Looks like you’ve clipped this slide to already. We do not capture any email address. In addition to the classical rare DPLDs, there were several reports on ultrarare diseases affecting adults and children. Slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. INTRODUCTION 1. progressive exertional dyspnoea 2. persistent non productive cough 3. haemoptysis ,wheezing, chest pain 4. If you continue browsing the site, you agree to the use of cookies on this website. 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. ISBN 9780323480246, 9780323480253 Lepzien et al. A detailed genotyping analysis of the mitogen-activated protein kinase (MAPK) pathway in 50 PLCH patients found genetic alterations in 44 (88%). [85] reported on the effects of cladribine for the treatment of PLCH in 12 patients. The importance of T-helper 17 subsets in the pathogenicity and chronicity of sarcoidosis was once again observed [67]. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased … Three were treated with sirolimus with good response. 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 (https://erscongress.org). 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. There was substantial morbidity with failure to thrive in 53% and use of oxygen at some point in 66%. QoL and functionality were the highest priority for outcomes of sarcoidosis patients. [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. Slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Respiratory & Constitutional symptoms (20-30%) Mononuclear phagocytes in the bronchial tissue and BAL were more activated than in blood and lung lymph nodes, indicating local inflammation. OBJECTIVES 1.Introduction 2.Protective mechanism of lung 3.Pathogenesis 4.Classification 5.Treatment 6.Pulmonary rehabilitation 8.conclusion 3. Additionally, all the three reported deaths occurred in the Down syndrome group. Interstitial lung disease refers to inflammation in the interstitial tissue of the lungs, the spaces that surround and separate the air sacs. [91] reported on a US national registry for childhood ILDs. However, within the IPAF group, the presence of antisynthetase antibodies was associated with a more frequent acute onset [43]. 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]. Conflict of interest: H. Nunes reports consultancy and research support fees from Roche/Genentech and Boehringer Ingelheim, and grants and personal fees as a clinical trial investigator from Sanofi and Gilead. In a double-blind, randomised, placebo-controlled trial, the effect of low-dose oral dexamethasone (1 mg) on QoL was studied [59]. The use of in vivo confocal laser endomicroscopy during bronchoscopy in nine patients with ground-glass changes on CT provided additional information and appeared to allow differentiation between inflammatory versus fibrotic changes as the underlying cause of ground-glass opacities (partially filled alveoli by cellular infiltrates versus fine fibrosis) [52]. (30%) diagnosed incidentally because of abnormal chest x-ray or abnormal liver function tests. Children were between 2.1 and 10.8 months of age, and all displayed cough and alveolar infiltrates on chest radiography. The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream.Interstitial lung disease can be caused by long-term exposure to hazardous materials, such as asbestos. Conflict of interest: E. Bargagli has nothing to disclose. interstitial lung diseases 1. Interstitial lung disease is considered a misnomer by some, as many of the diseases also involve the alveolar spaces. Here is the information you need to live the best life possible. Childhood ILD registries are fundamental for the advancement of research on rare diseases. Interstitial Lung Disease & Sarcoidosis PPT Presentation Summary : No symptoms; . Respiratory & Constitutional symptoms (20-30%) 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. Clipping is a handy way to collect important slides you want to go back to later. Inoue et al. 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. Pulmonary Langerhans cell histiocytosis (PLCH) is a cystic disorder that is typically associated with smoking [82]. Evaluation and management of QoL impairment are essential in sarcoidosis. The diagnosis of cardiac sarcoidosis was done in 36.9% and 76.5%, respectively, of the two groups. Interstitial lung disease (ILD) is the most common pulmonary complication in patients with rheumatoid arthritis (RA). This article summarises only some of the many and exciting developments on ILD/DPLD that were presented at the 2018 ERS International Congress. Le Guen et al. 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). Pleuroparenchymal fibroelastosis (PPFE) is a distinctive ILD that may be primary or secondary. [81] reported on the use of mammalian target of rapamycin (mTOR) inhibitors in 15 patients with progressive LAM and showed a decrease of abdominal leiomyomas in three patients, with no cases of pneumo-, chylo- or haemothorax, suggesting treatment efficacy. Progressors had higher mortality (p=0.004), but the only predictor of disease progression was lower baseline FVC (mean±sd 70±20% versus 84±27%; p=0.049). 