Underlying autoimmuneUsual interstitInflammationFibrosisNonspecific interstitial pneumoniaDiagnosisDisordersBiopsyPneumonia with autoimmune featuresCause of interstitial lung diRheumatoidForm of interstitial lung diCauses of interstitial lung diAlveolarILDsParenchymal lungPneumoniasFibroticPatientsAutoimmune DiseasesCarbon monoxidePathogenesisClinicalChronicDiffuseEmphysemaInflammatoryPresence of autoantibodiesNSIPPathophysiologyHypersensitivity pneumonitisComplicationsAirwayConnective TissueSevereLesionsAcuteRestrictiveRespiratory failureDiagnosticArteriesChestDrug-inducedPneumonitisHRCTExercise intolerance
Underlying autoimmune2
- [ 9 ] To address this, the European Respiratory Society and American Thoracic Society released a joint consensus statement in 2015 proposing the term "interstitial pneumonia with autoimmune features" (IPAF) be used for "idiopathic interstitial pneumonia" (IIP) with clinical features that suggest an underlying autoimmune process. (medscape.com)
- In 2015, the European Respiratory Society and American Thoracic Society released a joint consensus statement proposing the term interstitial pneumonia with autoimmune features (IPAF) be used for idiopathic interstitial pneumonia (IIP) with clinical features that suggest an underlying autoimmune process. (medscape.com)
Usual interstit6
- ILD associated with CTDs (CTD-ILD) may display a variety of histologic subtypes, including usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), diffuse alveolar damage, lymphoid interstitial pneumonia (LIP), bronchiectasis, constrictive bronchiolitis, follicular bronchiolitis, and alveolar hemorrhage. (medscape.com)
- Compared with the control group, patients with UCTD-ILD were significantly more likely to have ground-glass opacity on high-resolution computed tomography (HRCT) and NSIP pattern on biopsy, and less likely to have honeycombing on HRCT or usual interstitial pneumonia on biopsy. (nih.gov)
- determine if findings indicate a pattern of (1) usual interstitial pneumonia (UIP), (2) probable UIP, (3) indeterminate of UIP, or (4) alternate diagnosis. (medscape.com)
- A non-usual interstitial pneumonia pattern was associated with improved survival, as was the presence of the clinical domain. (medscape.com)
- In this setting, IPF is defined as a chronic progressive fibrotic ILD, with unknown aetiology and a pattern of usual interstitial pneumonia (UIP), which is represented by heterogeneous lesions at different stages of evolution with foci of proliferative fibroblasts. (ersjournals.com)
- Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. (medicalmarijuana.com)
Inflammation18
- Although the distinct cause of interstitial lung disease (ILD) in connective disease tissue (CTD) is not known, the underlying pathogenesis is thought to be secondary to varying degrees inflammation, alveolar injury, dysregulated tissue repair, and fibroproliferation. (medscape.com)
- In the early stages of lung injury, the degree of inflammation of interstitial and alveolar spaces is associated with subsequent fibrosis. (medscape.com)
- We studied 28 consecutive patients with idiopathic interstitial pneumonia (IIP) enrolled in the University of California, San Francisco Interstitial Lung Disease Center who met prespecified criteria for UCTD, as follows: at least one clinical manifestation of connective tissue disease, serologic evidence of systemic inflammation in the absence of clinical infection, and absence of sufficient American College of Rheumatology criteria for another connective tissue disease. (nih.gov)
- Institute of Immunology and Inflammation, Frontiers Science Center for Disease-related Molecular Network, West China Hospital, Chengdu, China. (bvsalud.org)
- Interstitial lung disease (ILD) encompasses a heterogeneous group of respiratory disorders characterised by inflammation and/or fibrosis of the lung interstitium. (springer.com)
- Interstitial lung disease (ILD) is a broad category of lung diseases that includes more than 130 disorders characterized by scarring (i.e. "fibrosis") and / or inflammation of the lungs. (nationaljewish.org)
- Combined collagen-induced arthritis and organic dust-induced airway inflammation to model inflammatory lung disease in rheumatoid arthritis. (cdc.gov)
- ILD involves scarring or inflammation of lung tissues that makes it difficult to breathe and provide oxygen to your bloodstream. (nortonhealthcare.com)
- Interstitial lung disease involves inflammation of the lung's air sacs and airways and scarring of tissue that causes the lungs to become stiff, small, and less effective at transferring oxygen into the blood and carbon dioxide out from the blood. (cdc.gov)
