Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0729233 (Thoracic)
 6,478 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thoracic radiotherapy may produce the morbidity-associated lung responses of alveolitis or fibrosing alveolitis in treated cancer patients. The genetic factors that influence a patient's likelihood of developing alveolitis and the relationship of this inflammatory response to the development of fibrosis are largely unknown. Herein we use genetic mapping to identify radiation-induced lung response susceptibility loci in reciprocal backcross mice bred from C3H/HeJ (alveolitis response) and C57BL/6J (fibrosing alveolitis/fibrosis response) strains. Mice were treated with 18-Gy whole thorax irradiation and their survival, lung histopathology, and bronchoalveolar lavage cell types were recorded. A genome-wide scan was completed using 139 markers. The C3H/HeJ alveolitis response included mast cell infiltration and increased neutrophil numbers in the lavage compared with the level in the C57BL/6J strain, which developed fibrosis. In backcross mice, posttreatment survival was dictated by the development of an alveolitis response with increased mast cell, bronchoalveolar lavage total cell, and neutrophil numbers. Fibrosis was measured only in a subset of mice developing alveolitis and, in these mice, was associated with neutrophil count. Genotyping revealed coinheritance of C3H alleles (chromosomes 2, 4, 19, and X) and C57BL/6J alleles (chromosomes 1, 7, 9, and 17) to result in higher fibrosis scores in backcross mice. Mice that inherited C57BL/6J alleles at the putative alveolitis susceptibility loci were spared this response and lived to the end of the experiment. In this animal model, independent loci control the development of alveolitis from fibrosis, whereas fibrosing alveolitis occurs with the coinheritance of these factors.
 ...
PMID:Distinct loci influence radiation-induced alveolitis from fibrosing alveolitis in the mouse. 1800 24

The purpose of the present study was to evaluate the accuracy of the diagnosis of idiopathic pulmonary fibrosis (IPF) by respiratory physicians in six European countries, and to calculate the interobserver agreement between high-resolution computed tomography reviewers and histology reviewers in IPF diagnosis. The diagnosis of usual interstitial pneumonia (UIP) was assessed by a local investigator, following the American Thoracic Society/European Respiratory Society consensus statement, and confirmed when a minimum of two out of three expert reviewers from each expert panel agreed with the diagnosis. The level of agreement between readers within each expert panel was calculated by weighted kappa. The diagnosis of UIP was confirmed by the expert panels in 87.2% of cases. A total of 179 thoracic high-resolution computed tomography scans were independently reviewed, and an interobserver agreement of 0.40 was found. Open or thoracoscopic lung biopsy was performed in 97 patients, 82 of whom could be reviewed by the expert committee. The weighted kappa between histology readers was 0.30. It is concluded that, although the level of agreement between the readers within each panel was only fair to moderate, the overall accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis in expert centres is good (87.2%).
 ...
PMID:Multidisciplinary interobserver agreement in the diagnosis of idiopathic pulmonary fibrosis. 1805 59

Idiopathic pulmonary fibrosis (IPF) is a devastating disease that afflicts patients with relentlessly progressive shortness of breath [Joint Statement of the American Thoracic Society and the European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. Am J Respir Crit Care Med 2000;161:646-64(1)]. Despite nearly 30 years of intense investigation, effective therapy for IPF remains elusive; median survival rates have stubbornly remained less than five years from the time of diagnosis [Bjoraker JA, Ryu JH, Edwin MK, Meyers J, Tazelaar H, Schroeder D, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;157:199-203(2), Flaherty KR, Thwaite E, Kazerooni EA, Gross B, Toews GB, Colby TV, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 2003;58:143-48(3)], and no medical therapy has been proved to be in any way effective for the treatment of this disease. Without medications that help IPF patients live longer, an important question to ask is whether there are interventions that might allow these people to live better-to be more active; to experience less dyspnea, less depression, less anxiety; to possess a greater sense of control over their disease; and to have better quality of life. Pulmonary rehabilitation helps to accomplish many of these goals in patients with chronic obstructive pulmonary disease, and emerging data suggest that it may do the same for patients with IPF.
 ...
PMID:Pulmonary rehabilitation in idiopathic pulmonary fibrosis: a call for continued investigation. 1884 71

