AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPsychiatry and PsychologyPhenomena and ProcessesDisciplines and OccupationsAnthropology, Education, Sociology and Social PhenomenaInformation ScienceHealth Care
Idiopathic Pulmonary FibrosisCystic FibrosisPulmonary FibrosisLungLung DiseasesLung Diseases, InterstitialCystic Fibrosis Transmembrane Conductance RegulatorPseudomonas aeruginosaSputumBleomycinBronchiFibrosisBronchoalveolar Lavage FluidDisease ProgressionAlveolitis, Extrinsic AllergicIdiopathic Interstitial PneumoniasPseudomonas InfectionsLung TransplantationRespiratory Function TestsRespiratory MucosaPulmonary AlveoliSarcoidosis, PulmonaryFibroblastsMyofibroblastsBronchoalveolar LavageHydroxyprolineVital CapacityPulmonary Diffusing CapacityPulmonary Surfactant-Associated Protein CCryptogenic Organizing PneumoniaPyridonesEpithelial CellsPneumocytesLung InjurySarcoidosisRespiratory System AgentsCollagen DiseasesLung NeoplasmsTotal Lung CapacityPulmonary EmphysemaLung Diseases, ObstructiveTransforming Growth Factor beta1CollagenBurkholderia cepacia complexBiopsyTomography, X-Ray ComputedTransforming Growth Factor betaScleroderma, SystemicPhenotypeLeukocyte ElastaseDyspneaMacrophages, AlveolarMice, Inbred C57BLRespiratory AspirationPulmonary Surfactant-Associated Protein AHypertension, PulmonaryDisease Models, AnimalRetrospective StudiesPneumonia, BacterialMucin-5BImmunohistochemistryBiological MarkersPrognosisPneumoniaNeutrophilsConnective Tissue DiseasesCase-Control StudiesSeverity of Illness IndexPulmonary Surfactant-Associated Protein DTreatment OutcomeAntibiotics, AntineoplasticInterleukin-8RNA, MessengerPrednisoloneLung Volume MeasurementsCells, CulturedForced Expiratory VolumeBiofilmsPulmonary MedicineTime FactorsCystitis, InterstitialCollagen Type IHSP47 Heat-Shock ProteinsTracheaDiagnostic Techniques, Respiratory SystemEmphysemaPrimary Graft DysfunctionSignal TransductionChronic DiseasePulmonary Gas ExchangeTherapeutic IrrigationInterstitial Cells of CajalSmokingMice, KnockoutAcute DiseasePredictive Value of TestsEpithelial-Mesenchymal TransitionHemoperfusionExpectorantsSpirometry
Chronic obstrucPneumoniasInflammation and fibrosisPatients with idiopathicHigh-resolution computed tomographyClinicalILDsPneumonitisDiagnosis of idiopathic pulmonarRheumatoid arthritisMortalityAbstractHRCTFibrotic lung diseaseTherapiesPirfenidoneSymptomsUnknown etiologyBiopsyEmphysemaDivision of PulmonaryBleomycin-inducedNintedanibTreatment of idiopathicAcuteTomographyParenchymaTransplantationInclude idiopathicSurvivalContributes to pulmonary fibrosisProgressiveHypertensionSystemic sclerosisInnate immunityDisordersType of interstitialMedicationHistologicallyPattern of usual interstitEpithelial cellsPathologyTissueBronchiolitisDiffusePathogenesis of pulmonaryHeterogeneous groupCysticFibroblast activationRespiratory diseasesMedscapeUsual interstitial
Chronic obstruc11
  • Common comorbid medical conditions found in patients with idiopathic pulmonary fibrosis (IPF) include chronic obstructive pulmonary disease , obstructive sleep apnea , gastroesophageal reflux disease , and coronary artery disease . (medscape.com)
  • The UW investigators believe that understanding the genetic determinants of lung volume may eventually lead to improved treatments for diseases such as pulmonary fibrosis and chronic obstructive pulmonary disease, both of which currently have limited effective therapeutic options. (uw.edu)
  • Chronic obstructive pulmonary disease (COPD) and other respiratory illnesses are debilitating and often require lifelong management. (viezec.com)
  • The most common type of lung disease is chronic obstructive pulmonary disease (COPD), which includes conditions such as emphysema and chronic bronchitis. (viezec.com)
  • The most common types of lung diseases include asthma, bronchitis, and COPD (chronic obstructive pulmonary disease). (viezec.com)
  • However, in some other indications, including chronic obstructive pulmonary disease (COPD), this remains less clear because a substantial improvement in quality of life may not be matched by an increase in overall survival. (ersjournals.com)
  • Diagnosing IPF in clinical practice can be challenging, as symptoms often appear similar to those of more common respiratory diseases, such as chronic obstructive pulmonary disease. (biomedcentral.com)
  • Although exercise training effectively improves exercise capacity and health related quality of life in patients with chronic obstructive pulmonary disease (COPD), 4 its role in patients with ILD is unclear. (bmj.com)
  • Chronic Obstructive Pulmonary Disease (COPD) is a disease of the airways and air sacs of the lung. (thelungresearchcenter.com)
  • The cutoff point of clinical chronic obstructive pulmonary disease questionnaire for more symptomatic patients. (medscape.com)
  • The prevalence, burden and risk factors associated with chronic obstructive pulmonary disease in Commonwealth of Independent States (Ukraine, Kazakhstan and Azerbaijan): results of the CORE study. (medscape.com)
Pneumonias4
Inflammation and fibrosis6
Patients with idiopathic8
High-resolution computed tomography2
  • All patients fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism criteria, had mild ILD at baseline diagnosed by High-Resolution Computed Tomography (HRCT), available baseline and ≥1 annual follow-up pulmonary function tests and no concomitant pulmonary hypertension or airflow obstruction. (bmj.com)
  • In addition to IPF, there are a number of other ILDs that may develop a progressive fibrosing phenotype characterised by declining lung function, an increasing extent of fibrosis on high-resolution computed tomography (HRCT), worsening symptoms and quality of life, and early mortality [ 2 - 5 ]. (ersjournals.com)
Clinical36
  • Objectives To identify the predictive clinical characteristics and establish a prediction model for the progression of mild interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). (bmj.com)
  • Subjects in the two INPULSIS trials had a clinical diagnosis of idiopathic pulmonary fibrosis (IPF) while subjects in the INBUILD trial had a progressive fibrosing ILD other than IPF and met protocol-defined criteria for ILD progression despite management. (ersjournals.com)
