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connective tissue disease-interstitial lung disease associated

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https://www.readbyqxmd.com/read/29916965/spontaneous-pneumomediastinum-in-connective-tissue-diseases
#1
Federica De Giacomi, Misbah Baqir, Christian W Cox, Teng Moua, Eric L Matteson, Jay H Ryu
BACKGROUND: Spontaneous pneumomediastinum (SP) is an uncommon disorder but has been described in cases with connective tissue diseases (CTDs), most commonly dermatomyositis and polymyositis. We aimed to explore this relationship by analyzing the characteristics of CTD patients with SP. METHODS: Using a computer-assisted search, we identified 25 patients with CTD and SP evaluated between January 1997 and December 2016 at our institution. Clinicoradiologic characteristics, treatment, and outcomes were extracted and chest computed tomography studies analyzed...
June 18, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29897157/could-quality-be-the-key-in-connective-tissue-disease-associated-interstitial-lung-disease
#2
EDITORIAL
Philip L Molyneaux, Toby M Maher
No abstract text is available yet for this article.
June 13, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29892541/lymphocytic-interstitial-pneumonia-in-a-patient-with-mixed-connective-tissue-disease-a-case-report
#3
Chin-Wei Kuo, Kung-Chao Chang, Han-Yu Chang, Tang-Hsiu Huang
Lymphocytic interstitial pneumonia (LIP) is an uncommon interstitial lung disease that is characterized by an interstitial infiltrate of lymphoplasmacytic cells. While idiopathic LIP appears to be extremely rare, most reported cases of LIP have been associated with coexisting immune derangements, particularly autoimmune diseases such as Sjögren's syndrome. In this report, we describe the presentation of LIP in a patient with underlying mixed connective tissue disease.
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29846166/vitamin-d-deficiency-in-connective-tissue-disease-associated-interstitial-lung-disease
#4
Mingting Deng, Lin Tang, Dongmei Huang, Zhongjie Wang, Junli Chen
OBJECTIVES: To determine and compare the prevalence of vitamin D deficiency in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). METHODS: The level of vitamin D was determined by the serum levels of 1,25(OH)2D3. We evaluated 144 patients in our study, including 53 subjects in the CTD-ILD group and 91 subjects in the CTD group without ILD. CTD was diagnosed following the American College of Rheumatology criteria, and ILD was diagnosed by high-resolution computed tomography...
May 24, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29777409/hemodynamic-heterogeneity-of-connective-tissue-disease-patients-with-borderline-mean-pulmonary-artery-pressure-and-its-distinctive-characters-from-those-with-normal-pulmonary-artery-pressure-a-retrospective-study
#5
Yusa Asari, Yoshioki Yamasaki, Kosei Tsuchida, Kengo Suzuki, Yoshihiro J Akashi, Takahiro Okazaki, Shoichi Ozaki, Hidehiro Yamada, Kimito Kawahata
To clarify whether patients with connective tissue disease (CTD)-associated borderline mean pulmonary artery pressure (mPAP) have distinctive hemodynamic characteristics from those with normal mPAP and whether pathogenesis is as heterogeneous as manifest pulmonary hypertension (PH). Seventy-five CTD patients who underwent right heart catheterization (RHC) from 2008 through 2016 were retrospectively analyzed. We compared between-group differences in clinical and hemodynamic findings: normal mPAP (n = 35), borderline mPAP (n = 15), and PH (n = 25)...
May 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29776440/radiological-pleuroparenchymal-fibroelastosis-associated-to-limited-cutaneous-systemic-sclerosis-a-case-report
#6
D Hassoun, S Dirou, P P Arrigoni, C Durant, M Hamidou, A Néel, C Agard
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease (ILD) characterized by progressive fibrotic lesions of the visceral pleura and the sub-pleural parenchyma, affecting predominantly the upper lobes. PPFE may occur in different contextes like bone marrow or lung transplantations, but also in the context of telomeropathy with mutations of telomerase reverse transcriptase (TERT), telomerase RNA component (TERC) or regulator of telomere elongation helicase 1 (RTEL1) genes...
May 18, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29773465/pulmonary-manifestations-in-systemic-lupus-erythematosus-pleural-involvement-acute-pneumonitis-chronic-interstitial-lung-disease-and-diffuse-alveolar-hemorrhage
#7
Georgina Aguilera-Pickens, Carlos Abud-Mendoza
Systemic lupus erythematosus is the diffuse autoimmune connective tissue disease that most frequently involves pulmonary involvement, affecting 20% of 90% of the patients. The percentage varies depending on the defining criteria (symptoms, pulmonary tests or histopathological studies). At least once during the disease course, 50% of those affected have pleural and/or pulmonary manifestations, which are associated with higher morbidity and mortality. Pulmonary involvement has no correlation with lupus activity biomarkers, and it is necessary to rule out infectious processes in the initial approach...
