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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/28619061/rituximab-versus-cyclophosphamide-for-the-treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-recital-study-protocol-for-a-randomised-controlled-trial
#2
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
#3
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/28544856/recent-advances-in-connective-tissue-disease-related-interstitial-lung-disease
#4
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
#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/28377364/-clinical-significance-of-hypersensitive-c-reactive-protein-fribrinogen-and-d-dimmer-in-connective-tissue-disease-related-interstitial-lung-disease
#8
Si-Jie Yuan, Hai-Ting Xie, Zhong-Li Li
OBJECTIVE: To investigate the clinical significance of plasma levels of hypersensitive C-reactive protein (hs-CRP), fibriogen and D-dimmer (D-DI) in patients with connective tissue disease (CTD)-related interstitial lung disease (CTD-ILD). METHODS: Sixty-nine patients with interstitial lung disease admitted in Zhujiang Hospital between January, 2010 and April, 2016, including 29 with CTD-ILD and 40 with non-CTD-ILD were analyzed for plasma levels of hs-CRP, fibriogen and D-DI, with 25 healthy subjects as the control group...
March 20, 2017: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/28300570/idiopathic-interstitial-pneumonia-associated-with-autoantibodies-a-large-case-series-followed-over-one-year
#9
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/28274531/an-analysis-of-the-clinical-features-of-lung-cancer-in-patients-with-connective-tissue-diseases
#10
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/28213592/the-evidence-of-benefits-of-exercise-training-in-interstitial-lung-disease-a-randomised-controlled-trial
#11
Leona M Dowman, Christine F McDonald, Catherine J Hill, Annemarie L Lee, Kathryn Barker, Claire Boote, Ian Glaspole, Nicole S L Goh, Anne M Southcott, Angela T Burge, Rebecca Gillies, Alicia Martin, Anne E Holland
BACKGROUND: Uncertainty exists regarding the clinical relevance of exercise training across the range of interstitial lung diseases (ILDs). OBJECTIVE: To establish the impact of exercise training in patients with ILDs of differing aetiology and severity. METHODS: 142 participants with ILD (61 idiopathic pulmonary fibrosis (IPF), 22 asbestosis, 23 connective tissue disease-related ILD (CTD-ILD) and 36 with other aetiologies) were randomised to either 8 weeks of supervised exercise training or usual care...
February 17, 2017: Thorax
https://www.readbyqxmd.com/read/27977621/lung-cancer-development-in-patients-with-connective-tissue-disease-related-interstitial-lung-disease-a-retrospective-observational-study
#12
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/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/27876024/evaluation-of-computer-based-computer-tomography-stratification-against-outcome-models-in-connective-tissue-disease-related-interstitial-lung-disease-a-patient-outcome-study
#14
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/27684041/interstitial-lung-disease-in-india-results-of-a-prospective-registry
#15
Sheetu Singh, Bridget F Collins, Bharat B Sharma, Jyotsna M Joshi, Deepak Talwar, Sandeep Katiyar, Nishtha Singh, Lawrence Ho, Jai Kumar Samaria, Parthasarathi Bhattacharya, Rakesh Gupta, Sudhir Chaudhari, Tejraj Singh, Vijay Moond, Sudhakar Pipavath, Jitesh Ahuja, Ravindran Chetambath, Aloke G Ghoshal, Nirmal K Jain, H J Gayathri Devi, Surya Kant, Parvaiz Koul, Raja Dhar, Rajesh Swarnakar, Surendra K Sharma, Dhrubajyoti J Roy, Kripesh R Sarmah, Bhavin Jankharia, Rodney Schmidt, Santosh K Katiyar, Arpita Jindal, Daya K Mangal, Virendra Singh, Ganesh Raghu
RATIONALE: Interstitial lung disease (ILD) is a heterogeneous group of acute and chronic inflammatory and fibrotic lung diseases. Existing ILD registries have had variable findings. Little is known about the clinical profile of ILDs in India. OBJECTIVES: To characterize new-onset ILDs in India by creating a prospective ILD using multidisciplinary discussion (MDD) to validate diagnoses. METHODS: Adult patients of Indian origin living in India with new-onset ILD (27 centers, 19 Indian cities, March 2012-June 2015) without malignancy or infection were included...