  • These findings indicate that patients with fibrosing ILDs other than IPF, who are progressing despite management, have a subsequent clinical course similar to patients with untreated IPF, with a high risk of further ILD progression and early mortality. (ersjournals.com)
  • Methods We performed a retrospective analysis of an IPF cohort (n=128) who had their first clinical visit at the Oxford Interstitial Lung Disease Service between 2013 and 2017. (bmj.com)
  • Recently, the clinical practice guidelines for IPF and this new entity, progressive pulmonary fibrosis, were published . (medscape.com)
  • Alternatively, studies have identified circulating biomarkers that correlate with disease progression, such as Krebs von den Lungen-6 (more commonly known as KL-6) and surfactant protein D, although these are not used in routine clinical practice. (medscape.com)
  • Purpose: Progressive fibrosing interstitial lung disease (PF-ILD) is a phenotype defined by rapid clinical progression towards respiratory failure. (mssm.edu)
  • Few studies have described the value of serial computed tomography (CT) in predicting clinical progression of ILD. (mssm.edu)
  • We explore which single and serial clinical and radiographic variables, in particular serial CT variables and a novel variable, the right lower lobe anterior bronchial angle (RLL-ABA), best predict mortality, oxygen requirement, hospital admissions, and lung transplant in CTD-ILD. (mssm.edu)
  • Conclusions: In addition to clinical and single CT variables, serial CT measurements such as change in extent of fibrosis, PA size, PA to aorta ratio, and RLL-ABA were predictive of mortality and CNO. (mssm.edu)
  • Importantly, this report identifies a number of poorly recognized challenges that exist in pulmonary academic settings, which likely contribute to diminished efficiency of commercialization efforts, ultimately hindering the rate of successful clinical translation. (bvsalud.org)
  • Idiopathic Pulmonary Fibrosis (IPF) is the newest clinical program in the pipeline for Cumberland's first new chemical entity: ifetroban - a potent and selective thromboxane receptor antagonist. (drugdiscoveryonline.com)
  • The FIGHTING FIBROSIS™ clinical trial is a multicenter, double-blind, placebo-controlled Phase II study in patients with IPF. (drugdiscoveryonline.com)
  • Medications, rehabilitation, clinical trials and lung transplant in select cases offer hope for our patients with ILD and PF. (henryford.com)
  • Measurement of force vital capacity is routinely used in clinical practice to assess the functional status of patients with lung disorders. (uw.edu)
  • Annelise Rønnow, president of FESCA (The Federation of European Scleroderma Associations), added: "There are only very few drugs assessed in clinical trials for scleroderma with lung involvement - a devastating reality for people living with the disease. (blogspot.com)
  • May 11, 2023 - SEATTLE - Avalyn Pharma Inc. , a clinical-stage biopharmaceutical company developing inhaled therapies to treat life-threatening pulmonary diseases, today announced that the company will be participating in the upcoming American Thoracic Society (ATS) annual meeting and its associated Respiratory Innovation Summit (RIS), which are being held at the Marriott Marquis in Washington, DC. (avalynpharma.com)
  • Usually acute and chronic diseases will share the same clinical signs, such as coughing and sneezing, but other signs might not become apparent until the disease becomes more chronic and severe, dyspnoea being an example. (vin.com)
  • Obesity and other respiratory diseases can compromise tracheal mechanics in dogs where the trachea lacks structural rigidity and the dorsal membrane is flaccid and wide, and so precipitate clinical signs. (vin.com)
  • The primary objective was to describe the clinical course of IPF over 12 months of follow-up, including changes in lung function measured by % predicted forced vital capacity (FVC% predicted). (biomedcentral.com)
  • UIP is the expected histopathological pattern in patients with clinical idiopathic pulmonary fibrosis (IPF), but the UIP-pattern can be seen in other conditions on occasion. (elsevierpure.com)
  • Because theses entities have different mechanisms of injury, approach to therapy, and expected clinical progression, it is imperative for the surgical pathologist to correctly classify them. (elsevierpure.com)
  • In this manuscript, we review the distinguishing clinical, radiologic and histopathological features of UIP of IPF, RILD and CHrHP, based, in part, on the existing literature, but also lessons learned from a busy lung biopsy consultation practice. (elsevierpure.com)
  • The second has been awarded to a team led by Kamonpun Ussavarungsi, MD, clinical assistant professor in the Division of Pulmonary, Critical Care, and Occupational Medicine. (internalmedicineiowa.org)
  • We intend to use artificial intelligence to develop a clinical model that will predict IPF progression and survival outcomes," Ussavarungsi said. (internalmedicineiowa.org)
  • These encouraging results, prompted us to undertake a phase III 1-yr clinical study to examine the therapeutic effects of pirfenidone on lung functional deterioration and disease progression in patients with IPF. (ersjournals.com)
  • The diagnosis of IPF was in accordance with the American Thoracic Society (ATS)/European Respiratory Society (ERS) Consensus statement 16 and the fourth version of the clinical diagnostic criteria guidelines for idiopathic interstitial pneumonia in Japan 17 . (ersjournals.com)
  • Aim III will determine the therapeutic efficacy and mechanisms of action of Fendrr in pre-clinical pulmonary fibrosis mouse models. (grantome.com)
  • The ability of these medications, either alone or in combination, to reduce the frequency of flare ups (exacerbations) or hospitalizations, or to reduce the rate of decline in lung function is the objective of most recent and upcoming clinical trials. (thelungresearchcenter.com)
  • The Lung Research Center has participated in most of the major clinical trials for the treatment of COPD, including the Torch Trial, The Uplift Trial and the recently completed Summit Trial. (thelungresearchcenter.com)