- 8 Damage subsequently results from autoimmunity-induced inflammation or tissue deposition of immune complexes. (lww.com)
- The diseases cause inflammation or scarring of the lung tissue (interstitial lung disease) or result in filling of the air spaces with exudate and debris (pneumonitis). (medscape.com)
- Interstitial lung disease is a term used to refer to a particular type of inflammation of the interstitium of the lungs. (medicalmarijuana.com)
- Interstitial pneumonia (medical condition): A category of chronic lung diseases characterized by scarring and/or inflammation of the lungs. (medicalmarijuana.com)
- Pneumonia" indicates "lung abnormality", which includes fibrosis and inflammation. (medicalmarijuana.com)
- The primary features of the lung injury includes inflammation , fibrosis , and granulomas development. (wikidoc.org)
- Although their impact on humans is still relatively unknown, occupational studies indicate that workers exposed to very high levels of plastic dust suffer chronic inflammation of the airway, in some (worst) cases interstitial lung disease and tissue scarring. (kcl.ac.uk)
- It is part of many different lung conditions that can cause inflammation in the air sacs of the lungs, as well as in lung tissue and the air space between the sacs. (medicalnewstoday.com)
- These tests show whether there is inflammation or damage in the lung tissue. (medicalnewstoday.com)
Fibrosis40
- Following the initial insult, further activation of fibroblasts and myofibroblasts leads to lung fibrosis and scarring. (medscape.com)
- Furthermore, these results show that the clinical entity idiopathic NSIP is different from idiopathic pulmonary fibrosis and appears to be an autoimmune disease. (nih.gov)
- Idiopathic pulmonary fibrosis (31%) and connective-tissue disease related ILD (21.7%) were the two most common subtypes. (springer.com)
- Firstly, there are the Idiopathic Interstitial Pneumonias (IIPs) including Idiopathic Pulmonary Fibrosis (IPF), the most common IIP, along with idiopathic non-specific idiopathic pneumonia (iNSIP), acute interstitial pneumonia (AIP) and respiratory bronchiolitis-associated ILD (RB-ILD), to name a few. (springer.com)
- Interstitial lung diseases cause scar tissue to form in the lungs (fibrosis). (clevelandclinic.org)
- Pulmonary fibrosis is a severe condition in interstitial lung diseases (ILD) such as idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-ILD, where the underlying mechanism is not well defined and with no curative treatments available. (lu.se)
- 2B receptor as a target in lung fibrosis, warranting further consideration in targeting fibrotic ILDs. (lu.se)
- Idiopathic pulmonary fibrosis (IPF) is, by definition, a progressive fibrosing interstitial lung disease (ILD) [ 1 ]. (ersjournals.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)
- I have a significant cohort of patients with interstitial lung disease, including sarcoidosis, connective tissue disease related lung disease, hypersensitivity pneumonitis, and idiopathic pulmonary fibrosis. (imperial.ac.uk)
- Idiopathic pulmonary fibrosis (IPF) represents probably the most aggressive form of ILD and systemic sclerosis is a multiorgan fibrotic disease frequently associated with ILD. (ersjournals.com)
- The lung is susceptible to various forms of short- and long-term injuries, both airborne and blood-borne, that may result in fibrosis. (ersjournals.com)
- Some forms of fibrosis, such as acute lung injury or cryptogenic organising pneumonia, are at least partially reversible, whereas others, in particular idiopathic pulmonary fibrosis (IPF), are progressive and fatal. (ersjournals.com)
- In systemic sclerosis (SSc) patients, the change in the trend of scleroderma-related deaths over the past 30 years demonstrates that lung involvement (both pulmonary hypertension and pulmonary fibrosis) is among the primary causes of mortality [ 6 ]. (ersjournals.com)
- In SSc, pulmonary disease can exist as a heterogeneous combination of pulmonary fibrosis and pulmonary arterial hypertension (PAH), but the pathogenic mechanisms that induce different pulmonary responses are poorly understood. (ersjournals.com)
- This schematic view of the morpho-functional unit of the lung (alveolus) depicts the main differences in cellular composition in idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) compared with normal physiological cellular components. (ersjournals.com)
- Drs Jeffrey Swigris and Ayodeji Adegunsoye discuss fibrosing interstitial lung disease and treatment challenges for individuals with idiopathic pulmonary fibrosis. (medscape.com)
