrence of interstitial lung disease (ILD) in undifferentiated connective tissue diseases (UCTD) were conducted in patients admitted to Respiratory Medicine Units. The aim of the present prospective study was to investigate lung involvement in UCTD patients admitted to a Rheumatology Unit. Eighty-one consecutive UCTD patients were enrolled in the study. Each patient underwent history and physical examination, routine laboratory investigations, antinuclear antibody (ANA) profiling, B-mode echocardiography, and lung function study according to previously reported methods...
June 8, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28592713/pulmonary-limited-granulomatosis-with-polyangiitis-coexisting-with-mixed-connective-tissue-disease
#8
Masaru Ando, Akihiko Goto, Mari Yamasue, Yuko Usagawa, Hiroaki Oka, Takehiko Shigenaga, Jun-Ichi Kadota
Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28574387/-clinical-and-epidemiological-peculiarities-of-cystic-echinococcosis-in-children
#9
Kh Melia, N Kokaia, M Manjgaladze, T Kelbakiani-Kvinikhidze, G Sulaberidze
The postoperative period of cystic echinococcosis was studied in 13 children. Demographic, epidemiological, clinical diagnosis, treatment, number location, and development of cysts and serologic data were analyzed. Age of children at diagnosis range 5 to 17 years. All patients with cystic echinococcosis had abdominal cysts. The liver was the main organ involved in ten patients (76,9%) - they had cysts located in the liver, two patients (15,4%) had lung cyst, one patient had concomitant lung and liver cysts...
April 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28572155/elastin-receptor-s-gal-occupancy-by-elastin-peptides-modulates-t-cell-response-during-murine-emphysema
#10
Aïda Meghraoui-Kheddar, Alexandre Pierre, Mehdi Sellami, Sandra Audonnet, Flora Lemaire, Richard Le Naour
Chronic obstructive pulmonary disease and emphysema are associated with increased elastin peptides (EP) production due to excessive breakdown of lung connective tissue. We recently reported that exposure of mice to EP elicited hallmark features of emphysema. EP effects are largely mediated through a receptor complex which includes the elastin-binding protein S-gal. In previous studies, we established a correlation between cytokine production and S-gal protein expression in EP treated immune cells. In this study, we investigated the S-gal-dependent EP effects on T helper (Th) and T cytotoxic (Tc) responses during murine EP-triggered pulmonary inflammation...
June 1, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28552057/exploitation-of-novel-molecular-targets-to-treat-idiopathic-pulmonary-fibrosis-a-drug-discovery-perspective
#11
Bhuvaneshwar Vaidya, Ruaab Patel, Aaron Muth, Vivek Gupta
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease and is caused by excessive lung scarring. IPF-associated severe mortality can be attributed to late diagnosis due to its generic symptoms, and more importantly, due to the lack of effective therapies available. Despite extensive research in past decades, lung transplant still remains the most effective treatment for IPF. Though two drugs recently approved by FDA, Pirfenidone and Nintedanib, have shown an ability to reduce the progression of disease...
May 26, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28544856/recent-advances-in-connective-tissue-disease-related-interstitial-lung-disease
#12
Atsushi Suzuki, Yasuhiro Kondoh, Aryeh Fischer
Interstitial lung disease (ILD) is a common manifestation of connective tissue disease (CTD). Although the majority of patients with CTD-ILD are stable or slowly progressive, a significant group exhibits a more severe and progressive decline. Interstitial pneumonia with autoimmune features (IPAF) describes the subset of patients with interstitial pneumonia who have features suggesting underlying autoimmunity, but whose features fall short of a clear diagnosis of CTD. Areas covered: In this focused review, we discuss recent advances in early detection, prognostic evaluation, and management of autoimmune forms of ILD...
June 2, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28508461/foxp3-polymorphisms-in-interstitial-lung-disease-among-chinese-han-population-a-genetic-association-study
#13
Jianyu Yao, Tianze Zhang, Lili Zhang, Kaiyu Han, Linyou Zhang
INTRODUCTION: Both genetic and environmental factors are implicated in the pathogenesis of interstitial lung disease (ILD). Single-nucleotide polymorphisms (SNPs) in FOXP3 genes were implicated in the causation of some autoimmune diseases; however, association of these genes and ILD has not been reported. OBJECTIVES: To investigate whether FOXP3 polymorphisms are associated with ILD in a representative Chinese population. METHODS: One hundred and fifty-seven ILD patients and 170 healthy controls were recruited; SNPs were genotyped by the Sequenom MassARRAY platform and SHEsis was used to estimate the haplotype frequencies of SNPs...
May 15, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28496317/is-periodontitis-a-comorbidity-of-copd-or-can-associations-be-explained-by-shared-risk-factors-behaviors
#14
REVIEW
Stephanie Hobbins, Iain Lc Chapple, Elizabeth Sapey, Robert A Stockley
COPD is recognized as having a series of comorbidities potentially related to common inflammatory processes. Periodontitis is one of the most common human inflammatory diseases and has previously been associated with COPD in numerous observational studies. As periodontitis and COPD are both chronic, progressive conditions characterized by neutrophilic inflammation with subsequent proteolytic destruction of connective tissue, it has been proposed that they share common pathophysiological processes. The mechanisms proposed to link COPD and periodontitis include mechanical aspiration of oral contents into the respiratory tree, overspill of locally produced inflammatory mediators into the systemic circulation or oral or lung-derived bacteremia activating an acute-phase response and also reactive oxygen species (ROS) and cytokine release by systemic neutrophils at distant sites...