  • Since, 1998, The Lung Research Center has participated in all of the major clinical trials that sought a treatment for this disease. (thelungresearchcenter.com)
  • Recent clinical trials have focused on testing new therapies for lung diseases and studying patterns of lung disease in different groups of people. (thelungresearchcenter.com)
  • He performs health services and outcomes research focused on understanding and improving the lives and care of patients with advanced lung disease and is actively involved in clinical trials to improve outcomes in interstitial lung disease. (stanford.edu)
  • The study will compare the time to a composite endpoint of relative decline in lung function [10% relative decline in forced vital capacity (FVC), first respiratory hospitalization, lung transplantation, or all-cause mortality] The secondary objectives will be to examine the effect of NAC on the components of the primary composite endpoint, the rates of clinical events, change in physiology, change in health status, and change in respiratory symptoms. (stanford.edu)
  • Secondary objectives will determine the clinical, genetic, epigenetic and biochemical factors that determine the trajectory of recovery or progression of ILD. (cov19longhaulfoundation.org)
  • Observations that favor a viral etiology include a clinical presentation similar to that of chronic myocarditis, findings of myocarditis or myocardial fibrosis in affected patients, a higher incidence following epidemics of coxsackievirus B infection, demonstration of persistent viral infection with molecular studies, and experimental production of the disease in animal models by viral infections of the myocardium. (medscape.com)
ILDs8
  • We used data from the INBUILD and INPULSIS trials to investigate the natural history of progressive fibrosing interstitial lung diseases (ILDs). (ersjournals.com)
  • ILDs cause this interstitial space to become inflamed or scarred making it more difficult for oxygen to get into the bloodstream. (stanford.edu)
  • These three groups of ILDs have slightly different presentations, and different findings on the CT scans, lung tissue biopsy (if done), and bloodwork results. (stanford.edu)
  • ILDs are characterized by scarring, decline in lung function, reduced exercise capacity and quality of life, and are associated with increased mortality. (avalynpharma.com)
  • The interstitial lung diseases (ILDs) are a diverse group of chronic lung conditions characterised by dyspnoea on exertion and poor health related quality of life. (bmj.com)
  • Interstitial lung diseases (ILDs) comprise an heterogenous group of more than 200 pulmonary disorders characterized by lung architectural distortion, with variable amount of fibrotic and/or inflammatory lesions, and associated with significant morbidity and mortality. (pneumon.org)
  • Interstitial lung diseases (ILDs) comprise an heterogenous group of more than 200 lung disorders characterized by variable amount of fibrotic and/or inflammatory lesions 1 . (pneumon.org)
  • Τhe aim of this article is to present developments in the current pharmacological therapies of ILDs and to highlight novel molecular targets and ongoing research trials that may significantly change the prognosis of this group of diseases in the future. (pneumon.org)
Pneumonitis11
  • If you undergo a lung biopsy, the pathologist can confirm a diagnsosi of Hypersensitivity Pneumonitis by findings in the lung tissue. (stanford.edu)
  • Most important among these are the rheumatic interstitial lung diseases (RILD) and chronic hypersensitivity pneumonitis (CHrHP). (elsevierpure.com)
  • Types of Interstitial Lung Disease (ILD) include Interstitial Pneumonia, IPF, Nonspecific Interstitial Pneumonitis (lung disease often present with an autoimmune condition such as Rheumatoid Arthritis or Scleroderma), Hypersensitivity Pneumonitis, Cryptogenic Organizing Pneumonia (COP), Sarcoidosis, and Asbestosis. (thelungresearchcenter.com)
  • Hypersensitivity pneumonitis, also known as extrinsic allergic alveolitis, is an uncommon non-immunoglobulin E (IgE), T-helper cell type 1 (Th1)-mediated inflam- matory pulmonary disease with systemic symptoms resulting from repeated inhalation and subsequent sensitization to a large variety of aerosolized antigenic organic dust particles. (cdc.gov)
  • The earliest forms of hypersensitivity pneumonitis were related to farming and, each year, new antigens causing occupational disease are described. (cdc.gov)
  • Hypersensitivity pneumonitis was originally described in 1713 as an occupational lung disease in grain workers and later, in 1932, in farmers inhaling moldy hay contam- inated with thermophilic actinomyces, hence the term farmer's lung.1 With this recog- nition, modernization of farming methods has resulted in the reduction in farmer's lung prevalence estimated at 0.5% to 3% of exposed farmers in studies spanning from 1980 to 2003. (cdc.gov)
  • National surveillance screening in the United Kingdom from 1992 to 2001 estimated 50 cases of hypersensitivity pneumonitis annually, rep- resenting 1.8% of all cases of work-related respiratory disease seen by chest physi- cians. (cdc.gov)
  • Farmer's lung is the prototype occupational hypersensitivity pneumonitis. (cdc.gov)
  • 9 Agricul- tural exposures were the most common occupation for hypersensitivity pneumonitis in the Czech Republic, with 69% of cases of farmer's lung (cattleman and dairyman), followed by malt workers and chemical workers. (cdc.gov)
  • The exertional dyspnea and bibasilar end-inspiratory rales are suggestive of some type of interstitial pneumonitis. (cdc.gov)
  • The underlying histopathology of IPF is usual interstitial pneumonitis (UIP). (medscape.com)
Diagnosis of idiopathic pulmonar1
Rheumatoid arthritis7
  • In recent years, the number of studies on rheumatoid arthritis-related interstitial lung disease (RA-ILD) has been increasing, which has led to many publications on this topic. (hindawi.com)
  • Rheumatoid arthritis (RA) is a systemic autoimmune disease characterized by articular and extra-articular manifestations affecting approximately 1-2% of the general population [ 1 ]. (hindawi.com)