Diffuse lung diseases (DLD), known as interstitial lung diseases or diffuse parenchymal lung diseases, are a large group of disorders of diverse etiology and causes, however, sharing similar clinical, radiological and pathophysiological characteristics. In the last fifteen years, DLD have attracted considerable interest of medical society. During that period, a consensus of the British Thoracic Society on the Diffuse Parenchymal Lung Disease and Statement of the American Thoracic Society (ATS) and European Respiratory Society (ERS) on idiopathic pulmonary fibrosis, idiopathic interstitial pneumonias and sarcoidosis have helped precisely define certain phenotypes. The newly developed technique of high resolution computed tomography, which can show finest details of lung parenchyma, has also helped precisely define diverse entities, which have aroused interest among molecular biologists and genetic researchers with a goal to define the etiology, pathogenesis and progression of these diseases. The renaissance of interest in this scientific field has also stimulated pharmaceutical industry to enhance its activity, which has resulted in intensified research of potentially new drugs, especially for fibrotic lung processes such as idiopathic pulmonary fibrosis. The disease outcome is very difficult to estimate in these, most often chronic diseases; however, it is very important to achieve the best possible if not the same quality of life as before the disease onset. Different questionnaires have been used and the results were not always as expected; for instance, worsened lung function tests were not most important in defining the quality of life. These vivacious activities have stimulated us to present to the patient and diligent reader recent advances in the management of DLD patient; the article is mostly dedicated to recent advances made in diagnostic procedures, hoping for a comparable success to be achieved in therapeutic approach.
 ...
PMID:[Diffuse lung disease--advances in patient management]. 1920 19

Idiopathic pulmonary fibrosis (IPF) is a chronic condition of unknown etiology with an life-limiting outcome. An excess of profibrotic and proinflammatory mediators as well as reactive oxygen species, resulting in progressive fixed tissue fibrosis, architectural distortion and loss of lung function making it plausible to inhibit these processes therapeutically. In this review new treatment options are discussed including substances with antiinflammatory properties which inhibit cytokines, eicosanoids or oxidants, drugs with antifibrotic efficacy as well as anticoagulative compounds. In the last 5 years treatment trials include only IPF patients characterized by the criteria of the American Thoracic Society and the European Respiratory Society. However, lack of validated outcome measures in most trials representing either disease improvement or progression, and/or sufficient large patient number are still hindering this kind of studies. Unfortunately most studies still failed to meet their primary end-points. Marginal trends or statistically significant differences between treatment groups were only apparent in subgroups or exploratory end-points of post-hoc analysis. Regardless of numerous trails published in recent years and per se promising new drugs, a change in current recommendations in the management of IPF is not warranted at present.
 ...
PMID:[New treatment options for idiopathic pulmonary fibrosis?]. 1970 7

The treatment of pulmonary fibrosis continues to pose major difficulties. Idiopathic pulmonary fibrosis (IPF), the most prevalent chronic fibrosing lung disease, is a devastating condition that carries a prognosis worse than that of many cancers. Abnormalities in multiple pathways involved in wound healing and inflammation lead to the development of this condition. High doses of corticosteroids are now contraindicated in IPF, although they have a role in other fibrosing lung diseases. More effective treatments are urgently required and a number of novel candidate therapies have been put forward, based on animal and in vitro work. As in other complex disorders, it is likely that combinations of agents, rather than single treatments, will be needed. The principle of combination therapy was recently endorsed by the guidelines of the British Thoracic Society, which make a weak recommendation for a combination of prednisolone, azathioprine and N-acetylcysteine. However, enrolment of patients into trials of new therapies is considered to be 'best current practice' as this offers sufferers with IPF the chance to receive new agents that may be more effective than current treatments. In pulmonary fibrotic disorders other than IPF, anti-inflammatory therapy is broadly appropriate and benefits most patients, but a clear treatment strategy is essential. The art of management is to distinguish accurately between inherently stable fibrotic disease (with treatment not required), progressive predominantly fibrotic disease (with low-dose long-term treatment warranted to retard progression) and the presence of major associated inflammation (justifying initial high-dose treatment).
 ...
PMID:New pharmacological strategies for the treatment of pulmonary fibrosis. 2086 57

