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connective tissue disease and lung

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)
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
Junfei Wang, Alen Faiz, Qi Ge, Cornelis J Vermeulen, Joanne Van der Velden, Kenneth J Snibson, Rob van de Velde, Sonia Sawant, Dikaia Xenaki, Brian Oliver, Wim Timens, Nick Ten Hacken, Maarten van den Berge, Alan James, John G Elliot, Liang Dong, Janette K Burgess, Anthony W Ashton
Neovascularization, increased basal membrane thickness and increased airway smooth muscle (ASM) bulk are hallmarks of airway remodelling in asthma. In this study, we examined connective tissue growth factor (CTGF) dysregulation in human lung tissue and animal models of allergic airway disease. Immunohistochemistry revealed that ASM cells from patients with severe asthma (A) exhibited high expression of CTGF, compared to mild and non-asthmatic (NA) tissues. This finding was replicated in a sheep model of allergic airways disease...
March 7, 2018: Journal of Cellular and Molecular Medicine
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
David Montani, Julien Henry, Caroline O'Connell, Xavier Jaïs, Vincent Cottin, David Launay, Gilbert Habib, Arnaud Bourdin, Mitja Jevnikar, Laurent Savale, Laurence Rottat, Gérald Simonneau, Olivier Sitbon, Marc Humbert, Yannick Allanore
BACKGROUND: Precapillary pulmonary hypertension (PH), and particularly pulmonary arterial hypertension (PAH), is a life-threatening complication of connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease). The relationship between PH and rheumatoid arthritis (RA) has not been clearly established. OBJECTIVES: The aim of the study was to evaluate the relationship between precapillary PH and RA. METHODS: We identified patients with PH and suspected RA included in the French PH Registry between 1 May 2004 and 31 December 2012 and evaluated the prevalence of confirmed RA in this population...
February 28, 2018: Respiration; International Review of Thoracic Diseases
Hideaki Yamakawa, Tsuneyuki Oda, Tomohisa Baba, Takashi Ogura
A 67-year-old woman was referred to our hospital because of gradually increasing dyspnoea on exertion for 6 months. Chest CT scan showed subpleural parenchymal fibrotic opacities with traction bronchiectasis in the bilateral upper lung fields. Serum rheumatoid factor and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) were positive. There was no evident reason to suspect connective tissue disease such as ANCA-associated vasculitis or rheumatoid arthritis. We performed a CT-guided percutaneous needle biopsy of the subpleural lesion that showed slight uptake on the fluorodeoxyglucose-positron emission tomography (FDG-PET) CT scan...
February 24, 2018: BMJ Case Reports
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...
February 21, 2018: Current Opinion in Pulmonary Medicine
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...
February 14, 2018: Lung
T Ben Salem, M Tougorti, S Bziouech, M Lamloum, M Khanfir, I Ben Ghorbel, M H Houman
INTRODUCTION: Raynaud's phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud's phenomenon were distinguished: Raynaud's disease and secondary Raynaud's phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud's phenomenon in an internal medicine department. METHODS: A descriptive retrospective study including patients with secondary Raynaud's phenomenon followed in a tertiary internal medicine department between 2000 and 2013...
February 2018: Journal de Médecine Vasculaire
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
Chia-Huei Peng, Chyong-Hsin Hsu, Nien-Lu Wang, Hung-Chang Lee, Shuan-Pei Lin, Wai-Tao Chan, Chun-Yan Yeung, Chuen-Bin Jiang
RATIONALE: Menkes disease (MD), also known as Menkes kinky hair disease, is a fatal neurodegenerative disease caused by a defect in copper metabolism. The symptoms involve multiple organ systems, such as the brain, lung, gastrointestinal tract, urinary tract, connective tissue, and skin. There is currently no cure for this disease entity, and patients with the classic form of MD usually die from complications between 6 months and 3 years of age. Intracranial hemorrhage secondary to tortuous intracranial arteries is a well-known complication of MD, but spontaneous retroperitoneal hemorrhage, to the best of our knowledge, has never been reported in a patient with MD...
February 2018: Medicine (Baltimore)
Meltem Ağca, Fatma Tokgöz Akyıl, Merve Hörmet, Oğuzhan Akman, Canan Akman, Ayçim Şen, Tülin Sevim
Pulmonary lymphangitic carcinomatosis (PLC) is defined as infiltration of the lymphatic vessels and perilymphatic connective tissue with tumor cells, which is secondary to malignancy. Therefore, it rarely appears as an initial finding preceding a diagnosis of malignancy. A 30-year-old male patient was hospitalized in our clinic with a pre-diagnosis of interstitial lung disease owing to the complaints of dry cough, progressive dyspnea, and acute respiratory insufficiency. He was diagnosed with signet ring cell carcinoma, which is a histologic subtype of adenocarcinoma, via gastroscopy, and lung involvement was consistent with PLC...