- Episode 1: Connective Tissue Disease-Interstitial Lung Disease Drs Jeffrey Swigris and Sonye Danoff discuss connective tissue disease-related interstitial lung disease (CTD-ILD) to provide context for understanding idiopathic pulmonary fibrosis. (medscape.com)
- ILD Highlights From ATS 2022 Key abstracts on interstitial lung disease from ATS 2022 include results on a phosphodiesterase 4B inhibitor for idiopathic pulmonary fibrosis and data showing the effect of antifibrotic drugs on mortality. (medscape.com)
- For example, idiopathic pulmonary fibrosis (IPF) is a clinical term describing a slowly progressive, chronic interstitial pneumonia. (medicalmarijuana.com)
- The scarring (fibrosis) involves the supporting framework (interstitium) of the lung. (medicalmarijuana.com)
- The term "usual" refers to the fact that UIP is the most common form of interstitial fibrosis. (medicalmarijuana.com)
- Pulmonary fibrosis share the pathogenesis process of interstitial lung disease which involve the pulmonary parenchyma . (wikidoc.org)
- Over time, rheumatoid arthritis (RA) can lead to lung tissue damage, which can result in a condition doctors call pulmonary fibrosis. (medicalnewstoday.com)
- It can also affect other organs, such as the lungs, causing severe breathing problems that health experts call pulmonary fibrosis. (medicalnewstoday.com)
- Pulmonary fibrosis is a type of interstitial lung disease . (medicalnewstoday.com)
- Typically, pulmonary fibrosis and related lung problems develop in people who have lived with RA for several years. (medicalnewstoday.com)
- The most well studied and most devastating ILD is Idiopathic Pulmonary Fibrosis (IPF), with other well studied ILDs being chronic hypersensitivity pneumonitis (CHP), Non-specific Interstitial pneumonia (NSIP) and Sarcoidosis-ILD. (ersnet.org)
- ILD dominates the morbidity and mortality in all these individual categories and while in some cases the disease can be self-limited or controlled by immunosuppression and antigen removal in the case of CHP, in other cases the fibrosis can take its own course, become self-sustained, progressive and fatal. (ersnet.org)
- Interstitial lung diseases such as idiopathic pulmonary fibrosis (IPF) are caused by persistent micro-injuries to alveolar epithelial tissues accompanied by aberrant repair processes. (lu.se)
- 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)
- The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study. (cdc.gov)
- State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the 'iMAging managEment of cySTic fibROsis' (MAESTRO) consortium. (cdc.gov)
- Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study. (cdc.gov)
- Seasonal variation of lung function in cystic fibrosis: longitudinal modeling to compare a Midwest US cohort to international populations. (cdc.gov)
- Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease with the Phe508del/minimal function genotype. (cdc.gov)
- Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease? (cdc.gov)
- Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis. (cdc.gov)
- Family History of Pulmonary Fibrosis Predicts Worse Survival in Patients with Interstitial Lung Disease. (cdc.gov)
Nonspecific interstitial pneumonia3
- Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? (nih.gov)
- The American Thoracic Society/European Respiratory Society International Consensus Classification panel identified the clinical entity idiopathic nonspecific interstitial pneumonia (NSIP) as a provisional diagnosis and recommended further study. (nih.gov)
- Although SSc-associated ILD (SSc-ILD) is associated more frequently with a nonspecific interstitial pneumonia pattern, which is characterised by homogeneous lesions with predominant inflammatory infiltrates, some aspects of IPF and SSc-ILD suggest that these diseases could share some common features [ 7 ]. (ersjournals.com)
Diagnosis11
- Our division provides state-of-the-art diagnosis and treatment of diseases affecting the joints and connective tissue (rheumatic diseases), including more than 100 types of arthritis and many autoimmune diseases, ranging in complexity and severity, and requiring the treatment and expertise of trained and experienced rheumatologists. (uab.edu)
- When breathing troubles occur, accurate diagnosis and prompt treatment can help restore lung health. (clevelandclinic.org)
- Expert pulmonologists at Cleveland Clinic Florida offer prompt diagnosis of all pulmonary diseases. (clevelandclinic.org)
- More than two dozen board-certified and fellowship-trained physicians with extensive experience specialize in diagnosis and management of all forms of lung diseases and sleep disorders, so you can get the care you need. (nortonhealthcare.com)
