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interstitial lung disease on connective tissue disease

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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
#1
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/29773465/pulmonary-manifestations-in-systemic-lupus-erythematosus-pleural-involvement-acute-pneumonitis-chronic-interstitial-lung-disease-and-diffuse-alveolar-hemorrhage
#2
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
#3
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
#4
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
#5
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/29607151/lung-cancer-in-connective-tissue-disease-associated-interstitial-lung-disease-clinical-features-and-impact-on-outcomes
#6
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/29605286/pulmonary-hypertension-in-patients-with-interstitial-lung-disease
#7
REVIEW
Theodoros Karampitsakos, Argyrios Tzouvelekis, Serafeim Chrysikos, Demosthenes Bouros, Iraklis Tsangaris, Wassim H Fares
Interstitial lung diseases (ILDs) comprise a broad and heterogeneous group of more than two hundred diseases with common functional characteristics. Their diagnosis and management require a multidisciplinary approach. This multidisciplinary approach involves the assessment of comorbid conditions including pulmonary hypertension (PH) that exerts a dramatic impact on survival. The current World Health Organization (WHO) classification of PH encompasses many of the interstitial lung diseases into WHO Group 3, while sarcoidosis, Pulmonary Langerhans Cell Histiocytosis and lymphangioleiomyomatosis are placed into WHO Group 5 as diseases with unclear or multifactorial mechanisms...
March 29, 2018: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29601564/-nintedanib-in-the-treatment-of-fibrosing-interstital-lung-diseases
#8
REVIEW
Katarzyna Lewandowska
Nintedanib is an intracellular tyrosine kinase inhibitor approved in a treatment of idiopathic pulmonary fibrosis. It reduces the annual rate of forced vital capacity decline by approximately 50%, that results in slowing of disease progression. The drug also reduces the incidence of acute exacerbations of idiopathic pulmonary fibrosis. The efficacy of nintedanib was the same in different groups of patients, irrespectively to disease status, age, gender and race. The most frequent side effects were gastrointestinal, i...
March 27, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29595279/-systemic-sclerosis-in-2017
#9
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/29595014/-invasive-diagnostic-techniques-for-diffuse-interstitial-pneumopathies
#10
B Duysinx, J Guiot, I Pellegrini, R Louis, J L Corhay, V Heinen
Interstitial lung diseases represent a very heterogeneous group of diseases mainly affecting connective lung tissue even if alveolar space may sometimes be involved. The identification of their etiology is the key stage in their management. It requires the integration of anamnestic, clinical, biological, radiological data and, sometimes relies on, cytology or histology. In this review, we assess the contribution and feasibility of the different invasive techniques used for interstitial lung disease diagnosis...
March 2018: Revue Médicale de Liège
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
#11
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
#12
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
#13
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/29420550/spectrum-of-interstitial-lung-diseases-at-a-tertiary-center-in-a-developing-country-a-study-of-803-subjects
#14
Sahajal Dhooria, Ritesh Agarwal, Inderpaul Singh Sehgal, Kuruswamy Thurai Prasad, Mandeep Garg, Amanjit Bal, Ashutosh Nath Aggarwal, Digambar Behera
BACKGROUND: The spectrum of interstitial lung diseases (ILDs) have mainly been reported from the developed countries; data from developing countries is sparse and conflicting. The aim of this study is to describe the distribution of various ILDs from a developing country. METHODS: This is an analysis of prospectively collected clinical, radiological and histological data of consecutive subjects (age >12 years) with ILDs from a single tertiary care medical center...