  • The retrieval steps and strategies were as follows: Title = rheumatoid arthritis AND Title = (interstitial lung disease OR interstitial pneumonia) AND Language = English AND Document type = (review OR article) AND Time span =1980 to 2021. (hindawi.com)
  • Patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD) had slower rates of decline in lung function over 1 year when treated with nintedanib (Ofev), an intracellular inhibitor of tyrosine kinases, according to a post-hoc analysis from a larger trial known as INBUILD. (medpagetoday.com)
  • Our researchers are trying to understand how interstitial lung disease (ILD) develops in people with rheumatoid arthritis (RA), and how it may impact their quality of life. (nationaljewish.org)
  • Background: Inflammation can play a role in diseases like heart disease and rheumatoid arthritis. (policylab.us)
  • Eligibility: People ages 18 and older with rheumatoid arthritis or idiopathic inflammatory myopathy (IIM). (policylab.us)
Mortality12
  • Previous studies explored baseline predictors for lung progression and mortality in SSc-ILD. (bmj.com)
  • Blood monocytes have been linked to all-cause mortality, and neutrophils to progression to IPF in patients with the indeterminate for usual interstitial pneumonia CT pattern. (bmj.com)
  • Objective To determine association between blood monocytes, neutrophils and lymphocytes levels (and their derived indexes), with lung function decline and mortality in IPF. (bmj.com)
  • The second challenge is that acute exacerbations are unpredictable, are associated with inpatient mortality rates of more than 50%, and occur more frequently as idiopathic pulmonary fibrosis progresses. (medscape.com)
  • Cox survival analysis was used to predict two endpoints, all-cause mortality and composite negative outcomes (CNO): new oxygen requirement, respiratory admission, lung transplant, and death. (mssm.edu)
  • Results: On serial CT, change in pulmonary artery (PA) size and RLL-ABA were predictive of mortality and CNO, and change in fibrosis was predictive of mortality alone. (mssm.edu)
  • On single CT, the extent of fibrosis, PA size, and PA to aorta ratio were predictive of mortality and CNO. (mssm.edu)
  • Although worldwide activity has increased year on year over the last decade ( fig. 1 ), demand has also increased meaning the significant shortage of suitable donor lungs accounts for an unacceptably high waiting list mortality rate in many countries. (ersjournals.com)
  • Lung transplantation should generally be considered when a patient's risk of mortality from their lung disease is greater than 50% within the next 2 years. (ersjournals.com)
  • It is a lethal disease that historically has carried a 60-80% 5 year mortality. (thelungresearchcenter.com)
  • Several articles have reported that ILAs are associated with increased mortality, and they can show radiologic progression. (koreamed.org)
  • Morbidity and mortality related to pneumonia and TRACHEOBRONCHITIS in ICU after lung transplantation. (medscape.com)
Abstract2
HRCT4
  • Additionally, patients taking GER medications had a lower fibrosis score on HRCT. (medscape.com)
  • HRCT, which distinguishes airspace from interstitial disease, is the most useful test and is always done. (merckmanuals.com)
  • two out of six expert radiologists independently evaluated the HRCT images to agree and determine whether the pattern of usual interstitial pneumonia (UIP) was present or not in accordance with the predetermined protocol (see online supplementary material). (ersjournals.com)
  • In cases of disagreement, the interpretation of the third radiologist influenced the final decision, and the diagnosis of patients with probable UIP pattern on HRCT was confirmed by the presence of histopathological UIP pattern in surgical lung biopsy samples. (ersjournals.com)
Fibrotic lung disease4
Therapies9
  • These therapies were subsequently allowed after 6 months in patients whose lung or autoimmune disease deteriorated. (medpagetoday.com)
  • The relevance of these findings is demonstrated within the context of TGFB1 signaling and idiopathic pulmonary fibrosis, suggesting therapies against this lethal disease. (nature.com)
  • I do believe, however, that each patient with IPF should be provided with the available data on antifibrotic therapies - and that's because of the profound benefits of lung volume preservation that we see - while we also need to carefully consider the potential adverse events that could occur in the patients in question. (medscape.com)
  • This has been particularly challenging within the pulmonary field, as there have been fewer FDA-approved drugs and higher failure rates for pulmonary therapies as compared to other common disease areas. (bvsalud.org)
  • Researchers want to know if an investigational medication called BBT-877, either alone or in conjunction with other therapies, may help slow lung function decline. (nationaljewish.org)
  • While FDA-approved antifibrotic therapies have shown efficacy to slow progression of the disease, there is no approved treatment which effectively halts disease progression and improves patients' symptoms. (drugdiscoveryonline.com)
  • That means we're up to date on the latest treatments, including studies on new medication therapies for idiopathic pulmonary fibrosis. (henryford.com)
  • By finely balancing the protective effects of immunosuppression against lung allograft rejection with the significant toxic effects of these therapies, excellent long-term survival can be achieved. (ersjournals.com)
  • Current approved therapies only slow IPF disease progression highlighting the need for better understanding of the disease process and the identification of new molecular targets. (lungdiseasesjournal.com)
Pirfenidone7
  • IPF is currently treated with pirfenidone and nintedanib, compounds which slow the rate of disease progression but fail to target underlying pathophysiological mechanisms. (lu.se)
  • Idiopathic pulmonary fibrosis (IPF) currently affects millions of people globally, and current treatments such as pirfenidone or nintedanib are often poorly tolerated in some patients due to frequently associated side effects. (nationaljewish.org)