- Our providers will give you a precise diagnosis and develop a customized treatment plan for your lung condition, so you can get back to living your best life. (nortonhealthcare.com)
- The results of a multiorgan workup performed at the time of diagnosis were predictive of which patients in this cohort manifested multisystem disease. (thedoctorsdoctor.com)
- Many of the published reports have focused on the role of BLB in the diagnosis of diffuse pulmonary diseases and the accuracy of histological diagnosis. (ersjournals.com)
- BLB was considered unhelpful in patients who had a specific diagnosis from other procedures or biopsies, or those in whom the procedure failed to obtain lung tissue. (ersjournals.com)
- The examination of surgical lung specimens and an integrated clinico-radiological evaluation allow the start of a complex differential diagnosis and prediction of survival, through the identification of different interstitial patterns [ 2 ]. (ersjournals.com)
- Possible diagnosis requires 3 major criteria without serologic evidence of disease or, if anti-U1 RNP is greater than 1:100, 2 major criteria or 1 major and 3 minor criteria. (medscape.com)
- We develop and implement magnetic resonance imaging (MRI) methods for evaluation and diagnosis of musculoskeletal disease. (lu.se)
Disorders15
- Similarly, interstitial lung diseases (ILDs) are a heterogeneous group of lung disorders-characterized by parenchymal lung injury as a consequence of systemic autoimmunity-classified on the basis of their radiologic as well as histopathologic features. (medscape.com)
- The interstitial lung diseases (ILD) are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. (jrheum.org)
- World-renowned specialists in Cleveland Clinic Florida's Department of Pulmonary Medicine, Critical Care and Sleep Medicine treat patients with disorders relating to the lungs, breathing and sleep. (clevelandclinic.org)
- Vascular pulmonary conditions are disorders that affect the blood vessels in your lungs and how blood travels between your heart and lungs. (clevelandclinic.org)
- Interstitial lung disease refers to multiple disorders that can inflame and scar lung tissue. (nortonhealthcare.com)
- 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)
- Some ILDs may present with specific neuromuscular disorders ( e.g. in sarcoidosis or connective tissue diseases) or heart involvement ( e.g. in sarcoidosis or systemic sclerosis). (ersjournals.com)
- The interstitial lung diseases (ILDs) are a group of diffuse parenchymal lung disorders that are classified according to specific clinical, radiological and histopathological features. (ersjournals.com)
- Lung fibrotic disorders are characterised by accumulation of fibroblasts, myofibroblasts and extracellular matrix (ECM), leading to chronic respiratory failure. (ersjournals.com)
- If caused by parenchymal lung disease, restrictive lung disorders are accompanied by reduced gas transfer, which may be marked clinically by desaturation after exercise. (medscape.com)
- The many disorders that cause reduction or restriction of lung volumes may be divided into two groups based on anatomical structures. (medscape.com)
- The second is extrinsic disorders or extrapulmonary diseases. (medscape.com)
- Diseases of these structures result in lung restriction, impaired ventilatory function, and respiratory failure (eg, nonmuscular diseases of the chest wall, neuromuscular disorders). (medscape.com)
- In cases of intrinsic lung disease, the physiological effects of diffuse parenchymal disorders reduce all lung volumes by the excessive elastic recoil of the lungs, relative to the outward recoil forces of the chest wall. (medscape.com)
- Interstitial lung disease is a group of disorders that involve pulmonary parenchyma . (wikidoc.org)
Biopsy16
- Patients with CTD who develop drug-induced ILD may also manifest a variety of histologic patterns in lung biopsy specimens. (medscape.com)
- evidence is insufficient to recommend for or against transbronchial lung biopsy or lung cryobiopsy. (medscape.com)
- guidelines strongly recommend against transbronchial lung biopsy or lung cryobiopsy in this situation. (medscape.com)
- The morphology domain is divided into three sections: interstitial pneumonia patterns suggested by HRCT imaging, histopathologic features identified by surgical lung biopsy, or evidence of additional thoracic compartment involvement as determined by diagnostic imaging, histopathologic findings, right heart catheterization (RHC) or pulmonary function testing. (medscape.com)
