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connective tissue disease-interstitial lung disease associated

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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/28614213/pathologic-separation-of-chronic-hypersensitivity-pneumonitis-from-fibrotic-connective-tissue-disease-associated-interstitial-lung-disease
#4
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/28508461/foxp3-polymorphisms-in-interstitial-lung-disease-among-chinese-han-population-a-genetic-association-study
#5
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/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
#6
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
#7
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/28461123/the-long-term-outcome-of-interstitial-lung-disease-with-anti-aminoacyl-trna-synthetase-antibodies
#8
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
#9
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...
July 2017: Rheumatology International
https://www.readbyqxmd.com/read/28350485/ct-findings-radiologic-pathologic-correlation-and-imaging-predictors-of-survival-for-patients-with-interstitial-pneumonia-with-autoimmune-features
#10
Jonathan H Chung, Steven M Montner, Ayodeji Adegunsoye, Cathryn Lee, Justin M Oldham, Aliya N Husain, Heber MacMahon, Imre Noth, Rekha Vij, Mary E Strek
OBJECTIVE: The objective of this study is to determine the CT findings and patterns of interstitial pneumonia with autoimmune features (IPAF) and to assess whether imaging can predict survival for patients with IPAF. MATERIALS AND METHODS: The study included 136 subjects who met the criteria for IPAF and had diagnostic-quality chest CT scans obtained from 2006 to 2015; a total of 74 of these subjects had pathologic samples available for review within 1 year of chest CT examination...
March 28, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28339528/the-utility-of-comprehensive-autoantibody-testing-to-differentiate-connective-tissue-disease-associated-and-idiopathic-interstitial-lung-disease-subgroup-cases
#11
Caroline V Cotton, Lisa G Spencer, Robert P New, Robert G Cooper
Interstitial lung disease (ILD) comprises many heterogeneous disease groups, the largest being CTD-associated and those labelled as idiopathic out of necessity. The mechanisms causing ILD are poorly understood, but most CTD- and idiopathic-ILD cases can respond to immunosuppression, clearly suggesting a pathological role for inflammation. By contrast, corticosteroid immunosuppression causes harm without benefit in the feared idiopathic pulmonary fibrosis, suggesting that inflammation plays little pathological role, and where ILD progresses rapidly to lethal outcome even with anti-fibrotic drug use...
October 20, 2016: Rheumatology
https://www.readbyqxmd.com/read/28334541/-autoantibodies-in-systemic-connective-tissue-disease-and-anca-associated-vasculitis-their-relationship-to-interstitial-lung-diseases-and-prognosis
#12
Martina Doubková, Jana Pokorná
Lung disease, including interstitial lung disease (ILD), is a frequent complication of systemic connective tissue disorders (CTD) and ANCA (anti-neutrophil cytoplasmic antibody) associated vasculitis (AAV). Pulmonary manifestations are prognostic factor of CTDs and vasculitis. Autoantibodies assessment is a part of differential diagnosis algorithm of lung diseases. Autoantibodies importance is mainly clinical-diagnostic. Using detection of some autoantibodies it is possible to determine prognosis of lung involvement, especially in CTDs...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28323056/fty720-promotes-pulmonary-fibrosis-when-administered-during-the-remodelling-phase-following-a-bleomycin-induced-lung-injury
#13
David R Gendron, Anne-Marie Lemay, Pascale Blais Lecours, Valérie Perreault-Vallières, Carole-Ann Huppé, Ynuk Bossé, Marie-Renée Blanchet, Geneviève Dion, David Marsolais
Fibrosis complicates numerous pathologies including interstitial lung diseases. Sphingosine analogs such as FTY720 can alleviate lung injury-induced fibrosis in murine models. Contradictorily, FTY720 also promotes in vitro processes normally leading to fibrosis and high doses in vivo foster lung fibrosis by enhancing vascular leakage into the lung. The goal of this study was to determine the effect of low doses of FTY720 on lung fibrosis triggered by an acute injury in mice. We first defined the time-boundaries delimiting the inflammatory and remodelling phases of an injury elicited by bleomycin based on neutrophil counts, total lung capacity and lung stiffness...
June 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28300570/idiopathic-interstitial-pneumonia-associated-with-autoantibodies-a-large-case-series-followed-over-one-year
#14
Bridget F Collins, Charles F Spiekerman, Megan A Shaw, Lawrence A Ho, Jennifer Hayes, Carolyn A Spada, Caroline M Stamato, Ganesh Raghu
BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia (IIP), particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF) or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: Retrospective single center study; ANOVA analyses compared differences in mean change in forced vital capacity (FVC) and diffusion capacity (DLCO) over 1-year among 124 well defined patients (20 AI-ILD [positive autoantibodies with or without symptoms of CTD], 15 IPAF, 36 CTD-ILD, 53 "Lone-IPF" [patients with IPF without any autoantibodies]) RESULTS: 75% of patients with AI-ILD, 33% IPAF, and 33% CTD-ILD had UIP...
