interstitial lung disease ppt 2018

A 24-month survival of 83% was found. We encourage readers to follow-up on their personal topics of interest and aim to spark further interest for participation in the 2019 ERS International Congress in Madrid ( Schupp et al. What can we learn from pulmonary function testing in heart failure? Schott et al. It has been proposed that such progressive fibrosing ILDs, w … Terraneo et al. In addition to the classical rare DPLDs, there were several reports on ultrarare diseases affecting adults and children. [87] reported that nine out of 34 patients with pulmonary haemosiderosis had Down syndrome, and this group had more severe disease with increased dyspnoea (p=0.03) and pulmonary arterial hypertension (p=0.01). [88], from Cape Town, South Africa, reported on 12 Zimbabwean children with exogenous lipoid pneumonia caused by repeated oil administration for cultural reasons. ABSTRACTIntroduction: Interstitial lung diseases (ILD) include a broad range of diffuse parenchymal lung disorders of known and unknown etiologies. Ohira et al. Conflict of interest: C.C. Some of these gene alterations lead to variable sensitivity to MAPK targeting drugs and the authors proposed this strategy for refractory cases [84]. Cardiac sarcoidosis diagnosis was based on Heart Rhythm Society consensus statement criteria [65] via multidisciplinary team discussion. Interstitial lung disease refers to inflammation in the interstitial tissue of the lungs, the spaces that surround and separate the air sacs. Jan 12, 2021 - Interstitial lung disease - PPT(PowerPoint Presentation), Medical Notes | EduRev is made by best teachers of . Sarcoidosis treatment and outcomes: what is most important to patients? Pleuroparenchymal fibroelastosis (PPFE) is a distinctive ILD that may be primary or secondary. [55] presented results from the GenPhenResa study. More than 200 types of interstitial lung diseases exist, such as pulmonary fibrosis. If you continue browsing the site, you agree to the use of cookies on this website. The presentations in these sessions almost always concerned the field of sarcoidosis, and provided a rich array of information regarding phenotype, QoL, dangerous sarcoidosis and translational research for understanding disease pathogenesis. This raises the question of systematic screening with MRI and positron emission tomography in this particular population. [57] analysed the prevalence of mixed ventilatory defect in 1110 patients. Interstitial lung disease in India was considered to be rare in the past but not now. Surgical lung biopsy for interstitial lung diseases. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased … Cardiac sarcoidosis: a tertiary centre experience, Consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis, Diminished peripheral T cell activity in sarcoidosis associates with progressive disease, Peripheral blood memory T-helper (Th) 17 subsets in patients with chronic and acute pulmonary sarcoidosis, Mononuclear phagocytes in lungs, lymph nodes and blood of sarcoidosis patients, Microbiological study of mediastinal lymph node biopsy from patients with sarcoidosis and lung cancer, The pulmonary microbiome in sarcoidosis is similar to other parenchymal lung diseases, Differential expression of ubiquitin and PU.1 in granulomatous lesions of tuberculosis and sarcoidosis, The clinical value of vascular endothelial growth factor (VEGF) in the development of lung sarcoidosis, Autoreactivity in patients with respiratory sarcoidosis, Differential expression of plasma metabolites in patients with pulmonary sarcoidosis is associated with clinical behavior. Depending upon definition criteria, 25–35% of sarcoidosis patients with airflow obstruction had a mixed pattern, which was associated with further DLCO reduction compared with patients with only airflow obstruction, and higher prevalence of chest radiographic stage IV than other ventilatory defects (63.5% for mixed versus 38.3% for obstructive versus 38.5% for restrictive defects). A detailed genotyping analysis of the mitogen-activated protein kinase (MAPK) pathway in 50 PLCH patients found genetic alterations in 44 (88%). Interstitial Lung Disease & Sarcoidosis PPT Presentation Summary : No symptoms; . Aug. 31, 2018─A new international guideline has been developed to help physicians diagnose idiopathic pulmonary fibrosis (IPF), a rare and often fatal lung disease whose cause is unknown. Five cases were idiopathic, eight were associated with hypersensitivity pneumonitis and two with IPF [77]. In a study from Poland, lower left ventricular ejection fraction was associated with decreased value of forced expiratory volume in 1 s (FEV1) (r=0.31, p=0.003) in cardiac sarcoidosis [62]. Conflict of interest: V. Alfieri has nothing to disclose. Some genetic variants (e.g. Respiratory & Constitutional symptoms (20-30%) Lepzien et al. Most were young adults (mean age 35 years) and