May 14, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29745875/interstitial-lung-disease-in-patients-with-mixed-connective-tissue-disease-pilot-study-on-predictors-of-lung-involvement
#8
Neha Narula, Tathagat Narula, Isabel Mira-Avendano, Benjamin Wang, Andy Abril
OBJECTIVES: Mixed connective tissue disease (MCTD) is an immune-mediated systemic disorder characterised by serum autoantibodies against U1-ribonucleoprotein and diverse multisystemic clinical manifestations. Approximately 50% of patients with MCTD develop a radiologic pattern of interstitial lung disease (ILD). Our single centre, cross-sectional study sought to identify clinical and serologic associations of ILD in patients with MCTD which may serve as predictors of lung disease and prognosis...
May 8, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29720509/state-of-the-art-in-interstitial-pneumonia-with-autoimmune-features-a-systematic-review-on-retrospective-studies-and-suggestions-for-further-advances
#9
REVIEW
Gianluca Sambataro, Domenico Sambataro, Sebastiano Emanuele Torrisi, Ada Vancheri, Mauro Pavone, Roberta Rosso, Matteo Schisano, Claudia Crimi, Francesca Pignataro, Aryeh Fischer, Nicoletta Del Papa, Carlo Vancheri
The term interstitial pneumonia with autoimmune features (IPAF) has been proposed to define patients with interstitial lung disease (ILD) associated with autoimmune signs not classifiable for connective tissue diseases (CTDs). This new definition overcomes previous nomenclatures and provides a uniform structure for prospective studies through specific classification criteria.This work evaluates the characteristics of IPAF patients reported in the literature, to highlight potential limits through a comparative analysis and to suggest better performing classification criteria...
June 30, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29693298/prognostic-factors-and-disease-behaviour-of-pathologically-proven-fibrotic-non-specific-interstitial-pneumonia
#10
Hideaki Yamakawa, Hideya Kitamura, Tamiko Takemura, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Tae Iwasawa, Eri Hagiwara, Shinji Sato, Takashi Ogura
BACKGROUND AND OBJECTIVE: Non-specific interstitial pneumonia (NSIP) has heterogeneous characteristics in terms of background, disease behaviour and prognosis. This study of fibrotic NSIP (f-NSIP) aimed to elucidate prognosis and disease behaviour from the viewpoint of clinical background and determine whether long-term change of pulmonary function could provide useful prognostic information. METHODS: We analysed the medical records of 157 consecutive patients diagnosed with f-NSIP by surgical lung biopsy...
April 24, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29667101/clinical-features-risk-factors-and-outcomes-of-patients-with-interstitial-pneumonia-with-autoimmune-features-a-population-based-study
#11
Jinghong Dai, Lei Wang, Xin Yan, Hui Li, Kefeng Zhou, Jian He, Fanqing Meng, Siyi Xu, Geyu Liang, Hourong Cai
To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint...
April 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29607151/lung-cancer-in-connective-tissue-disease-associated-interstitial-lung-disease-clinical-features-and-impact-on-outcomes
#12
Satoshi Watanabe, Keigo Saeki, Yuko Waseda, Akari Murata, Hazuki Takato, Yukari Ichikawa, Masahide Yasui, Hideharu Kimura, Yasuhito Hamaguchi, Takashi Matsushita, Kazunori Yamada, Mitsuhiro Kawano, Kengo Furuichi, Takashi Wada, Kazuo Kasahara
Backgrounds: Lung cancer (LC) adversely impacts survival in patients with idiopathic pulmonary fibrosis. However, little is known about LC in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). The aim of this study was to evaluate the prevalence of and risk factors for LC in CTD-ILD, and the clinical characteristics and survival of CTD-ILD patients with LC. Methods: We conducted a single-center, retrospective review of patients with CTD-ILD from 2003 to 2016...
February 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29595279/-systemic-sclerosis-in-2017
#13
Tomáš Soukup, Tomáš Veleta
Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29575410/performance-of-the-st-george-s-respiratory-questionnaire-in-patients-with-connective-tissue-disease-associated-interstitial-lung-disease
#14
Atsushi Suzuki, Yasuhiro Kondoh, Jeffrey J Swigris, Masahiko Ando, Tomoki Kimura, Kensuke Kataoka, Yasuhiko Yamano, Taiki Furukawa, Mari Numata, Koji Sakamoto, Yoshinori Hasegawa
BACKGROUND AND OBJECTIVE: The St George's Respiratory Questionnaire (SGRQ) is a self-administered questionnaire used to assess health-related quality of life (HRQoL) in various chronic respiratory diseases. Few studies have assessed the performance of the SGRQ in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). We aimed to examine the SGRQ's performance characteristics and generate data to support its reliability and validity in patients with CTD-ILD...