2017: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/28471958/association-of-serum-levels-of-laminin-type-iv-collagen-procollagen-iii-n-terminal-peptide-and-hyaluronic-acid-with-the-progression-of-interstitial-lung-disease
#15
Yiliang Su, Hongyan Gu, Dong Weng, Ying Zhou, Qiuhong Li, Fen Zhang, Yuan Zhang, Li Shen, Yang Hu, Huiping Li
Noninvasive and convenient tests to assess pulmonary fibrosis and disease progression in interstitial lung diseases (ILDs) are currently unavailable. The extracellular matrix molecules, laminin (LN), type IV collagen (IVC), procollagen III N-terminal peptide (PIIINP), and hyaluronic acid (HA) are involved in ILD development and progression. This study aims to investigate the association of disease progression and serum levels of LN, IVC, PIIINP, and HA in patients with ILD. This retrospective study included 323 patients (162 cases of idiopathic pulmonary fibrosis [IPF] and 161 cases of connective tissue diseases ILD [CTD-ILD]) treated in Shanghai Pulmonary Hospital between January 2013 and January 2015 and 160 healthy controls...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28471413/role-of-autoantibodies-in-the-diagnosis-of-connective-tissue-disease-ild-ctd-ild-and-interstitial-pneumonia-with-autoimmune-features-ipaf
#16
REVIEW
Adelle S Jee, Stephen Adelstein, Jane Bleasel, Gregory J Keir, MaiAnh Nguyen, Joanne Sahhar, Peter Youssef, Tamera J Corte
The diagnosis of interstitial lung disease (ILD) requires meticulous evaluation for an underlying connective tissue disease (CTD), with major implications for prognosis and management. CTD associated ILD (CTD-ILD) occurs most commonly in the context of an established CTD, but can be the first and/or only manifestation of an occult CTD or occur in patients who have features suggestive of an autoimmune process, but not meeting diagnostic criteria for a defined CTD-recently defined as "interstitial pneumonia with autoimmune features" (IPAF)...
May 4, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28467213/interstitial-pneumonia-with-autoimmune-features-value-of-histopathology
#17
Ayodeji Adegunsoye, Justin M Oldham, Eleanor Valenzi, Cathryn Lee, Leah J Witt, Lena Chen, Steven Montner, Jonathan H Chung, Imre Noth, Rekha Vij, Mary E Strek, Aliya N Husain
CONTEXT: - Patients with idiopathic interstitial pneumonia may display evidence of autoimmunity, without meeting criteria for a defined connective tissue disease. A recent European Respiratory Society/American Thoracic Society statement proposed research criteria for interstitial pneumonia with autoimmune features (IPAF), which includes findings from the clinical, serologic, and morphologic domains. OBJECTIVES: - To investigate the importance of histopathologic criteria within the morphologic domain and to report our methodology for identifying these features...
May 3, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28461123/the-long-term-outcome-of-interstitial-lung-disease-with-anti-aminoacyl-trna-synthetase-antibodies
#18
Kiminobu Tanizawa, Tomohiro Handa, Ran Nakashima, Takeshi Kubo, Yuji Hosono, Kizuku Watanabe, Kensaku Aihara, Kohei Ikezoe, Akihiko Sokai, Yoshinari Nakatsuka, Yoshio Taguchi, Kazuhiro Hatta, Satoshi Noma, Yoichiro Kobashi, Akihiko Yoshizawa, Toru Oga, Toyohiro Hirai, Kazuo Chin, Sonoko Nagai, Takateru Izumi, Tsuneyo Mimori, Michiaki Mishima
RATIONALE: Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined...
April 15, 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28424927/development-of-new-extra-glandular-manifestations-or-associated-auto-immune-diseases-after-establishing-the-diagnosis-of-primary-sj%C3%A3-gren-s-syndrome-a-long-term-study-of-the-antonius-nieuwegein-sj%C3%A3-gren-ans-cohort
#19
E J Ter Borg, J C Kelder
To investigate in a long-term study, the development of new extra-glandular manifestations (EGM) or associated auto-immune diseases (AID) from 1 year after establishing the diagnosis of primary Sjögren's syndrome (pSS). The primary goal was to examine the frequency and type of these manifestations and to find out which demographic, clinical and serological profile was most at risk. All outpatients diagnosed with primary Sjögren's syndrome were included in a retrospective study, with at least one check-up per year, from June 1991 until August 2015...
April 19, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28416697/small-molecule-mediated-inhibition-of-myofibroblast-transdifferentiation-for-the-treatment-of-fibrosis
#20
Michael J Bollong, Baiyuan Yang, Naja Vergani, Brittney A Beyer, Emily N Chin, Claudio Zambaldo, Danling Wang, Arnab K Chatterjee, Luke L Lairson, Peter G Schultz
Fibrosis, a disease in which excessive amounts of connective tissue accumulate in response to physical damage and/or inflammatory insult, affects nearly every tissue in the body and can progress to a state of organ malfunction and death. A hallmark of fibrotic disease is the excessive accumulation of extracellular matrix-secreting activated myofibroblasts (MFBs) in place of functional parenchymal cells. As such, the identification of agents that selectively inhibit the transdifferentiation process leading to the formation of MFBs represents an attractive approach for the treatment of diverse fibrosis-related diseases...
May 2, 2017: Proceedings of the National Academy of Sciences of the United States of America
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