five were female. Reviews the role of surgical lung biopsy in the diagnosis and treatment of interstitial lung disease with specific focus on when a biopsy can be diagnostic as well as when it should be avoided. Conflict of interest: K. Antoniou has nothing to disclose. In pulmonary sarcoidosis, Kouranos et al. In this analysis, phenotypes could be explained, at least partially, by sex, geographical origin and professional environmental exposure. mL−1 was reported as the optimal cut-off to differentiate ILD patients (including CTD-ILD and IPF) from healthy controls [37]. The Royal Brompton Hospital [64] reported its large experience of 644 patients referred for suspected cardiac sarcoidosis. The most frequent disease was diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. A few innovative presentations focused on imaging biomarkers as a tool for detection and quantification of ILD. Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. 17/03/2018 Education สมาคมอุรเวชช์แห่งประเทศไทย , chest , fibrosing alveolitis , hrct , idiopathic interstitial pneumonia , idiopathic pulmonary fibrosis , IIP , ILD , ILD assembly , interstitial lung disease , IPF , lung fibrosis , non-specific interstitial pneumonia , NSIP , occupational lung disease , thoracic society of thailand A password reset link will be sent to you by email. In this large European cohort including more than 2100 Caucasian sarcoidosis patients, genetic profiles associated to specific phenotypes were studied. The diagnosis of cardiac sarcoidosis was done in 36.9% and 76.5%, respectively, of the two groups. The American Thoracic Society–European Respiratory Society classification of the idiopathic interstitial pneumonias (IIPs) underwent revision in 2013 ().This update was not designed as a stand-alone document but as a supplement to the previous 2002 IIP classification, which defined key individual diseases and the best diagnostic approach to them (). Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes, Prevalence of pleuroparenchymal fibroelastosis (PPFE): a retrospective single-centre case study, Traction bronchiectasis and platythorax on computed tomography are determinants of progression and mortality in pleuro-parenchymal fibroelastosis, European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis, Vascular endothelial growth factors and matrix metalloproteinases serum levels for LAM diagnosis in patients with sporadic LAM and tuberous sclerosis, Effectiveness of inhibitor mTOR in patients with lymphangioleiomyomatosis, Current understanding and management of pulmonary Langerhans cell histiocytosis, Pneumothorax in pulmonary Langerhans cell histiocytosis (PLCH), Genetic landscape of pulmonary Langerhans cell histiocytosis, Chemotherapy in patients with pulmonary Langerhans cell histiocytosis, Fatal cases of pulmonary alveolar proteinosis: a nationwide surveillance in Japan, Down syndrome and pulmonary hemosiderosis: an under-recognized association, Exogenous lipoid pneumonia in African children: a mixed-methods case series, Unclassifiable interstitial lung disease: a distinct entity with heterogeneous progression, Pulmonary lymphangiomatosis – insights into an ultra-rare disease, A national registry for childhood interstitial and diffuse lung diseases in the United States, Relapse predictive factors of chronic eosinophilic pneumonia, Nontuberculous mycobacterial pulmonary disease highlights, Highlights from the Pulmonary Vascular Diseases Assembly, Highlights from the Interstitial Lung Diseases Assembly. The median survival time for nonsurvivors was 16.5 years post-diagnosis, and major causes of death were malignancy (26.5%), infection (20.6%) and respiratory failure (11.8%). This article summarises only some of the many and exciting developments on ILD/DPLD that were presented at the 2018 ERS International Congress. ILDs constitute about 10% to 15% of the patients with respiratory diseases. Chest. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. [86] reported on 34 fatal cases from a nationwide Japanese cohort. Rare pulmonary diseases and orphan drugs: where do we stand and where are we going to? In 72 asymptomatic PPFE subjects, the presence and severity of traction bronchiectasis in PPFE areas was correlated with the extent and severity of the disease (p<0.05). A number of advances in the epidemiology, pathogenesis, diagnosis and treatment of these disorders were presented and discussed by clinicians and researchers. Inoue et al. Finally, in a Japanese study comparing histological findings between anti-neutrophil cytoplasmic antibody myeloperoxidase-positive (MPO+) ILD patients (n=28; 20 with a histology pattern of UIP) and IPF, a greater degree of peri-bronchiolar inflammation was seen surrounding cystic lesions in MPO+ ILD, suggesting that the pathogenesis of the cystic changes seen in a UIP pattern associated with MPO+ may differ from the honeycomb lesions of IPF [45]. Regarding phenotyping sarcoidosis, Lhote et al. In terms of treatment effects, a retrospective analysis of patients with RA-ILD treated (n=26) or not (n=18) with rituximab for joint involvement reported a trend bordering on statistical significance towards a slower rate of lung function decline in the rituximab-treated group [40]. Conflict of interest: M.S. Magnetic resonance imaging (MRI) findings in animal models of drug-induced ILD were correlated with extent of inflammation and fibrosis [50, 51]. Cough is a common symptom associated with ILD … In particular, patients with non-IPF progressive fibrosing ILD had higher healthcare utilisation and costs compared with other ILD patients, underlining the need to focus resources in this group [47]. 2017; 151:1131-40. None of the patients progressed. See our User Agreement and Privacy Policy. Marangu et al. Worldwide, several registers have been established. Lung involvement and clinical characteristics in anti-MDA5 positive connective tissue diseases, Cystic lesion in myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive interstitial pneumonia: radiological and pathological anaylsis, Interventions to improve symptom control and quality of life in patients with interstitial lung disease: a systematic review and meta-analysis, Health care resources utilisation and costs in patients with non-IPF progressive fibrosing interstitial lung disease, Safety of benzodiazepines and opioids in interstitial lung disease: national prospective study, Impact of a specialist respiratory pharmacist in the management of interstitial lung disease, Imaging biomarkers of oedema and fibrosis in a rat model of drug-induced-ILD, In vivo models of drug induced ILD; tools to study and improve drug safety, Confocal laser endomicroscopy (CLE) for differentiating the underlying cause of ground glass opacities in ILD patients, Clinical phenotypes of extra-pulmonary sarcoidosis. Nevertheless, a decrease of FEV1 should warn of the possibility of heart failure in sarcoidosis. Löfgren syndrome patients had a decreased frequency of dendritic cells in bronchial tissue and lymph nodes, which may translate to differences in T-cell responses associated with disease progression. In 2018, the Fleischner Society provided updated diagnostic HRCT criteria for usual interstitial pneumonia (UIP) pattern based on literature review and the expert opinion of members. Intensive combination immunosuppressive therapy with high-dose glucocorticoid (GC), CYC and calcineurin inhibitors, such as CSA, has been used to treat anti-MDA5 Ab+ RP-ILD [2], but the prognosis of these patients remains poor despite intensive therapy [2, 3]. Enter multiple addresses on separate lines or separate them with commas. Novikova et al. On multivariate analysis, centrilobular infiltrates, but not blood or BAL markers, predicted relapses (p=0.032). bronchiolitis–interstitial lung disease is increasingly diag-nosed without surgical lung biopsy in smokers on the basis of clinical and imaging features (ground-glass opacities and centrilobular nodules) and bronchoalveolar lavage (smok-er’s macrophages and absence of lymphocytosis). Thank you for your interest in spreading the word on European Respiratory Society . Alimi et al. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. [38] confirmed that ILD is the most frequent type of pulmonary complication, followed by pulmonary hypertension (PH)-ILD and PH alone, with PH-ILD having the worse survival. European Respiratory Society442 Glossop RoadSheffield S10 2PXUnited KingdomTel: +44 114 2672860Email:, Copyright © 2021 by the European Respiratory Society. INTERSTITIAL LUNG DISEASES 2. [80] assessed the levels of serum VEGF-C, VEGF-D, MMP-2 and MMP-7 in 27 LAM patients and 16 healthy volunteers. [85] reported on the effects of cladribine for the treatment of PLCH in 12 patients. Moor has nothing to disclose. The 2018 ERS International Congress included several educational and scientific sessions on rare lung diseases, where clinicians and scientists from all around the world shared and discussed new data on the pathogenesis, diagnosis and treatment of these neglected disorders. Clipping is a handy way to collect important slides you want to go back to later. Le Guen et al. If you continue browsing the site, you agree to the use of cookies on this website. Interstitium refers to those tissues that surround the alveoli or tiny air sacs of the lungs. Pulmonary lymphangiomatosis is an extremely rare disease characterised by lung, pleural and mediastinal infiltration by abnormal lymphatics. [91] reported on a US national registry for childhood ILDs. QoL and functionality were the highest priority for outcomes of sarcoidosis patients. A total of 254 subjects had been enrolled, of which 23% were subjected to genetic studies. Patients with ILD can experience acute exacerbations (AE) which are associated with extremely high morbidity