- A lung biopsy may be the only way to diagnose the disease if the chest CT does not show the characteristic findings. (wikipedia.org)
- In a biopsy, a small portion of lung tissue is removed to determine if lung disease is present. (wikipedia.org)
- A study from a large referral center identified 19 cases (14 percent) from among 138 lung biopsy cases accrued over a 10-year period. (wikipedia.org)
- NEW YORK (Reuters Health)-Transbronchial lung cryobiopsy (TBLC) and surgical lung biopsy (SLB) had a high level of agreement for diagnosing interstitial lung disease in the first comparative study. (the-rheumatologist.org)
- of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic patterns on lung biopsy. (merckmanuals.com)
- Is bronchoscopic lung biopsy helpful in the management of patients with diffuse lung disease? (ersjournals.com)
- Few studies have evaluated how the results of bronchoscopic lung biopsy (BLB) affect clinical management. (ersjournals.com)
- Patients whose biopsy was of a lung mass or single dominant lung nodule were excluded. (ersjournals.com)
- In conclusion, bronchoscopic lung biopsy is a clinically useful test in ∼75% of procedures. (ersjournals.com)
- Several studies have reported the diagnostic yield and complications of bronchoscopic lung biopsy (BLB) in various patient populations 4 , 5 . (ersjournals.com)
- BLB was defined as a biopsy performed via bronchoscopy with the intention of obtaining adequate parenchymal lung tissue for meaningful histological analysis. (ersjournals.com)
- By this definition, attempts to biopsy a lung mass, solitary nodule or lung cavity were excluded. (ersjournals.com)
Pneumonia with autoimmune features1
- To highlight the unknowns around unclassifiable ILD and to allow further research into the condition, a research group established a new diagnostic entity termed interstitial pneumonia with autoimmune features (IPAF). (ersnet.org)
Cause of interstitial lung di1
- Exposure to hazardous materials over a long period of time can be a cause of interstitial lung disease. (nortonhealthcare.com)
Rheumatoid7
- A recent systematic review found no clear evidence that rheumatoid arthritis patients treated with cyclophosphamide have better lung function than those treated with mycophenolate mofetil. (the-rheumatologist.org)
- A variety of rheumatic diseases can affect the lungs, including systemic sclerosis, rheumatoid arthritis, vasculitis, lupus, polymyositis/dermatomyositis (PM/DM) and Sjögren's syndrome. (the-rheumatologist.org)
- Rheumatoid arthritis (RA) is characterized by extra-articular involvement including lung disease, yet the mechanisms linking the two conditions are poorly understood. (cdc.gov)
- Long-term exposure to asbestos and autoimmune diseases like rheumatoid arthritis are known causes of ILD. (nortonhealthcare.com)
- rheumatoid arthritis (RA), systemic sclerosis (SSc), mixed connective tissue disease (MCTD), Sjögren's disease (SjD), and idiopathic inflammatory myopathies (IIM). (medscape.com)
- How does rheumatoid arthritis affect the lungs? (medicalnewstoday.com)
- The vast majority of the ILDs are categorized in some connective tissue disease associated ILD (such as Rheumatoid arthritis, Scleroderma, Sjogren's). (ersnet.org)
Form of interstitial lung di2
Causes of interstitial lung di2
- Obtain a detailed history for possible environmental exposures or medication use to exclude potential causes of interstitial lung disease (ILD). (medscape.com)
- Our pulmonologists have the experience and training to find the underlying causes of interstitial lung disease. (nortonhealthcare.com)
Alveolar1
- The mnemonic "PAINT" has been used to divide the causes of restrictive lung disease into pleural, alveolar, interstitial, neuromuscular, and thoracic cage abnormalities. (medscape.com)
ILDs11
- Interstitial lung diseases (ILDs) are some of the first and most serious complications of connective tissue diseases (CTDs). (zaphod.dev)
- However, the pathogenesis of CTD-related ILDs (CTD-ILDs) is still unclear and their treatment often depends on functional and radiographic disease progression as well as on patient age and comorbidities. (zaphod.dev)
- We used data from the INBUILD and INPULSIS trials to investigate the natural history of progressive fibrosing interstitial lung diseases (ILDs). (ersjournals.com)
- Along with these clinical similarities, progressive fibrosing ILDs appear to share pathobiological mechanisms that may represent a common fibrotic response to tissue injury [ 6 - 10 ]. (ersjournals.com)
- Classification of the main interstitial lung diseases (ILDs). (ersjournals.com)
