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https://www.readbyqxmd.com/read/29445936/pulmonary-arterial-hemodynamic-assessment-by-a-novel-index-in-systemic-sclerosis-patients-pulmonary-pulse-transit-time
#1
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
https://www.readbyqxmd.com/read/29359173/immune-myopathies-with-perimysial-pathology-clinical-and-laboratory-features
#2
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
https://www.readbyqxmd.com/read/29353024/outcomes-and-mortality-prediction-model-of-critically-ill-adults-with-acute-respiratory-failure-and-interstitial-lung-disease
#3
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
https://www.readbyqxmd.com/read/29316328/anti-fibrotic-effects-of-pirfenidone-by-interference-with-the-hedgehog-signalling-pathway-in-patients-with-systemic-sclerosis-associated-interstitial-lung-disease
#4
Hua Xiao, Guang-Feng Zhang, Xiang-Ping Liao, Xiao-Jie Li, Jian Zhang, Haobo Lin, Zhe Chen, Xiao Zhang
AIM: To determine whether pirfenidone attenuates lung fibrosis by interfering with the hedgehog (Hh) signalling pathway in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Twenty-five SSc-ILD patients (20 first visit, five who underwent pirfenidone treatment for 6 months) and 10 healthy controls were recruited. Lung tissues were obtained by open-chest surgery, and primary lung fibroblasts were isolated, cultured and stimulated with pirfenidone...
January 7, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29297205/cyclophosphamide-for-connective-tissue-disease-associated-interstitial-lung-disease
#5
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
#6
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...
December 15, 2017: 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
#7
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...
December 27, 2017: Clinical Drug Investigation
https://www.readbyqxmd.com/read/29229096/clinical-characteristics-of-patients-with-anti-aminoacyl-trna-synthetase-antibody-positive-idiopathic-interstitial-pneumonia
#8
Hirokazu Yura, Noriho Sakamoto, Minoru Satoh, Hiroshi Ishimoto, Tetsuya Hanaka, Chiyo Ito, Tomoko Hasegawa, Shin Tanaka, Takuto Miyamura, Shota Nakashima, Atsuko Hara, Tomoyuki Kakugawa, Keishi Oda, Takashi Kido, Yasushi Obase, Yuji Ishimatsu, Kazuhiro Yatera, Atsushi Kawakami, Hiroshi Mukae
BACKGROUND: Anti-aminoacyl-tRNA synthetase (ARS) antibodies have been detected in patients with polymyositis/dermatomyositis (PM/DM) and are especially correlated with interstitial lung disease (ILD). The aim of this study was to clarify the clinical features of patients with anti-ARS antibody positive idiopathic interstitial pneumonias (IIPs). METHODS: Patients were classified into three groups: 1) IIP with anti-ARS antibodies (ARS(+)IIP), 2) IIP without anti-ARS antibodies (ARS(-)IIP), and 3) PM/DM-associated ILD with anti-ARS antibodies (ARS(+)PM/DM-ILD)...
November 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29222007/current-concepts-in-pathogenesis-diagnosis-and-management-of-smoking-related-interstitial-lung-diseases
#9
REVIEW
Anupam Kumar, Sujith V Cherian, Robert Vassallo, Eunhee S Yi, Jay H Ryu
Tobacco exposure results in various changes to the airways and lung parenchyma. While emphysema represents the more common injury pattern, in some individuals, cigarette smoke injures alveolar epithelial and other lung cells resulting in diffuse infiltrates and parenchymal fibrosis. Smoking can trigger interstitial injury patterns mediated via recruitment and inappropriate persistence of myeloid and other immune cells including eosinophils. As our understanding of the role of cigarette smoke constituents in triggering lung injury continues to evolve, so does our recognition of the spectrum of smoking-related interstitial lung changes...