  • AP01-005 enrolled individuals with idiopathic pulmonary fibrosis (IPF) who had completed the Phase 1b ATLAS study of AP01 (inhaled pirfenidone) , plus two new AP01 naïve cohorts: patients with IPF or non-IPF interstitial lung disease (i.e., progressive pulmonary fibrosis (PPF)) who were intolerant of or ineligible for oral antifibrotic medication. (avalynpharma.com)
  • AP01 is an inhaled aerosol formulation of pirfenidone that is delivered using an optimized eFlow ® Technology Nebulizer, a high efficiency vibrating membrane nebulizer similar in design and operation to PARI's 510(k) cleared/FDA-approved eFlow-based nebulizers marketed with other products for other disease indications. (avalynpharma.com)
  • This administration method allows a smaller pirfenidone dose to be delivered as a soft mist directly to the lung, maximizing pirfenidone's effect on diseased lung tissue while sparing tissue outside of the lungs from the debilitating toxicities associated with oral delivery. (avalynpharma.com)
  • The approval of nintedanib (OFEV) and pirfenidone (Esbriet) has provided two new drugs that slow the progression of the disease. (thelungresearchcenter.com)
  • Genetic variations in idiopathic pulmonary fibrosis and patient response to pirfenidone. (cdc.gov)
Symptoms11
  • Of the 204 included patients, 34% reported symptoms of GER, 45% had a history of GER disease, 47% reported use of medications for GER, and 5% of patients had previously undergone Nissen fundoplication. (medscape.com)
  • The symptoms may also depend upon the underlying disease due to which there is inflammation and scarring in the interstitium. (epainassist.com)
  • Just like the inhaled exposures, it is important for your physician to obtain a detailed medication history during your ILD evaluation, especially as it relates to timing of the medication with changes in your lung symptoms. (stanford.edu)
  • Symptoms and signs of idiopathic pulmonary fibrosis typically develop over 6 months to several years and include dyspnea on exertion and nonproductive cough. (msdmanuals.com)
  • IPF is characterized by irreversible loss of lung function due to fibrosis, which leads to symptoms of increasing cough and dyspnea as well as impaired quality of life. (medscape.com)
  • Medication can result in the relief of symptoms of COPD and reduction in flare ups of the disease. (thelungresearchcenter.com)
  • However, long-term symptoms following COVID-19 are more common in people who suffered more severe acute disease. (cov19longhaulfoundation.org)
  • The patient has no other symptoms of respiratory or cardiac disease. (cdc.gov)
  • The pulmonologist diagnoses asbestosis on the basis of the patient's exposure history, latency of symptoms (occurring 45 years after first exposure), chest radiograph findings, and pulmonary function results. (cdc.gov)
  • Granulomatous diseases cause a great range of symptoms not only in the head and neck but also throughout the body. (medscape.com)
  • Pulmonary symptoms include cough and hemoptysis. (medscape.com)
Unknown etiology2
Biopsy3
Emphysema6
  • Other less common types of lung diseases include emphysema, lung cancer, and pneumonia. (viezec.com)
  • Emphysema is a type of COPD that damages the air sacs in the lungs. (viezec.com)
  • It provides assessment of the potential etiology, extent, and distribution of disease and is more likely to detect underlying or coexisting disease (eg, occult mediastinal adenopathy, cancer, emphysema). (merckmanuals.com)
  • Cigarette smoking together with concomitant emphysema might predispose IPF patients to lung cancer (LC), mostly to non-small cell lung cancer (NSCLC), increasing the risk of cancer development. (encyclopedia.pub)
  • The lung tissue in patients with COPD usually has evidence of emphysema, which is caused by the destruction of the elastic properties of the lung, often by enzymes released from white blood cells that are recruited to the lung by cigarette smoke. (thelungresearchcenter.com)
  • Pulmonary interstitial emphysema in fatal asthma: case report and histopathological review. (medscape.com)
Division of Pulmonary1
  • Several University of Washington researchers participated in this project, including co-lead author, Dr. Sina Gharib, associate professor of medicine in the division of pulmonary and critical care, and Drs. Susan Heckbert, professor of epidemiology, and Bruce Psaty, professor of medicine. (uw.edu)
Bleomycin-induced4
  • In this study we show Ogg1-targeting siRNA mitigates bleomycin-induced pulmonary fibrosis in male mice, highlighting OGG1 as a tractable target in lung fibrosis. (lu.se)
  • In addition, TH5487 decreases levels of pro-inflammatory mediators, inflammatory cell infiltration, and lung remodeling in a murine model of bleomycin-induced pulmonary fibrosis conducted in male C57BL6/J mice. (lu.se)
  • Finally, we have provided evidence that adenovirus-mediated gene transfer of FENDRR into mouse lungs attenuates bleomycin-induced pulmonary fibrosis in preventive and therapeutic modes. (grantome.com)
  • An increase in transforming growth factor α (TGFα) and EGFR in rats with bleomycin-induced lung injury 16 has been observed. (lungdiseasesjournal.com)
Nintedanib6
  • BUSINESS WIRE) -- Boehringer Ingelheim announced today that the U.S. Food and Drug Administration (FDA) has granted Fast Track designation to nintedanib for the treatment of systemic sclerosis with associated interstitial lung disease (SSc-ILD). (blogspot.com)
  • Nintedanib, which is marketed as OFEV®, is approved for a rare lung disease called idiopathic pulmonary fibrosis, or IPF, and has been shown to slow disease progression as measured by annual rate of decline in lung function. (blogspot.com)
  • Because SSc-ILD and IPF share similarities in how the underlying lung scarring, or fibrosis, forms in people with the disease, Boehringer Ingelheim is evaluating the impact of nintedanib on SSc-ILD. (blogspot.com)
  • Drugs used to manage connective tissue disease (CTD) associated with interstitial lung disease (ILD) (CTD-ILD) include nintedanib, corticosteroids, and antineoplastic agents. (medscape.com)