July 2017: Turkish Thoracic Journal
John E Pearl, Mrinal Das, Andrea M Cooper
Accurate prediction of which patient will progress from a sub-clinical Mycobacterium tuberculosis infection to active tuberculosis represents an elusive, yet critical, clinical research objective. From the individual perspective, progression can be considered to be the product of a series of unfortunate events or even a run of bad luck. Here, we identify the subtle physiological relationships that can influence the odds of progression to active TB and how this progression may reflect directed dysbiosis in a number of interrelated systems...
January 29, 2018: Cellular & Molecular Immunology
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
Whitney Gannon, David J Lederer, Mauer Biscotti, Azka Javaid, Nina Patel, Daniel Brodie, Matthew Bacchetta, Matthew Baldwin
BACKGROUND: We aimed to examine short- and long-term mortality in a mixed population of interstitial lung disease (ILD) patients with acute respiratory failure, and to identify those at lower versus higher risk of in-hospital death. METHODS: We conducted a single-center retrospective cohort study of 126 consecutive adults with ILD admitted to an intensive care unit (ICU) for respiratory failure at a tertiary care hospital between 2010 and 2014 who did not undergo lung transplantation during their hospitalization...
January 15, 2018: Chest
Najla Fiaturi, Joshua W Russo, Heber C Nielsen, John J Castellot
Lung immaturity is the major cause of morbidity and mortality in premature infants, especially those born <28 weeks of gestation. These infants are at high risk of developing respiratory distress syndrome (RDS), a lung disease caused by insufficient surfactant production and immaturity of saccular/alveolar type II epithelial cells in the lung. RDS treatment includes oxygen and respiratory support that improve survival but also increase the risk for bronchopulmonary dysplasia (BPD), a chronic lung disease characterized by arrested alveolarization, airway hyperreactivity, and pulmonary hypertension...
January 18, 2018: Journal of Cell Communication and Signaling
Maryam Moradi, Mohammad Ali Rezaee, Mehdi Mohammadi, Mohammad Jafar Rezaie, Ali Jalili, Mohammad Reza Rahmani
In recent studies, mesenchymal stromal cells (MSCs) have been increasingly employed to treat various diseases like pulmonary fibrosis (PF). There are very few MSCs in tissues so in order to obtain their sufficient numbers for therapeutic applications, their in vitro expansion is necessary. The aim of this study was to investigate the effects of long-term culture of the human umbilical cord vein MSCs (hUCV-MSCs) on pulmonary fibrosis in mice. MSCs were first isolated from human umbilical cord vein and cultured up to 18 passages...
December 2017: Iranian Journal of Allergy, Asthma, and Immunology
Shinichirou Ishimoto, Hiroyuki Sakurai, Ryouta Higure, Riken Kawachi, Mie Shimamura
Pulmonary artery aneurysms (PAA) and pseudoaneurysms (PAP) are caused by infections, vasculitis, trauma, pulmonary hypertension, congenital heart disease, and connective tissue disease. Most cases of such aneurysm occur in the trunk or major branches of the pulmonary artery, while the peripheral type is less common. The treatment modalities are medical therapy, surgery, and percutaneous catheter embolization. The mortality rate associated with rupture is approximately 50%. We encountered a case of a 53-year-old man with a pulmonary artery pseudoaneurysm secondary to pneumonia and cavity formation during chemotherapy for acute myeloid leukemia (AML)...
January 15, 2018: Annals of Thoracic and Cardiovascular Surgery
Valentin Coirier, Alain Lescoat, Céline Chabanne, Maxime Fournet, Guillaume Coiffier, Stéphane Jouneau, Elisabeth Polard, Patrick Jégo
Pulmonary arterial hypertension (PAH) is a rare disorder that can be drug-induced, mostly following treatment by appetite-suppressant drugs. We report four cases of patients who developed PAH following a treatment by leflunomide for rheumatoid arthritis, psoriatic arthritis or undetermined connective tissue disease. All patients described a progressive dyspnea from grade II to IV of NYHA classification; clinical examination found signs of heart failure. PAH was finally diagnosed and confirmed by right heart catheterisation...
January 9, 2018: Joint, Bone, Spine: Revue du Rhumatisme
Francesco Varone, Giacomo Sgalla, Bruno Iovene, Teresa Bruni, Luca Richeldi
Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of pulmonary function, ultimately leading to respiratory failure and death. Two novel compounds, nintedanib and pirfenidone, have shown efficacy in reducing the rate of decline of lung function in IPF patients. The multiple tyrosine kinase inhibitor nintedanib has extensively being studied as a potential angiogenesis inhibitor in clinical against various neoplastic disorders. Afterwards, this compound was successfully tested in IPF...
February 2018: Expert Opinion on Pharmacotherapy
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