- By definition, pulmonary oedema from chronic heart failure, neoplastic diseases (lymphangitis carcinomatosa, lymphoma, lepidic growth adenocarcinoma) and chronic infections (pneumocystosis, miliary tuberculosis) are excluded from ILDs of identified cause. (ersjournals.com)
- In addition to that classification, several rare histologic patterns of ILDs have been described, including acute fibrosing and organising pneumonia (AFOP) and bronchiolocentric patterns of interstitial pneumonia. (ersjournals.com)
- The term "non-specific interstitial pneumonia" can apply to a distinct disease classified amongst IIPs (idiopathic non-specific interstitial pneumonia), or a pulmonary histologic pattern that can also be encountered in diverse ILDs of known cause, or even in other IIPs. (ersjournals.com)
- Ventilatory inefficiency and impairment of pulmonary gas exchange are key mechanisms of dyspnoea and exercise intolerance in patients with interstitial lung diseases (ILDs). (ersjournals.com)
- Frequently, however, ILDs can also be associated with a specific environmental exposure or an underlying connective tissue disease. (ersjournals.com)
- The term idiopathic interstitial pneumonia encompasses a wide range of different ILDs with unique clinical, radiological and pathological features. (ersjournals.com)
Parenchymal lung1
- Macedo P, Coker RK, Partridge MR, 2007, Is there a uniform approach to the management of diffuse parenchymal lung disease (DPLD) in the UK? (imperial.ac.uk)
Pneumonias4
- General reference Idiopathic interstitial pneumonias (IIPs) are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic. (merckmanuals.com)
- An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. (merckmanuals.com)
- Idiopathic interstitial pneumonias (IIPs) are most often sporadic, but familial cases constitute up to 2-20% of cases. (ersjournals.com)
- Interstitial pneumonias are a confusing and frustrating set of diseases both for the treating physician and for the diagnostic pathologist. (medicalmarijuana.com)
Fibrotic4
- Pulmonary 5-HT 2B receptor expression in fibrotic interstitial lung diseases. (lu.se)
- They include idiopathic fibrotic diseases, connective-tissue diseases, drug-induced lung disease, environmental exposures (inorganic and organic dusts), and primary diseases of the lungs (including sarcoidosis). (medscape.com)
- Honeycomb appearance of a fibrotic lung. (wikidoc.org)
- OGG1 and SMAD7 interact to induce fibroblast proliferation and differentiation and display roles in fibrotic murine and IPF patient lung tissue. (lu.se)
Patients42
- [ 7 , 8 ] It has been suggested that some patients with ILD and autoimmune features who do not meet clinical criteria for CTDs may have a lung-predominant form of a CTD. (medscape.com)
- Fifty percent of patients with SSc go on to develop clinically significant lung disease (SSc-ILD). (medscape.com)
- When these proposed criteria were applied to 422 patients previously diagnosed with idiopathic interstitial pneumonia (IIP) or undifferentiated CTD-ILD (UCTD), 144 (34%) met IPAF criteria. (medscape.com)
- Over the last 6 years, we and other investigators have published series of cases in which MMF was observed to be safe, well tolerated, and associated with stable or improved lung function in patients with ILD related to various CTD 4 , 5 , 6 , 7 , 8 , 9 , 10 . (jrheum.org)
- We hypothesized that MMF would be associated with minimal toxicity and would be effective in preserving lung function in patients with CTD-ILD. (jrheum.org)
- Some disease subgroups may respond better than others, however, more work is needed to define its role in managing these patients. (bris.ac.uk)
- HALAVEN is indicated for the treatment of patients with metastatic breast cancer who have previously received at least two chemotherapeutic regimens for the treatment of metastatic disease. (drugs.com)
- Reuters Health)-The safety and effectiveness of cancer immunotherapy is largely unknown in patients with autoimmune diseases, researchers say-and that might account for up to a quarter of individuals with lung cancer. (the-rheumatologist.org)
- Providing patients compassionate care and comprehensive treatment of diseases affecting the joints and connective tissues. (uab.edu)
- Our team of lung specialists partner with patients and their caregivers to develop customized, comprehensive care plans based on the latest research and treatment options. (nationaljewish.org)
- HYCAMTIN® for injection, as a single agent, is indicated for the treatment of patients with metastatic ovarian cancer after disease progression on or after initial or subsequent chemotherapy. (globalrph.com)