March 11, 2017: Chest
https://www.readbyqxmd.com/read/28297158/unclassifiable-interstitial-lung-diseases-clinical-characteristics-and-survival
#15
Charlotte Hyldgaard, Elisabeth Bendstrup, Athol U Wells, Ole Hilberg
BACKGROUND AND OBJECTIVE: Unclassifiable disease in chronic interstitial lung disease (ILD) is a common and challenging problem but has been insufficiently studied. The 2013 update of the international multidisciplinary classification of the idiopathic interstitial pneumonias presented a classification based on observed disease behaviour with the purpose of providing guidance to clinicians in the management of these patients. The aim of this study was to apply the new disease behaviour classification (DBC) and the previously validated ILD-gender age physiology (GAP) score to a cohort of unclassifiable ILD patients and to assess the prognostic value of these two composite approaches...
April 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28279287/a-42-year-old-woman-with-anemia-shock-and-ischemic-stroke-after-lung-transplantation
#16
Abhishek Biswas, Ali Ataya, Juan C Salgado, Satish Chandrasekharan, Tiago N Machuca, Amir M Emtiazjoo
A 42-year-old woman with mixed connective tissue disease-associated interstitial lung disease underwent bilateral lung transplantation. She had an uneventful surgery and was extubated 3 h later. Induction immunosuppression therapy included methylprednisolone 500 mg intraoperatively, basiliximab (anti-IL-2 monoclonal antibody) on days 0 and 4 after transplantation, and methylprednisolone 125 mg intravenously bid for 2 days following surgery. Maintenance immunosuppression therapy consisted of prednisone 20 mg daily, mycophenolate mofetil 750 mg bid, and enteral tacrolimus 0...
March 2017: Chest
https://www.readbyqxmd.com/read/28275485/serum-kl-6-and-surfactant-protein-d-as-monitoring-and-predictive-markers-of-interstitial-lung-disease-in-patients-with-systemic-sclerosis-and-mixed-connective-tissue-disease
#17
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. METHODS: We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28274531/an-analysis-of-the-clinical-features-of-lung-cancer-in-patients-with-connective-tissue-diseases
#18
Atsuro Saijo, Masaki Hanibuchi, Hisatsugu Goto, Yuko Toyoda, Toshifumi Tezuka, Yasuhiko Nishioka
BACKGROUND: Patients with connective tissue diseases (CTDs) are at increased risk for lung cancer (LC); interstitial lung disease (ILD) is a common form of organ dysfunction in cases of CTD. However, the influence of ILD on the treatment and prognosis in LC patients with CTD is unclear. METHODS: Between January 2010 and December 2014, 27 patients among all patients with CTD at our institution were diagnosed with primary LC. We retrospectively analyzed the clinical features, treatment modalities, and outcomes of these patients, and evaluated the potential prognostic factors...
March 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28248800/mixed-connective-tissue-disease-and-epitope-spreading-an-historical-cohort-study
#19
Laura Escolà-Vergé, Iago Pinal-Fernandez, Andreu Fernandez-Codina, Eduardo L Callejas-Moraga, Juan Espinosa, Ana Marin, Moises Labrador-Horrillo, Albert Selva-O'Callaghan
OBJECTIVES: Mixed connective tissue disease (MCTD) is characterized by the presence of anti-U1-snRNP autoantibodies and a variable set of associated clinical features. Some MCTD patients test positive over time to autoantibodies against Sm, proteins spatially related with U1-snRNP. This situation has been attributed to expanding of the autoimmune response by a phenomenon known as epitope spreading. Our aim was to study the frequency of this phenomenon in MCTD patients and the specific clinical features of those with epitope spreading...
April 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28245857/the-diagnostic-value-of-the-bronchoalveolar-lavage-in-interstitial-lung-diseases
#20
Boubacar Efared, G Ebang-Atsame, Sani Rabiou, Abdoulsalam S Diarra, Layla Tahiri, Nawal Hammas, Mohamed Smahi, Bouchra Amara, Mohamed C Benjelloun, Mounia Serraj, Laila Chbani, Hinde El Fatemi
OBJECTIVE: Bronchoalveolar lavage (BAL) is a diagnostic tool often used during the management of interstitial lung diseases (ILD). However, its diagnostic value in discrimination between entities comprising the very heterogenous group of ILD, is still a controversial issue. The objective of our study is to assess the diagnostic value of BAL in the management of ILD, by comparing the cytological findings in BAL fluid among the different diseases of this group. METHODS: It was a retrospective, observational study of 151 patients between January 2012 and December 2015...
March 1, 2017: Journal of Negative Results in Biomedicine
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