March 25, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29538197/prevalence-of-lung-cancer-in-patients-with-interstitial-lung-disease-is-higher-than-in-those-with-chronic-obstructive-pulmonary-disease
#15
Hye In Jung, Jae Seok Park, Mi-Young Lee, ByeongJu Park, Hyun Jung Kim, Sun Hyo Park, Won-Il Choi, Choong Won Lee
We aimed to explore lung cancer prevalence in interstitial lung disease (ILD) patients with or without connective tissue disorder (CTD) and idiopathic pulmonary fibrosis (IPF) in comparison with chronic obstructive pulmonary disorder (COPD).We evaluated lung cancer prevalence associated with ILD and IPF using Korean Health Insurance Review and Assessment Service (HIRA) data from January to December 2011. This database (HIRA-NPS-2011-0001) was sampled using random sampling of outpatients; 1,375,842 sample cases were collected, and 670,258 (age ≥ 40 ys) were evaluated...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29535639/vascular-endothelial-cell-specific-connective-tissue-growth-factor-ctgf-is-necessary-for-development-of-chronic-hypoxia-induced-pulmonary-hypertension
#16
Liya Pi, Chunhua Fu, Yuanquing Lu, Junmei Zhou, Marda Jorgensen, Vinayak Shenoy, Kenneth E Lipson, Edward W Scott, Andrew J Bryant
Chronic hypoxia frequently complicates the care of patients with interstitial lung disease, contributing to the development of pulmonary hypertension (PH), and premature death. Connective tissue growth factor (CTGF), a matricellular protein of the Cyr61/CTGF/Nov (CCN) family, is known to exacerbate vascular remodeling within the lung. We have previously demonstrated that vascular endothelial-cell specific down-regulation of CTGF is associated with protection against the development of PH associated with hypoxia, though the mechanism for this effect is unknown...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29501253/clinical-significance-of-respiratory-virus-detection-in-patients-with-acute-exacerbation-of-interstitial-lung-diseases
#17
Takeshi Saraya, Hirokazu Kimura, Daisuke Kurai, Masaki Tamura, Yukari Ogawa, Sunao Mikura, Mitsuru Sada, Miku Oda, Takayasu Watanabe, Kosuke Ohkuma, Manami Inoue, Kojiro Honda, Masato Watanabe, Takuma Yokoyama, Masachika Fujiwara, Haruyuki Ishii, Hajime Takizawa
BACKGROUND: The impact of viral infections on acute exacerbations in idiopathic pulmonary fibrosis (IPF) and/or non-IPF interstitial lung disease (ILDs) has been scarcely described. OBJECTIVES: To elucidate the frequency of virus infections in patients with IPF or non-IPF ILDs including idiopathic interstitial pneumonia (IIP) or connective tissue disease (CTD)-associated pneumonia, and its influence on their short-term mortality. METHODS: We prospectively enrolled adult patients with acute exacerbation of IPF and non-IPF ILDs who were admitted to the hospital during the last 3 years, and examined the respiratory samples obtained from nasopharyngeal, sputum, and bronchoalveolar lavage fluid...
March 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29474209/pulmonary-phenotypes-associated-with-genetic-variation-in-telomere-related-genes
#18
Thijs W Hoffman, Coline H M van Moorsel, Raphael Borie, Bruno Crestani
PURPOSE OF REVIEW: Genomic mutations in telomere-related genes have been recognized as a cause of familial forms of idiopathic pulmonary fibrosis (IPF). However, it has become increasingly clear that telomere syndromes and telomere shortening are associated with various types of pulmonary disease. Additionally, it was found that also single nucleotide polymorphisms (SNPs) in telomere-related genes are risk factors for the development of pulmonary disease. This review focuses on recent updates on pulmonary phenotypes associated with genetic variation in telomere-related genes...
May 2018: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/29445936/pulmonary-arterial-hemodynamic-assessment-by-a-novel-index-in-systemic-sclerosis-patients-pulmonary-pulse-transit-time
#19
Mehmet Dogan, Tolga Han Efe, Tolga Cimen, Cem Ozisler, Mehmet Ali Felekoglu, Ahmet Goktug Ertem, Mehmet Erat, Omer Yiginer, Murat Tulmac
OBJECTIVES: Systemic sclerosis (SSc) is a chronic, inflammatory, and autoimmune connective tissue disease that is associated with vascular lesions, and fibrosis of the skin and visceral organs. Cardiac complications may occur as a secondary effect of SSc as a result of pulmonary arterial hypertension and interstitial lung disease. The objective of this study was to assess whether the pulmonary pulse transit time (pPTT) could serve as a diagnostic marker for pulmonary arterial alterations in patients with SSc, prior to development of pulmonary hypertension...
April 2018: Lung
https://www.readbyqxmd.com/read/29359173/immune-myopathies-with-perimysial-pathology-clinical-and-laboratory-features
#20
Robert C Bucelli, Alan Pestronk
Objective: Immune myopathies with perimysial pathology (IMPP) have a combination of damage to perimysial connective tissue and muscle fiber necrosis, more prominent near the perimysium. We studied the clinical and laboratory correlates of patients with pathologically defined IMPP. Methods: This is a retrospective chart and pathology review of 57 consecutive patients with IMPP myopathology and, for comparison, 20 patients with dermatomyositis with vascular pathology (DM-VP)...
March 2018: Neurology® Neuroimmunology & Neuroinflammation
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