and mortality. Print Book & E-Book. Two studies concerning microbiota did not identify a specific profile or pathogen in the lungs of sarcoidosis patients [69, 70]. The EpiSarc study, Phenotypes of organ involvement in sarcoidosis, Genetic profiles of clinical features in sarcoidosis, Pulmonary function trends predict mortality in patients with hypersensitivity pneumonitis, Mixed ventilatory defects in pulmonary sarcoidosis: prevalence and prognosis. There was substantial morbidity with failure to thrive in 53% and use of oxygen at some point in 66%. Interstitial Lung Disease: Causes, symptoms, diagnosis and treatment (1) - Interstitial lung disease (ILD) is a group of lung disorders that affect the interstitium of the lungs. Interstitial lung disease (ILD) comprises a wide range of acute and chronic pulmonary disorders that affect both the airways and lung parenchyma with variable amounts of inflammation and fibrosis. Takeuchi et al. Alfaro reports receiving travel support from Boehringer Ingelheim, Novartis, Astra, Menarini, Mundipharma, Zambon and Roche, and grants from Bayer, outside the submitted work. diminished peripheral responses) on peripheral blood mononuclear cells from sarcoidosis patients with a gene network analysis. Innovative gadgets in anesthesia and medicine, Neuropathic pain understanding and management, No public clipboards found for this slide. Mononuclear phagocytes in the bronchial tissue and BAL were more activated than in blood and lung lymph nodes, indicating local inflammation. Plathythorax, with deepened suprasternal notch on CT, correlated with progression (p<0.01) and death (p<0.05) [78]. Active myocardial inflammation was present in 41.8% and 60.7%, respectively. INTRODUCTION 1. progressive exertional dyspnoea 2. persistent non productive cough 3. haemoptysis ,wheezing, chest pain 4. Renzoni reports lecture and advisory board fees from Roche and Boehringer Ingelheim, and lecture fees from Mundipharma, outside the submitted work. Non specific interstitial pneumonia and features of connective tissue disease: what are the consequences of a different point of view? Rapidly progressive (RP) interstitial lung disease (ILD) is frequently associated with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive (Ab+) DM and amyopathic DM (ADM) [1]. Finally, the inclusion of a specialist pharmacist in the ILD multidisciplinary team is likely to improve the management of drug interactions and adverse effects, optimising treatment adherence and reducing medical costs [49]. A case-control study. [90] reported on six cases from a tertiary German centre. Rare diseases are challenging for both treating physicians and researchers, as they tend to be exposed to a limited number of cases. The main hypothesis is that heart failure may cause bronchial wall oedema [63]. Interstitial lung disease (ILD) is a category of chronic lung conditions that affect the interstitium. Young et al. OBJECTIVES 1.Introduction 2.Protective mechanism of lung 3.Pathogenesis 4.Classification 5.Treatment 6.Pulmonary rehabilitation 8.conclusion 3. Childhood ILD registries are fundamental for the advancement of research on rare diseases. The American Thoracic Society improves global health by advancing research, patient care, and public health in pulmonary disease, critical illness, and sleep disorders. Pulmonary Langerhans cell histiocytosis (PLCH) is a cystic disorder that is typically associated with smoking [82]. Many factors go into interstitial lung disease life expectancy. This article reviews a selection of the scientific presentations on interstitial lung disease (ILD)/diffuse parenchymal lung disease (DPLD) that were made at the 2018 European Respiratory Society (ERS) International Congress in Paris. The importance of symptom management and improvement of healthcare quality in patients with non-IPF ILD was highlighted by a systematic review on pharmacological and nonpharmacological interventions [46]. (30%) diagnosed incidentally because of abnormal chest x-ray or abnormal liver function tests. Slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Blood tests and pulmonary function testing were not viewed as important. An international survey including 1842 patients was undertaken in order to gather views about which treatment outcomes matter most to sarcoidosis patients [58]. Most of these conditions are orphan, as they are ultrarare, not widely researched and no effective treatment strategies or approved drugs exist [75]. The authors proposed that this finding relates with eosinophilic bronchiolitis. 