  • Nintedanib is indicated to slow the rate of decline in pulmonary function in patients who have interstitial lung disease (ILD) associated with scleroderma. (medscape.com)
  • Variations in S100A8/A12 Gene Expression Are Associated with the Efficacy of Nintedanib and Acute Exacerbation Development in Idiopathic Pulmonary Fibrosis Patients. (cdc.gov)
Treatment of idiopathic1
Acute7
  • This inflammatory reaction can be described as chronic rather than acute, with a slow ongoing progression of the immune system attempting to eliminate the foreign fibers. (wikipedia.org)
  • In some acute diseases, dyspnoea might be the predominant sign noted. (vin.com)
  • In particular, it has been postulated that the "cytokine storm" caused by the exaggerated inflammatory response following SARS-CoV-2 infection, as well as micro-thrombotic hypotheses, may predispose patients with COVID-19 pneumonia to aberrant mechanisms of repair and fibrosis [ 15 ] culminating in acute lung injury and interstitial lung disease [ 16 ] . (encyclopedia.pub)
  • Acute interstitial pneumonia. (promeposec.com)
  • SARS-CoV-2 leads to pronounced inflammation within the lung and leads to the development of acute respiratory distress syndrome in a substantial proportion of those infected. (cov19longhaulfoundation.org)
  • A quality control worker in a feed factory developed acute disease after taking samples of cattle feed treated with phytase, a fungal-derived enzyme used to treat cattle feed to strengthen bone. (cdc.gov)
  • It is thought to begin with acute injury to the pulmonary parenchyma, leading to chronic interstitial inflammation, then to fibroblast activation and proliferation, and finally progressing to the common endpoint of pulmonary fibrosis and tissue destruction. (medscape.com)
Tomography1
  • Any evidence of Stage IV disease If pleural effusion is present the following criteria must be met to exclude malignant involvement (T4 disease): When pleural fluid is visible on both the computed tomography scan and chest X-ray, a pleuracentesis is required to confirm that the pleural fluid is cytologically negative. (who.int)
Parenchyma1
  • Pulmonary fibrosis in surgical lung biopsies is said to have a 'usual interstitialpneumonia-pattern' (UIP-pattern) of disease when scarring of the parenchyma is present in a patchy, 'temporally heterogeneous' distribution. (elsevierpure.com)
Transplantation15
  • Two main challenges in the process of lung transplantation evaluation are to be considered. (medscape.com)
  • The first challenge is the timing of referral to the transplantation program, as antifibrotic medications attenuate disease progression and make the decision on referral timing more complex as physicians elect to wait longer for those patients with moderate disease, pending a treatment response. (medscape.com)
  • The inability to accurately predict an event means that future exacerbations cannot be considered when referring for lung transplantation. (medscape.com)
  • Henry Ford provides lung transplantation for some people with the most advanced ILD. (henryford.com)
  • Summary Over the last 25 years, lung transplantation has developed into a well-established treatment option for selected patients with very advanced lung disease. (ersjournals.com)
  • In 2010, over a 12-month period, more than 3500 new lung transplant procedures were reported to the International Society of Heart and Lung Transplantation (ISHLT) Registry [ 1 ]. (ersjournals.com)
  • Furthermore, a meticulous approach to post-transplant management in the immediate post-operative period, in the early and long-term has contributed to continually improving long-term survival after lung transplantation. (ersjournals.com)
  • To outline recent advances made in improving the success of lung transplantation as a therapy for very advanced lung disease. (ersjournals.com)
  • To discuss where the major developments in lung transplantation may come over the next 5-10 years. (ersjournals.com)
  • Lung transplantation should be viewed as a potential treatment option for highly selected patients with very advanced chronic lung disease who continue to progress despite maximal medical therapy. (ersjournals.com)
  • Lung Transplantation is by no means a cure and should be thought of as another modality of treatment. (ersjournals.com)
  • Life after lung transplantation necessitates use of a large number of medications on a daily basis, frequent hospital visits and the risk of a large list of potential adverse effects due to use of powerful immunosuppression. (ersjournals.com)
  • Lung transplantation is performed provided there are no other changes in the body. (promeposec.com)
  • He is the Associate Medical Director for Lung and Heart/Lung Transplantation at Stanford and director of the Pulmonary Fibrosis Foundation Care Center at Stanford. (stanford.edu)
  • Impact of age and telomere length on circulating T cells and rejection risk after lung transplantation for idiopathic pulmonary fibrosis. (cdc.gov)
Include idiopathic1
Survival8
  • Elevated CXCL6 levels in the BAL of two cohorts of patients with IPF were associated with poor survival (Hazard ratio of death or progression=1.89 (95%CI 1.16-3.08, N=179, p=0.01). (bvsalud.org)
  • In experienced centres, specific patient groups, such as those with cystic fibrosis (CF), can expect a median survival of well over 10 years with some recipients functioning well more than 20 years after their transplant. (ersjournals.com)
  • When successful, the procedure is also associated with a marked improvement in quality of life and a proven survival advantage in patients with CF, idiopathic pulmonary fibrosis and pulmonary hypertension. (ersjournals.com)
  • Secondary end-points included the progression-free survival (PFS) time. (ersjournals.com)
  • Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease (ILD) of unknown aetiology, with a median survival of 2-4 years from the time of diagnosis. (encyclopedia.pub)
  • Among them, idiopathic pulmonary fibrosis (IPF) is a rare lung disease with an unknown cause and a median survival of 2-4 years from the time of diagnosis. (encyclopedia.pub)
  • We are advancing the understanding of lung disease and innovative treatments that improve quality-of-life and survival for patients. (thelungresearchcenter.com)