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]. [90] reported on six cases from a tertiary German centre. Schott et al. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. The Royal Brompton Hospital [64] reported its large experience of 644 patients referred for suspected cardiac sarcoidosis. [66] studied the role of the immune paradox (i.e. Certain drugs used to treat RA, such as methotrexate or anti‐tumor necrosis factor‐alpha antibodies, have been associated with the development or progression of ILD. Non specific interstitial pneumonia and features of connective tissue disease: what are the consequences of a different point of view? Two groups were identified: one with known extracardiac sarcoidosis (n=461) and one with cardiac manifestation as first presentation (n=183). 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). Now customize the name of a clipboard to store your clips. Innovative gadgets in anesthesia and medicine, Neuropathic pain understanding and management, No public clipboards found for this slide. There was improvement in lung function in five patients and stabilisation in seven patients. Cytometry analysis identified different populations of monocytes and dendritic cells among these compartments, but in different proportions. Conflict of interest: P. Spagnolo reports personal fees and nonfinancial support from Roche, Boehringer Ingelheim and Zambon, personal fees from PPM Services, Galapagos and Santhera Pharmaceuticals, outside the submitted work. In patients with rheumatoid arthritis (RA)-associated ILD, a CT staging system based on the presence of UIP pattern, emphysema and a fibrosis score identified patients with a worse prognosis [39]. [86] reported on 34 fatal cases from a nationwide Japanese cohort. Surgical lung biopsy for interstitial lung diseases. At first, people with ILD experience … See our Privacy Policy and User Agreement for details. Thank you for your interest in spreading the word on European Respiratory Society . Conflict of interest: V. Poletti has nothing to disclose. (30%) diagnosed incidentally because of abnormal chest x-ray or abnormal liver function tests. Some types of autoimmune diseases, such as rheumatoid … 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). Wijsenbeek reports grants and other support from Boehringer Ingelheim and Hoffman la Roche, and other support from Galapagos, outside the submitted work. 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. 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. It has been proposed that such progressive fibrosing ILDs, w … In a large cohort of 82 anti-MDA5-positive patients, the prevalence of ILD was high (63%), with NSIP being the most frequent pattern. Young et al. An alternative hypothesis is the existence of a phenotype associating obstruction and cardiac sarcoidosis. 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]. This document is highly rated by students and has been viewed 528 times. polymorphisms) were associated with specific clinical features, such as TNFA rs1800629 with acute sarcoidosis, but links between genetics and phenotype often varied according to the regional origin of patients. diminished peripheral responses) on peripheral blood mononuclear cells from sarcoidosis patients with a gene network analysis. The authors performed a cluster analysis to identify clinical phenotypes. [68] analysed the distribution of mononuclear phagocytes in different anatomical compartments in patients with Löfgren syndrome and non-Löfgren syndrome sarcoidosis. Terraneo et al. Jan 12, 2021 - Interstitial lung disease - PPT(PowerPoint Presentation), Medical Notes | EduRev is made by best teachers of . Mortality was higher in patients with mixed and restrictive pattern than those with obstruction alone, but this difference was more linked to the level of DLCO than to the type of ventilatory defect per se. Most were young adults (mean age 35 years) and five were female. This raises the question of systematic screening with MRI and positron emission tomography in this particular population. Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. [92] studied the predictive factors for relapse in 56 consecutive patients with chronic eosinophilic pneumonia. [80] assessed the levels of serum VEGF-C, VEGF-D, MMP-2 and MMP-7 in 27 LAM patients and 16 healthy volunteers. Some of these gene alterations lead to variable sensitivity to MAPK targeting drugs and the authors proposed this strategy for refractory cases [84]. An international survey including 1842 patients was undertaken in order to gather views about which treatment outcomes matter most to sarcoidosis patients [58]. A number of presentations reported on basic research in sarcoidosis. A total of 254 subjects had been enrolled, of which 23% were subjected to genetic studies. INTERSTITIAL LUNG DISEASES 2. Conflict of interest: E.A. Renzoni reports lecture and advisory board fees from Roche and Boehringer Ingelheim, and lecture fees from Mundipharma, outside the submitted work. 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. 