- HYCAMTIN for injection, as a single agent, is indicated for the treatment of patients with small cell lung cancer (SCLC) with platinum-sensitive disease who progressed at least 60 days after initiation of first-line chemotherapy. (globalrph.com)
- To explore the possibility of predicting the clinical types of Corona-Virus-Disease-2019 (COVID-19) pneumonia by analyzing the non-focus area of the lung in the first chest CT image of patients with COVID-19 by using automatic machine learning (Auto-ML). 136 moderate and 83 severe patients were selected from the patients with COVID-19 pneumonia. (nature.com)
- To stratify patients with polymyositis/dermatomyositis-associated interstitial lung disease (ILD) who were initially treated with an intensive regimen consisting of high-dose corticosteroids, a calcineurin inhibitor, and intravenous cyclophosphamide (triple-combo therapy) into subgroups based on mortality outcomes by a cluster analysis using a large-scale multicenter retrospective cohort of Japanese patients with myositis-associated ILD (JAMI). (frontiersin.org)
- Initial predictors for mortality previously reported in patients with myositis-associated ILD were used as variables and included age, sex, disease duration, classification of myositis, requirement of supplemental oxygen, anti-aminoacyl tRNA synthetase (ARS) antibody, anti-melanoma differentiation-associated gene 5 (MDA5) antibody, and serum levels of C-reactive protein (CRP) and Krebs von den Lungen-6 (KL-6). (frontiersin.org)
- In particular, interstitial lung disease (ILD) is one of the major manifestations associated with poor mortality in patients with polymyositis (PM)/dermatomyositis (DM) ( 2 ). (frontiersin.org)
- The management of ILD in patients with PM/DM aims to ameliorate, stabilize, or slow its progression based on the disease behavior of ILD ( 3 ). (frontiersin.org)
- The UCSF Lung Health Center at Parnassus offers comprehensive evaluation and treatment for patients with all types of lung disease other than lung cancer. (ucsfhealth.org)
- The UCSF Advanced COPD Clinic cares for patients with severe chronic obstructive pulmonary disease and emphysema. (ucsfhealth.org)
- We are also one of the few Bay Area clinics to offer treatments for severe emphysema - such as endobronchial valves and lung transplantation - to patients with COPD or alpha-1 antitrypsin deficiency (a genetic disease that increases the risk of developing emphysema). (ucsfhealth.org)
- Norton Pulmonary Specialists is a leader in treating interstitial lung disease (ILD) for patients in Louisville and Southern Indiana. (nortonhealthcare.com)
- The board-certified and fellowship-trained physicians with Norton Pulmonary Specialists are leaders in caring for Louisville and Southern Indiana patients with asthma, chronic obstructive pulmonary disease (COPD), emphysema and other lung diseases. (nortonhealthcare.com)
- OBJECTIVES: To describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH), and to examine clinical predictors of disease prognosis. (thedoctorsdoctor.com)
- PATIENTS: Nineteen children with cutaneous findings in the first 4 weeks of life and subsequently diagnosed with LCH based on compatible tissue histologic analysis, confirmed by electron microscopy and/or immunohistochemical analysis. (thedoctorsdoctor.com)
- One third of patients had disease limited to the skin and/or mucous membranes. (thedoctorsdoctor.com)
- Twelve of the 19 patients had multisystem disease, and 2 died of disease. (thedoctorsdoctor.com)
- The association with thyroid disease in the proband was explained partially by the involvement of the pituitary, with the relative risk decreasing when patients with diabetes insipidus and thyroid involvement were excluded from analysis. (thedoctorsdoctor.com)
- The objective of the present study was to evaluate the clinical usefulness of BLB in the overall management of patients with diffuse pulmonary diseases. (ersjournals.com)
- In the 25% of bronchoscopic lung biopsies that were clinically unhelpful, the reason for failure in approximately one-third of patients was the failure of the procedure to obtain an adequate quantity of lung parenchyma for a meaningful histological analysis. (ersjournals.com)
- 3 Males and patients with later age of onset tend to have more severe disease and poorer prognosis. (lww.com)
- The researchers studied the protocol in 197 patients with clinically indicated CT for various known and unknown lung function impairment. (news-medical.net)