December 5, 2017: Chest
https://www.readbyqxmd.com/read/29202589/interstitial-lung-disease-in-systemic-autoimmune-rheumatic-diseases-a-comprehensive-review
#10
Fabiola Atzeni, Maria Chiara Gerardi, Giuseppe Barilaro, Ignazio Francesco Masala, Maurizio Benucci, Piercarlo Sarzi-Puttini
Interstitial lung diseases (ILDs) are among the most serious complications associated with systemic rheumatic diseases, and lead to significant morbidity and mortality; they may also be the first manifestation of connective tissue diseases (CTDs). The aim of this narrative review is to summarise the data concerning the pathogenesis of CTD/ILD and its distinguishing features in different rheumatic diseseas. Areas covered: The pathogenesis, clinical aspects and treatment of ILD associated with rheumatic systemic diseases and CTDs were reviewed by searching the PubMed, Medline, and Cochrane Library databases for papers published between 1995 and February 2017 using combinations of words or terms...
January 2018: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29159696/reflex-testing-of-speckled-cytoplasmic-patterns-observed-in-routine-ana-hep-2-indirect-immunofluorescence-with-a-multiplex-anti-synthetase-dot-blot-assay-a-multicentric-pilot-study
#11
Maria Infantino, Boaz Palterer, Roberta Biagiotti, Fabio Almerigogna, Maurizio Benucci, Arianna Damiani, Valentina Grossi, Annalisa Azzurri, Patrizia Casprini, Giovanni Bacci, Maria Grazia Giudizi, Mariangela Manfredi
Immunofluorescence on HEp2-cells is the standard diagnostic assay for the detection of anti-nuclear antibodies (ANA). Cytoplasmic speckled patterns are a common finding, and are associated with various antibodies, including anti-synthetase antibodies. However, classic ENA testing generally identifies only anti-Jo-1. Moreover, anti-synthetase syndrome is increasingly recognized as a pleomorphic entity, possibly presenting as isolated arthritis or interstitial lung disease. Sera referred for routine ANA testing were selected on the basis of the presence of a fine dense speckled cytoplasmic pattern (254 samples) and compared to control sera with negative cytoplasm (239 samples)...
November 21, 2017: Immunologic Research
https://www.readbyqxmd.com/read/29140119/ct-features-of-the-usual-interstitial-pneumonia-pattern-differentiating-connective-tissue-disease-associated-interstitial-lung-disease-from-idiopathic-pulmonary-fibrosis
#12
Jonathan H Chung, Christian W Cox, Steven M Montner, Ayodeji Adegunsoye, Justin M Oldham, Aliya N Husain, Rekha Vij, Imre Noth, David A Lynch, Mary E Strek
OBJECTIVE: A substantial proportion of cases of usual interstitial pneumonia (UIP) are due to connective tissue disease (CTD)-associated interstitial lung disease (ILD). The purpose of this study was to determine whether specific CT findings can help differentiate a UIP pattern of CTD-ILD from a UIP pattern of idiopathic pulmonary fibrosis (IPF) and whether these signs are associated with survival. MATERIALS AND METHODS: Adults visiting an ILD clinic from 2006 to 2015 enrolled in a research registry with a multidisciplinary diagnosis of CTD-ILD or IPF and a UIP pattern at high-resolution CT were included in the study...
November 15, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/29129886/-management-of-connective-tissue-disease-associated-interstitial-lung-disease
#13
Jun Kishi
No abstract text is available yet for this article.
2017: Arerugī, [Allergy]
https://www.readbyqxmd.com/read/29054712/-spontaneous-pneumomediastinum-a-rare-complication-of-dermatomyositis
#14
A Allaoui, F Aboudib, W Bouissar, K Echchilali, M Moudatir, F Z Alaoui, H Elkabli
Dermatomyositis is a rare connective tissue disease of unknown origin, including inflammatory myopathy and cutaneous manifestations. Several pulmonary complications associated to dermatomyositis were described; especially interstitial lung disease. Some rare and particular pulmonary complications were reported in the literature such as pneumodiastinum and pneumothorax. We are describing here, a case report about a female patient, who presented with dermatomyositis associated to pneumomediastinum as a severe and lethal complication without pneumothorax...