This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format. For each diagnosis and treatment question, the committee graded the quality of the evidence available (high, moderate, low, or very low), and made a recommendation (yes or no, strong or weak). Recommendations were based on majority vote. It is emphasized that clinicians must spend adequate time with patients to discuss patients' values and preferences and decide on the appropriate course of action.
 ...
PMID:An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. 2238 72

The American Thoracic Society and European Respiratory Society guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) have been published recently. However, the influence, practical application, and utility of the prior consensus statement for IPF have never been evaluated. Demographics, diagnostic criteria, pulmonary function data, and disposition of patients with IPF evaluated at an interstitial lung disease center between 2000 and 2009 were analyzed. Enrollment in clinical drug trials, lung transplantation, and mortality also were assessed. A total of 521 patients with IPF were evaluated, with pulmonary function testing available in 446. In the 64% of patients without surgical lung biopsy, the most common major criterion not fulfilled was bronchoscopy. Lung transplantation was performed in 16.1% of patients, whereas 27.4% of prescreened patients were enrolled in a prospective drug study. Patients with mild, moderate, and severe disease categorized by FVC % predicted had median survivals of 55.6, 38.7, and 27.4 months, respectively. The attrition rate of patients who survived beyond 5 years was attenuated in subsequent years. IPF remains a deadly disease with a poor prognosis. Bronchoscopy does not appear to be required for an accurate diagnosis. A minority of patients were accommodated within a clinical trial or with transplantation. Categorization by baseline FVC % predicted effectively discriminates groups with different long-term outcomes. Our analysis supports the view that the value of statements also can be realized in the subsequent demonstration of their impact on patient management, which might enable further refinements in a continuous, iterative rediscovery process.
 ...
PMID:Long-term course and prognosis of idiopathic pulmonary fibrosis in the new millennium. 2172 85

Advancing age is associated with increased risk for some forms of interstitial lung disease (ILD), and this risk is especially reflected by the considerably increased incidence of idiopathic pulmonary fibrosis (IPF) in the elderly. Although the causes of this increased risk are not well-defined, both ageing and IPF have been associated with shortening of telomeres due to telomerase deficiency. Thoracic imaging with high-resolution computed tomographic (HRCT) scanning plays a key role in the diagnosis of ILD in the elderly, and a characteristic appearance of the lung parenchymal changes on HRCT may provide a confident diagnosis and obviate the need for invasive testing such as surgical lung biopsy. An effective treatment for IPF remains elusive, but many patients will benefit from supportive care and treatment of various co-morbid conditions that are often found in patients with IPF.
 ...
PMID:Interstitial lung disease in the elderly: pathogenesis, diagnosis and management. 2179 86

In 2000, the American Thoracic Society and European Respiratory Society published the first consensus statement providing guidelines on the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF). This statement presented, for the first time, diagnostic criteria for IPF and recommendations for treatment. Results from several studies have reshaped the thinking on IPF, and as a result, the guidelines have been recently revised using an evidence-based approach. Meanwhile, several epidemiologic studies have yielded data that identify potential risk factors and that better define the societal burden of IPF. This article summarizes the approach to diagnosing IPF and reviews epidemiologic data on IPF.
 ...
PMID:Idiopathic pulmonary fibrosis: diagnosis and epidemiology. 2236 44


<< Previous 1 2 3 4 5 6 7 Next >>