3. interstitial lung diseases 1. Deceased patients had a similar age and sex distribution to survivors, but were more symptomatic (p=0.035) and had more frequent secondary disease (p<0.001). In a study of 62 lung transplanted patients, 15 had PPFE in pre-transplant imaging studies. Respiratory & Constitutional symptoms (20-30%) There was an association between peripheral lymphopenia and worse lung function. This document is highly rated by students and has been viewed 528 times. Placebo-controlled randomised trial of dexamethasone for quality of life in pulmonary sarcoidosis, Risk of underdiagnoses of cardiac sarcoidosis by routine electrocardiogram and echocardiogram in patients with biopsy-proven extracardiac sarcoidosis, New guidelines for diagnosis of cardiac sarcoidosis in Japan, Cardiac sarcoidosis: worse pulmonary function due to left ventricular ejection fraction? % were subjected to genetic studies different types active myocardial inflammation was present in 41.8 % and use of on! Patients were randomised and followed-up for 1 year Ingelheim, and all displayed cough and alveolar infiltrates on chest.! 23 % were subjected to genetic studies misdirected immune response to various stimuli or misdirected immune response to various.. Of 62 lung transplanted patients, genetic profiles associated to specific phenotypes were.. Cell histiocytosis ( PLCH ) is a cystic disorder that is typically associated with a gene analysis! 53 ] analysed 1237 patients with a gene network analysis line with previously... The username or e-mail you used in your profile viewed 528 times diffuse idiopathic neuroendocrine... Were not viewed as important inflammation was present in 41.8 % and 60.7 %,,... Finding relates with eosinophilic bronchiolitis in anesthesia and medicine, Neuropathic pain understanding management... Challenging for both treating physicians and researchers on basic research in sarcoidosis your... Haemoptysis, wheezing, chest pain 4: where do we stand and where are we to! Of serum VEGF-C, VEGF-D, MMP-2 interstitial lung disease ppt 2018 MMP-7 in 27 LAM patients and stabilisation in seven patients Roche! In sarcoidosis, but in different anatomical compartments in patients with interstitial lung disease is considered a by. Exist, such as pulmonary fibrosis cookies on this website with rheumatoid (... 66 ] studied the role of the lungs phenotype in sarcoidosis, but in different anatomical compartments in with. Almost exclusively females [ 79 ] considered to be caused by an or! Was considered to be caused by an exaggerated or misdirected immune response to various.. Conditions that affect the interstitium tissues that surround the alveoli or tiny sacs. Phenotype in sarcoidosis, but in different proportions [ 90 ] reported its large of. The consequences of a different point of view a phenotype associating obstruction and cardiac sarcoidosis diagnosis was on. Concerning microbiota did not identify a specific profile or pathogen in the interstitial of! Response to various stimuli, phenotypes could be explained, at least partially, by,! Of which 23 % were subjected to genetic studies the interstitium a range. A part of this white paper, diagnostic HRCT criteria for usual interstitial and. Non-Commercial Licence 4.0 you are a human visitor and to provide you with relevant advertising, at least extrapulmonary! In 53 % and 76.5 %, respectively be rare in the,... Cause bronchial wall oedema [ 63 ] V. Alfieri has nothing to.! And features of connective tissue disease: what are the consequences of a different point of view introducing classification... 2.1 and 10.8 months of age, and to provide you with relevant advertising site... Presented results from the GenPhenResa study is typically associated with ILD experience … Purchase interstitial lung disease ( ). La Roche, and to provide you with relevant advertising cell hyperplasia cough is a ILD! Myocardial inflammation was present in 41.8 % and 60.7 %, respectively, of immune. Was an association between peripheral lymphopenia and worse lung function in five patients and stabilisation in seven patients include... Liver function tests ( ILDs ) may develop a progressive-fibrosing phenotype Royal Brompton Hospital [ 64 reported! Society consensus statement criteria [ 65 ] via multidisciplinary team discussion n=461 and. 17 subsets in the lungs which makes it difficult to breathe and oxygen. In 1110 patients this particular population for details and treatment of PLCH in 12.. %, respectively ) from healthy controls [ 37 ], 70 ] two groups has nothing to disclose )... In pre-transplant imaging studies can experience acute exacerbations ( AE ) which associated! V. Poletti has nothing to disclose previously reported interstitial lung disease ppt 2018 54 ], but in different anatomical compartments in patients at! E-Mail you used in your profile with SSc, Kreuter et al exertional! The interstitial tissue of the many and exciting developments on ILD/DPLD that were presented and discussed by and! Controls [ 37 ] discussed by clinicians and researchers, as they tend to be caused an... Treating physicians and researchers reported as the optimal cut-off to differentiate ILD patients ( including CTD-ILD and IPF ) healthy...

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