October 2017: Revue de Pneumologie Clinique
https://www.readbyqxmd.com/read/29050128/-lung-transplantation-for-connective-tissue-disease-associated-interstitial-lung-disease-recipient
#15
J Zhang, B Wu, M Zhou, D Liu, L Fan, D Wei, H Yang, J Y Chen
Objective: To investigate the timing and complications of lung transplantation for the treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD). Method: The clinical data of connective tissue-associated disease lung transplantation recipients from September, 2015 to February, 2017 were collected. Results: 11 patients with CTD-ILD were evaluated by lung transplantation and were treated with lung transplantation. Including 2 cases dermatomyositis/polymyositis and interstitial lung disease (PM/DM-ILD), 4 cases rheumatoid arthritis and interstitial lung disease (RA-ILD), 4 cases of primary Sjgren's syndrome and interstitial lung disease (pSS-ILD), 1 case of systemic sclerosis and interstitial lung disease (SSc-ILD)...
October 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29045979/-changes-of-serum-krebs-von-den-lungen-6-levels-in-interstitial-lung-disease-associated-with-dermatomyositis-and-secondary-sj%C3%A3-gren-s-syndrome-a-case-report
#16
J F Yu, Y B Jin, J He, Y An, Z G Li
Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. The underlying pathogenesis of ILDs is complex and associated with multiple rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, pollymyositis and dermatomyositis, Sjögren's syndrome, and systemic lupus erythematosus. As the disease progresses, excessive pulmonary fibrosis impairs alveolar gas exchange and damages pulmonary function...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29038968/anti-fibrotic-nintedanib-a-new-opportunity-for-systemic-sclerosis-patients
#17
REVIEW
Ana Catarina Duarte, Maria José Santos, Ana Cordeiro
Systemic sclerosis is a connective tissue disease characterized by progressive skin thickening and a wide spectrum of internal organ involvement. Pathogenesis includes vasculopathy, inflammation, and fibrosis. Although immunosuppressants such as cyclophosphamide and mycophenolate mofetil have shown some benefit in interstitial lung disease management, it is still a major cause of morbi-mortality in these patients. Therefore, there is a current need for new therapies. Here, we report a 65-year-old female patient with limited cutaneous systemic sclerosis, anti-topoisomerase-positive and extensive lung disease...
October 16, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29035287/cardio-pulmonary-involvement-in-systemic-sclerosis-a-study-at-a-tertiary-care-center
#18
Geetakiran Arakkal, Sudha Rani Chintagunta, Vasavilatha Chandika, Sudha Vani Damarla, Srinivas Manchala, B Udaya Kumar
BACKGROUND: Systemic sclerosis is a multisystem disorder characterized by microangiopathy, dysregulation of the immune system and massive deposition of collagen in the connective tissue of the skin, blood vessels and various internal organs. Although the mortality from renal crises has dropped significantly due to the use of angiotensin-converting enzyme inhibitors, cardiac and pulmonary involvement accounts for significant morbidity and mortality. We studied 28 patients with systemic sclerosis at Gandhi Medical College and Hospital, Hyderabad, over a period of two years for cardiopulmonary involvement...
November 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/28976395/-pulmonary-hypertension-in-the-elderly
#19
Serdar Küçükoğlu, Ümit Yaşar Sinan
Pulmonary hypertension is defined as 25mmHg or higher mean pulmonary arterial pressure measured by right heart catheterization. Miscellaneous diseases may cause PH. Among these are pulmonary arterial hypertension (PAH), left heart disease, pulmonary disorders and hypoemia, and chronic thromboembolic disease. Pulmonary hypertension (PH) is seen frequently in the elderly. Although diseases resulting in systolic and diastolic dysfunction with accompanying pulmonary venous hypertension (PVH) account for a large percentage of PH in this population, PH associated with other respiratory diseases, such as chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and sleep apnea can also be seen...
September 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28945277/malt-lymphoma-arising-on-a-background-of-reactive-pulmonary-lymphoid-hyperplasia-in-a-patient-with-systemic-lupus-erythematosus
#20
Ute Laggner, Reena Khiroya, Andrew C Wotherspoon, Sujal R Desai, Andrew G Nicholson
No abstract text is available yet for this article.
September 25, 2017: Histopathology
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