  • N-acetylcysteine exposure is associated with improved survival in anti-nuclear antibody seropositive patients with usual interstitial pneumonia. (medscape.com)
Contributes to pulmonary fibrosis1
  • The central hypothesis of this project is that the down-regulation of FENDRR in IPF contributes to pulmonary fibrosis by promoting fibroblast activation via increased expression and activity of pro-fibrotic miR-214 due to iron overload and a reduced competition between FENDRR and miR-214, respectively. (grantome.com)
Progressive14
Hypertension7
  • The development of pulmonary hypertension in idiopathic pulmonary fibrosis is an independent marker of poor prognosis and should prompt consideration for referral. (medscape.com)
  • Pulmonary Hypertension Pulmonary hypertension is increased pressure in the pulmonary circulation. (msdmanuals.com)
  • In pulmonary hypertension, pulmonary vessels may become constricted. (msdmanuals.com)
  • Cor Pulmonale Cor pulmonale is right ventricular (RV) enlargement secondary to a lung disorder that causes pulmonary artery hypertension. (msdmanuals.com)
  • 12 - 14 Pulmonary arterial hypertension is also common 15 and may worsen with exercise. (bmj.com)
  • Eplerenone attenuates pathological pulmonary vascular rather than right ventricular remodeling in pulmonary arterial hypertension. (medscape.com)
  • Study protocol for a randomised controlled trial of exercise training in pulmonary hypertension (ExTra_PH). (medscape.com)
Systemic sclerosis5
  • Researchers are studying a new medication called PRA023 that has anti-inflammatory and antifibrotic properties to determine how well it works against systemic sclerosis (SSc) with interstitial lung disease (ILD). (nationaljewish.org)
  • This Fast Track designation is an encouraging step in our ongoing research and commitment to advancing care of those with systemic sclerosis with interstitial lung disease," said Christopher Corsico, M.D., Chief Medical Officer, Boehringer Ingelheim. (blogspot.com)
  • Systemic sclerosis, also known as scleroderma, is a rare disease characterized by the thickening and scarring of connective tissue of multiple organs in the body, typically affecting women between ages 25 and 55. (blogspot.com)
  • Most people with the disease will develop some degree of lung scarring, or interstitial lung disease (ILD), which is the leading cause of death among people with systemic sclerosis. (blogspot.com)
  • Angiotensin-converting enzyme 1 gene polymorphisms in patients with systemic sclerosis-associated interstitial lung disease: a single centre retrospective observational study. (cdc.gov)
Innate immunity1
  • My early work helped ignite interest in role of innate immunity in lung injury, repair, and remodeling. (yale.edu)
Disorders7
  • Editorial on the Research Topic Inflammation and aging in chronic and degenerative diseases: Current and future therapeutic strategies Inflammation and aging represent the most common risk factors for several chronic and degenerative disorders (Furman et al. (researchgate.net)
  • 2021). Of note, inflammation and aging are both pathophysiological processes that have been associated with an increased risk of different chronic-degenerative diseases, including tumors, neurological and cardiovascular disorders (Gupta et al. (researchgate.net)
  • Interstitial lung disease (ILD) is a large group of disorders that can affect the small air sacs (alveoli) or surrounding structures (interstitium) of the lung. (henryford.com)
  • ILD refers to a category of lung disorders that involve the interstitium, the lacelike support structures of the lung. (henryford.com)
  • Interstitial Lung Disease refers to a large group of lung disorders that cause inflammation or scarring in the lung tissue. (stanford.edu)
  • Overview of Interstitial Lung Disease Interstitial lung diseases are a heterogeneous group of disorders characterized by alveolar septal thickening, fibroblast proliferation, collagen deposition, and, if the process remains unchecked. (merckmanuals.com)
  • Diffuse parenchymal lung diseases (DPLDs) comprise a heterogenous group of disorders. (medscape.com)
Type of interstitial1
Medication4
  • Though the medication for interstitial lung disease will work to manage the disease, there are certain other non-medication methods through which the disease can be managed. (epainassist.com)
  • Participants may receive medical evaluations and procedures, study medication, disease-related education and possible financial compensation for time, travel and participation. (nationaljewish.org)
  • Avalyn is developing a pipeline of inhaled therapeutics designed to reduce systemic exposure and deliver medication to the site of disease. (avalynpharma.com)
  • Usually caused by cigarette smoking, the disease can be slowed or stopped by smoking cessation and can be treated with medication. (thelungresearchcenter.com)
Histologically2
  • Although similar histologically, granulomatous diseases of the head and the neck require a thorough approach because of the wide variety of disease processes. (medscape.com)
  • Pathologically, all DPLDs manifest histologically with disease largely within the interstitial compartment of the lung. (medscape.com)
Pattern of usual interstit1
  • Although the diagnosis of IPF can be extremely challenging due to the heterogenous nature of this disease [ 4 ] , it is recognized by clinicopathological criteria, including the radiographic and histological hallmarks pattern of usual interstitial pneumonia (UIP) [ 5 ] . (encyclopedia.pub)
Epithelial cells3
Pathology2
  • Interstitial pneumonia in children is often combined with congenital pathology, such as reirofibromatosis and Marfan syndrome. (promeposec.com)
  • These changes can be secondary in nature and determined against the background of any other interstitial pathology. (promeposec.com)
Tissue12
  • People who live near mines can also be exposed to airborne asbestos fibers Asbestosis is the scarring of lung tissue (beginning around terminal bronchioles and alveolar ducts and extending into the alveolar walls) resulting from the inhalation of asbestos fibers. (wikipedia.org)
