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Connective tissue disease and the lung 2014

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13 papers 500 to 1000 followers
By Jason Mann No BS pulmonary critical care fellow
Julia Capobianco, Alexandre Grimberg, Bruna M Thompson, Viviane B Antunes, Dany Jasinowodolinski, Gustavo S P Meirelles
Collagen vascular diseases are a diverse group of immunologically mediated systemic disorders that often lead to thoracic changes. The collagen vascular diseases that most commonly involve the lung are rheumatoid arthritis, progressive systemic sclerosis, systemic lupus erythematosus, polymyositis and dermatomyositis, mixed connective tissue disease, and Sjögren syndrome. Interstitial lung disease and pulmonary arterial hypertension are the main causes of mortality and morbidity among patients with collagen vascular diseases...
January 2012: Radiographics: a Review Publication of the Radiological Society of North America, Inc
David A Lynch
Collagen vascular disease is one of the most common causes of chronic infiltrative lung disease. Patterns of lung injury from collagen vascular disease include nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia, organizing pneumonia, bronchiectasis, obliterative bronchiolitis, and pulmonary arterial hypertension. The prevalence of each entity varies according to the specific disease entity. NSIP and pulmonary hypertension are common in scleroderma, whereas usual interstitial pneumonia, bronchiectasis, and obliterative bronchiolitis are commonly found in rheumatoid arthritis...
November 2009: Journal of Thoracic Imaging
Joshua J Solomon, Aryeh Fischer
The connective tissue diseases (CTDs) are a group of systemic disorders characterized by autoimmunity and autoimmune-mediated organ damage. The lung is a frequent target and all components of the respiratory system are at risk. Interstitial lung disease (ILD) represents a broad group of diffuse parenchymal lung injury patterns characterized by varying degrees of inflammation and fibrosis, is a common manifestation of CTD particularly common in systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis, and is a leading cause of significant morbidity and mortality...
October 2015: Journal of Intensive Care Medicine
Aryeh Fischer, Luca Richeldi
Lung disease is a common manifestation of connective tissue disease (CTD) and is associated with significant morbidity and mortality. The evaluation of lung disease, and interstitial lung disease (ILD) in particular, in patients with CTD is complex because of the heterogeneity of the CTDs, the varied types and degrees of severity of ILD encountered, and because ILD can be identified at any point in time in these patients. Cross-disciplinary, thorough evaluations are needed when CTD patients develop ILD or when evaluating ILD patients for the presence of occult CTD...
April 2014: Seminars in Respiratory and Critical Care Medicine
J Jacob, A Nair, David M Hansell
Pulmonary disease is common in patients with connective tissue disease (CTD) and confers significant morbidity and mortality. High-resolution computed tomography (HRCT) provides considerable information regarding the distribution and severity of thoracic disease in CTD. Different anatomical compartments of the lung can be simultaneously affected in a given individual. Furthermore, lung injury may not be solely related to the underlying CTD, but may be caused by treatment or infections. Finally, the risk of developing cancer is increased in patients with certain CTDs...
April 2014: Seminars in Respiratory and Critical Care Medicine
Francesco Bonella, Ulrich Costabel
This article reviews major biomarkers in serum and bronchoalveolar lavage fluid (BALF) with respect to their diagnostic and prognostic value in connective tissue disease-associated interstitial lung disease (CTD-ILD). In some CTD such as systemic sclerosis (SSc), the incidence of ILD is up to two-third of patients, and currently ILD represents the leading cause of death in SSc. Because of the extremely variable incidence and outcome of ILD in CTD, progress in the discovery and validation of biomarkers for diagnosis, prognosis, patients' subtyping, response to treatment, or as surrogate endpoints in clinical trials is extremely important...