March 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27421215/connective-tissue-disease-related-interstitial-lung-disease
#16
REVIEW
M Kristen Demoruelle, Shikha Mittoo, Joshua J Solomon
Interstitial lung disease (ILD) is commonly present in patients with an underlying connective tissue disease (CTD), particularly those with systemic sclerosis, rheumatoid arthritis, and inflammatory myositis. The clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. Distinguishing features in the clinical, radiographic, and histopathologic characteristics of CTD-ILD subsets can predict prognosis and treatment response. Treatment often consists of combinations of immunosuppressive medications, but there is a paucity of guidance in the literature to help clinicians determine appropriate screening and management of CTD-ILD...
February 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27309544/the-correlation-between-mir-200c-and-the-severity-of-interstitial-lung-disease-associated-with-different-connective-tissue-diseases
#17
Z Jiang, J-H Tao, T Zuo, X-M Li, G-S Wang, X Fang, X-L Xu, X-P Li
OBJECTIVES: To explore the correlation between microRNA (miR)-200c and the severity of interstitial lung disease (ILD) associated with connective tissue diseases (CTDs). METHOD: We recruited 218 patients with CTDs who were evaluated with high-resolution computed tomography (HRCT) and the pulmonary function test (PFT). Peripheral blood mononuclear cells (PBMCs) were acquired from 23 patients with systemic sclerosis (SSc), 29 with dermatomyositis/polymyositis (DM/PM), 30 with primary Sjögren's syndrome (pSS), 47 with rheumatoid arthritis (RA), and 23 normal controls to detect the expression level of miR-200c by quantitative reverse transcription polymerase chain reaction (QRT-PCR)...
March 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/27103387/characterisation-of-patients-with-interstitial-pneumonia-with-autoimmune-features
#18
Justin M Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N Husain, Steven Montner, Jonathan H Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij, Mary E Strek
Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint...
June 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27094018/mean-pulmonary-arterial-pressure-as-a-prognostic-indicator-in-connective-tissue-disease-associated-with-interstitial-lung-disease-a-retrospective-cohort-study
#19
Kota Takahashi, Hiroyuki Taniguchi, Masahiko Ando, Koji Sakamoto, Yasuhiro Kondoh, Naohiro Watanabe, Tomoki Kimura, Kensuke Kataoka, Atsushi Suzuki, Satoru Ito, Yoshinori Hasegawa
BACKGROUND: Pulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality. However, except for systemic sclerosis and mixed connective tissue disease, the impact of mean pulmonary arterial pressure (MPAP) on survival in CTD-ILD has not been sufficiently elucidated. We hypothesized that pulmonary arterial pressure may be a prognostic factor in CTD-ILDs regardless of the kind of CTD...
April 19, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27060110/rituximab-in-autoimmune-connective-tissue-disease-associated-interstitial-lung-disease
#20
Charles Sharp, Melanie McCabe, Nick Dodds, Anthony Edey, Lloyd Mayers, Huzaifa Adamali, Ann B Millar, Harsha Gunawardena
OBJECTIVE: CTD-associated interstitial lung disease (ILD) often fails to respond to conventional immunomodulatory agents. There is now considerable interest in the use of rituximab in systemic autoimmune CTD in patients refractory to standard treatments. The aim of this study was to review the experience of North Bristol NHS Trust managing patients with CTD-associated ILD with rituximab and explore possible associations with treatment response. METHODS: We conducted a retrospective analysis of all patients who received rituximab under the Bristol CTD-ILD service, having failed to respond to other immunomodulatory treatments...
July 2016: Rheumatology
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