  • Due to the asbestos fibers' natural resistance to digestion, some macrophages are killed and others release inflammatory chemical signals, attracting further lung macrophages and fibrolastic cells that synthesize fibrous scar tissue, which eventually becomes diffuse and can progress in heavily exposed individuals. (wikipedia.org)
  • Patients could be taking stable doses of medications for RA or connective tissue disease at baseline, except azathioprine, cyclosporine, mycophenolate mofetil (Cellcept), tacrolimus, rituximab (Rituxan), cyclophosphamide, or oral glucocorticoids in dosages above 20 mg/day. (medpagetoday.com)
  • 2021). Besides these well-known pathogenetic mechanisms related to inflammaging, other processes are involved in age-related and inflammatory-related diseases including enzyme dysfunctions, cell death, impaired tissue renewal and tissue degeneration (Li, 2013). (researchgate.net)
  • While idiopathic pulmonary fibrosis is the archetype of PF-ILD, connective tissue disease associated interstitial lung disease (CTD-ILD) can also manifest as PF-ILD. (mssm.edu)
  • OGG1 and SMAD7 interact to induce fibroblast proliferation and differentiation and display roles in fibrotic murine and IPF patient lung tissue. (lu.se)
  • In the ILD Multidisciplinary Conference, our pulmonologists (lung specialists) review each patient's case with radiologists (experts in imaging) and pathologists (doctors who study tissue in the lab). (henryford.com)
  • The interstitium refers to the tissue area in and around the wall of the airsacs (alveoli) of the lung area where oxygen moves from the alveoli into the capillary network (small blood vessels) that covers the lung like a thin sheet of blood. (stanford.edu)
  • The changes in the lung tissue can also cause a dry, hacky cough for some patients. (stanford.edu)
  • The key histologic findings of idiopathic pulmonary fibrosis are subpleural fibrosis with sites of fibroblast proliferation (fibroblast foci) and dense scarring, alternating with areas of normal lung tissue (heterogeneity). (msdmanuals.com)
  • lung tissue. (gmu.edu)
  • Importantly, these mediators can be detected in the tissue of patients with various forms of pulmonary fibrosis, cementing the association with human disease. (yale.edu)
Bronchiolitis1
  • The exaggerated immune response to repeated inhalation of these particles leads to infiltration and proliferation of activated pulmonary macrophages and lymphocytes, resulting in lymphocytic alveolitis and bronchiolitis with noncaseating granulomas. (cdc.gov)
Diffuse3
  • Diffuse cutaneous subset, presence of antitopoisomerase-I antibodies, decreased baseline forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) were reported to be significantly associated with lung progression in patients with SSc, while positive anticentromere antibody (ACA) was protective. (bmj.com)
  • The radiograph is characterized by diffuse bilateral darkening of the lung fields. (promeposec.com)
  • As a group, diffuse interstitial diseases of the lung are uncommon. (medscape.com)
Pathogenesis of pulmonary2
  • Recent data indicate that macrophages play essential pro-fibrotic roles in the pathogenesis of pulmonary fibrosis. (novartis.com)
  • Although small non-coding RNAs, such as microRNAs, have been extensively studied in IPF, very little is known regarding the roles of lncRNAs in the pathogenesis of pulmonary diseases, such as IPF. (grantome.com)
Heterogeneous group1
  • 16 In addition, ILD is a heterogeneous group of diseases. (bmj.com)
Cystic5
  • Cystic abnormality (honeycombing) occurs in all patients and increases with advanced disease. (msdmanuals.com)
  • For example, cystic fibrosis is a condition that results in the production of abnormally thick mucus in the lungs, which can lead to infections and other problems. (viezec.com)
  • Serum (1→3)-β-D-glucan and galactomannan levels in patients with cystic fibrosis: a retrospective cohort study. (medscape.com)
  • Implementation of a successful eradication protocol for Burkholderia Cepacia complex in cystic fibrosis patients. (medscape.com)
  • Effects of a partially supervised conditioning programme in cystic fibrosis: an international multi-centre randomised controlled trial (ACTIVATE-CF): study protocol. (medscape.com)
Fibroblast activation3
  • The objective of the current application is to understand how long non-coding RNAs (lncRNAs) regulate pulmonary fibroblast activation in IPF. (grantome.com)
  • Our preliminary studies have shown that TGF?1 and hypoxia reduce FENDRR expression in pulmonary fibroblasts and that the overexpression of FENDRR inhibits fibroblast activation. (grantome.com)
  • The cumulative damage to the pulmonary epithelium results in epithelial cell apoptosis, fibroblast activation and transforming growth factor-beta (TGF-β)-mediated differentiation to myofibroblasts and extracellular matrix remodeling. (pneumon.org)
Respiratory diseases3
  • A very interesting and informative panel discussion on Pulmonary Rehabilitation (PR) of patients of respiratory diseases was held as part of the 20th National Conference on Environmental Sciences and Pulmonary Diseases (20th NESCON), organized by the Academy of Respiratory Medicine, under the auspices of Environmental Medical Association in Mumbai. (theseoultimes.com)
  • Pulmonary rehabilitation is an evidence based multidisciplinary and comprehensive intervention for patients with chronic respiratory diseases who are symptomatic. (theseoultimes.com)
  • Avalyn is a biopharmaceutical company developing targeted therapeutics for the treatment of rare respiratory diseases including idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILD). (avalynpharma.com)
Medscape3
Usual interstitial3
  • Die Diagnosesicherung erfordert den Nachweis des Befundmusters einer usual interstitial pneumonia (UIP) im hochaufl senden D nnschicht-Computertomogramm (HR-CT) oder histologisch durch eine Lungenbiopsie sowie den Ausschluss anderer Ursachen, wie Asbestose oder Kollagenosen. (aerzteblatt.de)
  • Usual interstitial pneumonia-pattern fibrosis in surgical lung biopsies. (elsevierpure.com)
  • Dive into the research topics of 'Usual interstitial pneumonia-pattern fibrosis in surgical lung biopsies. (elsevierpure.com)