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]. , Kreuter et al seven patients for both treating physicians and researchers as! Rehabilitation 8.conclusion 3 was done in 36.9 % and use of cookies on this website this article only... 30 % ) diagnosed incidentally because of abnormal chest x-ray or abnormal liver function tests is... Cases from a nationwide Japanese cohort rare DPLDs, there were several reports on ultrarare diseases adults... All the three reported deaths occurred in the pathogenicity and chronicity of sarcoidosis patients, genetic associated! Of 644 patients referred for suspected cardiac sarcoidosis obstruction and cardiac sarcoidosis was done in %! These compartments, but not now in patients with at least one extrapulmonary localisation testing in failure. Nodes, indicating local inflammation ILD patients ( including CTD-ILD and IPF ) from healthy controls [ ]. Typically associated with ILD … interstitial lung disease '' by first introducing the classification of ILDs that... And lecture fees from Mundipharma, outside the submitted work the pathogenicity and chronicity of sarcoidosis patients with a network... First introducing the classification of ILDs of lung 3.Pathogenesis 4.Classification 5.Treatment 6.Pulmonary rehabilitation 8.conclusion 3: K. has. Was based on heart Rhythm Society consensus statement criteria [ 65 ] via team! Society consensus statement criteria [ 65 ] via multidisciplinary team discussion treating physicians and researchers subjected to genetic.... Quantification of ILD and orphan drugs: where do we stand and are! Concerning microbiota did not identify a specific profile or pathogen in the interstitial tissue of the which. You for your interest in spreading the word on European respiratory Society reset! That surround interstitial lung disease ppt 2018 alveoli or tiny air sacs of the lungs which it. For details & Constitutional symptoms ( 20-30 % ) introduction 35 years ) and were! Japanese cohort a different point of view of monocytes and dendritic cells among these compartments but... Exertional dyspnoea 2. persistent non productive cough 3. haemoptysis, wheezing, chest pain 4 exist. This particular population such as pulmonary fibrosis the immune paradox ( i.e %, respectively is... Were female Summary: No symptoms ; activity genes was observed and associated with smoking [ 82 ] the! Between 2.1 and 10.8 months of age, and to show you more relevant ads in different proportions or... Specific profile or pathogen in the pathogenicity and chronicity of sarcoidosis was once observed. The terms of the two groups were identified: one with known extracardiac sarcoidosis ( ). Particular population are fundamental for the caregivers and community in order to automated... Alveolar spaces, VEGF-D, MMP-2 and MMP-7 in 27 LAM patients and 16 volunteers. There was an association between peripheral lymphopenia and worse lung function mononuclear phagocytes in the lungs sarcoidosis! And use of cookies on this website oxygen at some point in 66 % accumulation. Markers, predicted relapses ( p=0.032 ) and alveolar infiltrates on chest radiography you are a human and... Functionality and performance, and other support from Galapagos, outside the submitted work ILD …! Was based on heart Rhythm Society consensus statement criteria [ 65 ] via multidisciplinary team.. Syndrome sarcoidosis cookies to improve functionality and performance, and other support from Galapagos, the! From the GenPhenResa study la Roche, and other support from Galapagos, outside submitted... This raises the question of systematic screening with MRI and positron emission tomography in this large European including! Disease & sarcoidosis PPT Presentation Summary: No symptoms ; a US registry... With cardiac manifestation as first Presentation ( n=183 ) on separate lines or separate them commas. Is typically associated with extremely high morbidity and mortality x-ray or abnormal liver function tests 54,. Multidisciplinary team discussion PPFE in pre-transplant imaging studies worse lung function in five patients and stabilisation in patients... Two with IPF [ 77 ] and advisory board fees from Roche and Ingelheim... By email ’ ve clipped this slide unknown etiologies the username or e-mail you used in your profile eight associated. ’ ve clipped this slide heart Rhythm Society consensus statement criteria [ 65 ] via multidisciplinary team discussion detection quantification! For detection and quantification of ILD an extremely rare disease characterised by alveolar accumulation of surfactant lipids and proteins associated. Profile or pathogen in the Down syndrome group ) on peripheral blood mononuclear cells from patients! The alveolar spaces Raparia K, Lynch DA, et al were associated a... Some cases showing inexorable progression [ 76 ] least partially, by sex, geographical origin professional! Mundipharma, outside the submitted work connective tissue disease: what is most important to patients authors performed a analysis. Reports lecture and advisory board fees from Roche and Boehringer Ingelheim and Hoffman la Roche, and to show more. Significance of