April 2014: Seminars in Respiratory and Critical Care Medicine
Athol U Wells, George A Margaritopoulos, Katerina M Antoniou, Chris Denton
Despite many unanswered questions regarding the pathogenesis of interstitial lung disease in systemic sclerosis (SSc-ILD) and the lack of accurate epidemiological risk factors, there have been major advances in the identification and prognostic evaluation of SSc-ILD. The evaluation of disease severity is a multidisciplinary exercise, requiring the integration of pulmonary function tests, high-resolution computed tomography data, and symptomatic severity and these factors all need to be considered in the detection of disease progression...
April 2014: Seminars in Respiratory and Critical Care Medicine
Mridu Gulati, Danielle Antin-Ozerkis
Patients with connective tissue disease often suffer from pulmonary complications, including interstitial lung disease and pulmonary hypertension. Supportive care for these patients aims to relieve symptoms and improve activity level and quality of life. A holistic approach to the management of patients with advanced connective tissue disease-associated pulmonary disorders includes a full assessment of patient symptoms as well as a careful search for side effects of treatment and treatable comorbidities. This article addresses supportive measures such as supplemental oxygen and pulmonary rehabilitation...
April 2014: Seminars in Respiratory and Critical Care Medicine
Toby M Maher
With improvements in the systemic treatment of the various connective tissue diseases (CTDs) the pulmonary complications of these conditions are now, for many patients, the major cause of morbidity and impaired quality of life. Furthermore, at least in scleroderma, pulmonary disease has become the leading cause of death for this patient group. Although, the pathogenesis of CTD-related pulmonary disease is poorly understood there is an assumption that it arises as a sequelae of immune-mediated injury to the lung...
April 2014: Seminars in Respiratory and Critical Care Medicine
Maryl Kreider, Kristin Highland
Sjögren syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic inflammation of exocrine glands and a variety of extraglandular sites. Lung involvement as defined by symptoms and either pulmonary function testing or radiographic abnormalities occurs in approximately 10 to 20% of patients. Subclinical lung disease is even more frequent and often includes evidence of small airways disease and airway inflammation. In general, patients will have evidence of both airway and interstitial lung disease by radiographs and pathology...
April 2014: Seminars in Respiratory and Critical Care Medicine
Fiona Lake, Susanna Proudman
Rheumatoid arthritis (RA) is a common chronic systemic autoimmune disease characterized by joint inflammation and, in a proportion of patients, extra-articular manifestations (EAM). Lung disease, either as an EAM of the disease, related to the drug therapy for RA, or related to comorbid conditions, is the second commonest cause of mortality. All areas of the lung including the pleura, airways, parenchyma, and vasculature may be involved, with interstitial and pleural disease and infection being the most common problems...
April 2014: Seminars in Respiratory and Critical Care Medicine
Robert W Hallowell, Dana P Ascherman, Sonye K Danoff
The idiopathic inflammatory myopathies are a group of connective tissue diseases marked by varying degrees of muscle inflammation and clinical involvement of multiple organs, most notably, the lung. Pulmonary manifestations consist primarily of interstitial lung disease (ILD), which is associated with significant morbidity and mortality in myositis patients. Several myositis-specific antibodies have been discovered, as well as antibodies targeting various aminoacyl-tRNA synthetase enzymes. These antibodies are associated with various clinical features and a risk for developing ILD, and their presence carries a prognostic value in myositis patients...
April 2014: Seminars in Respiratory and Critical Care Medicine
Shikha Mittoo, Charlene D Fell
Systemic lupus erythematosus (SLE) is a systemic inflammatory disease, characterized serologically by an autoantibody response to nucleic antigens, and clinically by injury and/or malfunction in any organ system. During their disease course, up to 50% of SLE patients will develop lung disease. Pulmonary manifestations of SLE include pleuritis (with or without effusion), inflammatory and fibrotic forms of interstitial lung disease, alveolar hemorrhage, shrinking lung syndrome, pulmonary hypertension, airways disease, and thromboembolic disease...
April 2014: Seminars in Respiratory and Critical Care Medicine
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