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      Evaluating the interstitial lung disease multidisciplinary meeting: a survey of expert centres

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          Abstract

          Background

          Multidisciplinary meetings (MDM) are the current “gold standard” in interstitial lung disease (ILD) diagnosis and comprise inter-disciplinary discussion of multiple forms of information to provide diagnostic and management outputs. Although bias could be potentially inserted at any step in the discussion process, to date there has been no consensus regarding the appropriate constitution and governance of MDM. We sought to determine the features of ILD MDMs based within ILD centres of excellence around the world.

          Methods

          An internet based questionnaire was sent to twelve expert centres in Europe, North America, and Australia seeking information regarding the structure and governance of their MDM. Data was analysed for consistent themes and points of contrast.

          Results

          Responses were received from 10 out of 12 centres. Similarities were demonstrated with regards to contributing attendees, meeting frequency and case numbers reviewed. Significant heterogeneity in attendee speciality group type, quantity and method of data presentation, approach to diagnosis formulation and documentation, and information provision was apparent.

          Conclusions

          The constitution of ILD MDMs differs considerably between expert centres. Such differences may result in discordant outcomes, and emphasise the need for further evidence regarding the appropriate constitution and governance of ILD MDMs.

          Electronic supplementary material

          The online version of this article (doi:10.1186/s12890-016-0179-3) contains supplementary material, which is available to authorized users.

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          Most cited references17

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          Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society.

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            Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model.

            Our purpose was to identify clinical, radiological and physiological (CRP) determinants of survival and to develop a CRP scoring system that predicts survival in newly diagnosed cases of idiopathic pulmonary fibrosis (IPF). The study population consisted of 238 patients with biopsy confirmed usual interstitial pneumonia. For each patient, clinical manifestations, chest radiographs, and pulmonary physiology were prospectively assessed. We used Cox proportional-hazards models to assess the effect of these parameters on survival. The effects of age and smoking were included in the analysis. Survival was related to age, smoking status (longer in current smokers), clubbing, the extent of interstitial opacities and presence of pulmonary hypertension on the chest radiograph, reduced lung volume, and abnormal gas exchange during maximal exercise. A mathematical CRP score for predicting survival was derived from these parameters. We showed that this CRP score correlated with the extent and severity of the important histopathologic features of IPF, i.e., fibrosis, cellularity, the granulation/connective tissue deposition, and the total pathologic derangement. Using these models, clinicians are in a better position to provide prognostic information to patients with IPF and to improve the selection of the most appropriate patients for lung transplantation or other standard or novel therapeutic interventions.
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              Hypersensitivity pneumonitis: insights in diagnosis and pathobiology.

              Hypersensitivity pneumonitis (HP) is a complex syndrome resulting from repeated exposure to a variety of organic particles. HP may present as acute, subacute, or chronic clinical forms but with frequent overlap of these various forms. An intriguing question is why only few of the exposed individuals develop the disease. According to a two-hit model, antigen exposure associated with genetic or environmental promoting factors provokes an immunopathological response. This response is mediated by immune complexes in the acute form and by Th1 and likely Th17 T cells in subacute/chronic cases. Pathologically, HP is characterized by a bronchiolocentric granulomatous lymphocytic alveolitis, which evolves to fibrosis in chronic advanced cases. On high-resolution computed tomography scan, ground-glass and poorly defined nodules, with patchy areas of air trapping, are seen in acute/subacute cases, whereas reticular opacities, volume loss, and traction bronchiectasis superimposed on subacute changes are observed in chronic cases. Importantly, subacute and chronic HP may mimic several interstitial lung diseases, including nonspecific interstitial pneumonia and usual interstitial pneumonia, making diagnosis extremely difficult. Thus, the diagnosis of HP requires a high index of suspicion and should be considered in any patient presenting with clinical evidence of interstitial lung disease. The definitive diagnosis requires exposure to known antigen, and the assemblage of clinical, radiologic, laboratory, and pathologic findings. Early diagnosis and avoidance of further exposure are keys in management of the disease. Corticosteroids are generally used, although their long-term efficacy has not been proved in prospective clinical trials. Lung transplantation should be recommended in cases of progressive end-stage illness.
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                Author and article information

                Contributors
                helen.jo@sydney.edu.au
                tameracorte@me.com
                Yuben.moodley@uwa.edu.au
                levinkovi@gmail.com
                G.Westall@alfred.org.au
                Peter.Hopkins@health.qld.gov.au
                Daniel.Chambers@health.qld.gov.au
                i.glaspole@alfred.org.au
                Journal
                BMC Pulm Med
                BMC Pulm Med
                BMC Pulmonary Medicine
                BioMed Central (London )
                1471-2466
                1 February 2016
                1 February 2016
                2016
                : 16
                : 22
                Affiliations
                [ ]Royal Prince Alfred Hospital, Sydney, Australia
                [ ]University of Sydney, Sydney, Australia
                [ ]University of Western Australia, Perth, Australia
                [ ]Fiona Stanley Hospital, Perth, Australia
                [ ]Respiratory Health Institute, Perth, Australia
                [ ]The Alfred Hospital, Melbourne, Australia
                [ ]Monash University, Melbourne, Australia
                [ ]Prince Charles Hospital, Brisbane, Australia
                [ ]University of Queensland, Brisbane, Australia
                Article
                179
                10.1186/s12890-016-0179-3
                4736654
                26831722
                7a0b1f4a-f48d-4f69-82b6-8bdf247523e2
                © Jo et al. 2016

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 8 July 2015
                : 13 January 2016
                Categories
                Research Article
                Custom metadata
                © The Author(s) 2016

                Respiratory medicine
                idiopathic pulmonary fibrosis,interstitial lung disease,multidisciplinary meeting,diagnosis

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