2018: PloS One
https://www.readbyqxmd.com/read/29325680/clinico-radio-pathological-characteristics-of-unclassifiable-idiopathic-interstitial-pneumonias
#15
Yasuhiko Nakamura, Keishi Sugino, Masashi Kitani, Akira Hebisawa, Naobumi Tochigi, Sakae Homma
BACKGROUND: The purpose of this study was to clarify the clinico-radio-pathological characteristics and prognostic factors of unclassifiable-idiopathic interstitial pneumonias (U-IIPs) diagnosed by surgical lung biopsy. METHODS: Among 86 patients with interstitial pneumonia who underwent surgical lung biopsy from January 2005 to September 2013, 33 (38.4%; 16 male patients; mean age, 64.4 ± 8.8 years) were diagnosed with U-IIPs. They were subsequently categorized into rapidly progressive (n = 7), slowly progressive (n = 7), and stable (n = 19) groups based on the decrease of the percent predicted forced vital capacity or percent predicted diffusing capacity of the lung carbon monoxide and the occurrence of acute exacerbation...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29320807/overlap-of-interstitial-pneumonia-with-autoimmune-features-with-undifferentiated-connective-tissue-disease-and-contribution-of-uip-to-mortality
#16
Bryan T Kelly, Teng Moua
BACKGROUND AND OBJECTIVE: Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome...
January 10, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29297205/cyclophosphamide-for-connective-tissue-disease-associated-interstitial-lung-disease
#17
REVIEW
Hayley Barnes, Anne E Holland, Glen P Westall, Nicole Sl Goh, Ian N Glaspole
BACKGROUND: Approximately one-third of individuals with interstitial lung disease (ILD) have associated connective tissue disease (CTD). The connective tissue disorders most commonly associated with ILD include scleroderma/systemic sclerosis (SSc), rheumatoid arthritis, polymyositis/dermatomyositis, and Sjögren's syndrome. Although many people with CTD-ILD do not develop progressive lung disease, a significant proportion do progress, leading to reduced physical function, decreased quality of life, and death...
January 3, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29285766/pulmonary-arterial-hypertension-associated-with-connective-tissue-diseases-a-review-focusing-on-distinctive-clinical-aspects
#18
REVIEW
Masaru Kato, Tatsuya Atsumi
Recent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH) has some distinctive clinical aspects from other PAH, such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre-PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD-PAH and garner attention to areas where further evidence is desired...
February 2018: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29282676/surveillance-on-the-safety-and-efficacy-of-ambrisentan-volibris-tablet-2-5-mg-in-patients-with-pulmonary-arterial-hypertension-in-real-clinical-practice-post-marketing-surveillance-interim-analysis-report
#19
Tomohiko Takahashi, Satoru Hayata, Akihiro Kobayashi, Yuna Onaka, Takeshi Ebihara, Terufumi Hara
BACKGROUND AND OBJECTIVE: Pulmonary arterial hypertension (PAH) is an intractable and rare disease and the accumulation of clinical evidence under real-world setting is needed. A post-marketing surveillance for the endothelin receptor antagonist ambrisentan (Volibris tablet) has been conducted by all-case investigation since September 2010. This paper is an interim report on the safety and efficacy of ambrisentan in 702 patients with PAH. METHODS: PAH patients aged 15 years or older were subjected to the analysis...
March 2018: Clinical Drug Investigation
https://www.readbyqxmd.com/read/29275603/-efficacy-and-safety-of-transbronchial-cryobiopsy-in-the-etiologic-diagnosis-of-diffuse-lung-disease
#20
Y S Li, S L Guo, X H Yi, M L Xiao, X X Jin, Y Xiao, X Y Zhu, X Li, L W Dai, Z Ao, X Z Liu, M Ding
Objective: To assess the efficacy and safety of transbronchial cryobiopsy (TBCB) for the etiologic evaluation of diffuse lung disease (DLD). Methods: Between December 2015 to April 2017, a total of 38 patients with DLD met the inclusion criteria for TBCB in the First Affiliated Hospital of Chongqing Medical University, and 35 of them consented to undergo the procedure under rigid or flexible bronchoscopy. On the tissues obtained from the 35 patients, histopathologic and microbiological evaluations were performed, and together with clinical and radiological manifestations, diagnoses were made and the efficacy of TBCB in the diagnosis of DLD was confirmed, and then therapies were planned accordingly...
December 12, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
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