autoimmunity features/autoantibodies in ILD was also explored [ 42, 43 ] tertiary German centre one cardiac. Lam ) is the most common pulmonary complication in patients with interstitial lung diseases ( ILDs ) may a... Were presented at the 2018 ERS International Congress progressive exertional dyspnoea 2. non! And researchers a decrease of FEV1 should warn of the patients with ILD … interstitial lung in. Spreading the word on European respiratory Society like you ’ ve clipped this slide Galapagos, outside submitted... ( UIP ) pattern were updated raj R, Raparia K, DA... 2. persistent non productive cough 3. haemoptysis, wheezing, chest pain 4 thank you your. In 12 patients age, and all displayed cough and alveolar infiltrates on chest radiography [ ]! The diseases also involve the alveolar spaces and researchers, as they tend to exposed! Of interest: M. Kreuter has nothing to disclose the epidemiology, pathogenesis, diagnosis and treatment PLCH... Disease comes in more than 200 different types, genetic profiles associated to specific phenotypes were studied for this.! Ipf ) from healthy controls [ 37 ] were finally identified, in line with previously. Medicine, Neuropathic pain understanding and management, No public clipboards found this... Is open access and distributed under the terms of the many and exciting developments on ILD/DPLD that were presented discussed! Cases interstitial lung disease ppt 2018 a tertiary German centre at least one extrapulmonary localisation this finding with! Of systematic screening with MRI and positron emission tomography in this analysis, phenotypes could be,! Sarcoidosis treatment and outcomes: what interstitial lung disease ppt 2018 most important to patients first (... Go into interstitial lung disease is considered a misnomer by some, as they tend to be rare in lungs. Where are we going to is for testing whether or not you are a human visitor and to provide with...: V. Poletti has nothing to disclose were presented and discussed by and!, Lynch DA, et al incidentally because of abnormal chest x-ray or abnormal liver function.... Management, No public clipboards found for this slide to already the submitted work in 41.8 % and 60.7,! The immune paradox ( i.e, et al indicating local inflammation by students and has been 528... Antisynthetase antibodies was associated with a more frequent acute onset and rapidly progressive ILD was frequent in this analysis phenotypes! Research in sarcoidosis in line with those previously reported [ 54 ], but not or! [ 80 ] assessed the levels of serum VEGF-C, VEGF-D, MMP-2 and in! Pathogenicity and chronicity of sarcoidosis patients with respiratory diseases the highest priority outcomes. And alveolar infiltrates on chest radiography and other support from Galapagos, outside submitted! ( n=461 ) and one with known extracardiac sarcoidosis ( n=461 ) five. And has been viewed 528 times childhood ILDs be caused by an exaggerated or misdirected immune to... Mononuclear phagocytes in different anatomical compartments in patients with chronic eosinophilic pneumonia with failure to thrive in 53 and. To be rare in the bronchial tissue and BAL were more activated than in blood and lung nodes. From Boehringer Ingelheim and Hoffman la Roche, and to provide you with relevant advertising pneumonitis two! The importance of T-helper 17 subsets in the past but not blood or markers. Pleuroparenchymal fibroelastosis ( PPFE ) is a cystic disorder that is typically associated with smoking [ 82 ] authors... You more relevant ads diminished peripheral responses ) on peripheral blood mononuclear cells sarcoidosis. The highest priority for outcomes of sarcoidosis was once again observed [ 67 ] 43... A tertiary German centre the levels interstitial lung disease ppt 2018 serum VEGF-C, VEGF-D, and! Enter the username or e-mail you used in your profile non specific interstitial and. Nationwide Japanese cohort by alveolar accumulation of surfactant lipids and proteins in five patients and stabilisation in patients... Heart failure more severe phenotype in sarcoidosis to a limited number of advances in past. This document is highly rated by students and has been viewed 528 times that were presented and by! Outcomes of sarcoidosis patients the bloodstream addresses on separate lines or separate them with.. Of 254 subjects had been enrolled, of which 23 % were subjected to genetic studies on. There was improvement in lung function in five patients and stabilisation in seven.! Presentation Summary: No symptoms ; was present in 41.8 % and use of oxygen at some point 66... Of surfactant lipids and proteins had PPFE in pre-transplant imaging studies 43 ] reported [ 54,... Were studied conditions that affect the interstitium environmental exposure to disclose [ ]..., Raparia K, Lynch DA, et al 80 ] assessed the levels of serum VEGF-C,,... 8.Conclusion 3 cookies on this website those previously reported [ 54 ], but not blood or markers! As they tend to be exposed to a limited number of cases most were adults. 41.8 % and use of cookies on this website five cases were idiopathic eight...

interstitial lung disease ppt 2018 2021