- It can provide important preoperative identification of areas of emphysema and perfusion defects in patients with chronic thromboembolic pulmonary hypertension, a progressive disease caused by blood clots that do not clear from the lungs. (news-medical.net)
- Dr. Shin and colleagues first applied the protocol to patients with interstitial lung disease, a group of diseases that cause progressive scarring of the lung tissue. (news-medical.net)
- The American College of Rheumatology (ACR) has released a summary of upcoming guidelines on screening, monitoring, and treatment for interstitial lung disease (ILD) in patients with systemic autoimmune rheumatic disease. (medscape.com)
- More clinical data on ILD in patients with rheumatic disease would help strengthen evidence, she said, particularly for best practices in frequency of testing. (medscape.com)
- Short-term glucocorticoids are conditionally recommended as a first-line ILD treatment for patients with systemic autoimmune rheumatic disease-related ILD (SARD-ILD), excluding SSc-ILD. (medscape.com)
- Objective To clarify the underlying diseases, clinical manifestations, and treatment strategies for Amyloid A (AA) amyloidosis (AAA) in Japanese patients. (go.jp)
- Pirfenidone aimed to evaluate its efficacy at treating patients with unclassifiable disease, including patients with IPAF. (ersnet.org)
- Specific anti-RNP autoantibodies that preferentially bind to the apoptotic form of the U1 protein have been demonstrated in a group of patients with mixed connective tissue disease (29 of 53 tested). (medscape.com)
- Use of a Genomic Classifier in Patients with Interstitial Lung Disease: A Systematic Review. (cdc.gov)
- as monotherapy for the treatment of those patients who have received at least two chemotherapy regimens for their metastatic disease. (who.int)
- Biocon Biologics Ltd), BT-ON014 - in combination with docetaxel for the treatment of those patients who have not received chemotherapy for their metastatic disease - in combination with an aromatase inhibitor for the treatment of postmenopausal patients with hormone-receptor positive MBC, not previously treated with trastuzumab. (who.int)
Autoimmune Diseases1
- Autoimmune diseases are also possible causes. (nortonhealthcare.com)
Carbon monoxide2
- One was a carbon monoxide diffusing capacity test that estimates the ability of the lung to transfer oxygen from the air to the person's bloodstream. (cdc.gov)
- bDiffusion capacity of the lungs for carbon monoxide. (medscape.com)
Pathogenesis2
- Proteomics: Potential techniques for discovering the pathogenesis of connective tissue diseases-interstitial lung disease. (bvsalud.org)
- Although the exact pathogenesis is not fully understood, there are many initiating factors which cause the pulmonary tissue injury. (wikidoc.org)
Clinical10
- AILDR is an important clinical and research tool providing a platform for epidemiological data that will prove essential in promoting understanding of a rare cohort of lung disease and provide foundations for our aspiration to standardise investigation and treatment pathways of ILD across Australasia. (springer.com)
- Similar to infections in terrestrial hosts, morbillivirus infections may occur in marine mammals in cycles without overt clinical disease in susceptible animals, as documented for harbor seals ( 2 , 3 ). (cdc.gov)
- The BTS Clinical Statement on air travel for passengers with respiratory disease was published in Thorax in 2022. (imperial.ac.uk)
- Interstitial lung disease (ILD) encompasses a large and diverse group of pathological conditions that share similar clinical, radiological and pathological manifestations, despite potentially having quite different aetiologies and comorbidities. (ersjournals.com)
- Interstitial lung disease is a major cause of morbidity and mortality across several systemic autoimmune rheumatic diseases," Sindhu R. Johnson, MD, PhD, lead author of the new guidelines and director of the Clinical Epidemiology and Health Care Research program at the University of Toronto, Ontario, Canada, said in an ACR press release . (medscape.com)
- It's important to understand that we don't know whether most of these recommendations were just driven by expert opinion versus actual evidence from randomized, controlled clinical trials," said Volkmann, who codirects the Connective Tissue Disease-Related Interstitial Lung Disease (CTD-ILD) Program at the University of California, Los Angeles. (medscape.com)
- However, the clinical manifestations of diabetic kidney disease are diverse. (go.jp)
- Therefore, the clinical classification of diabetic kidney disease is clinically important and valuable. (go.jp)
- In Japan, two clinical staging systems divided by the estimated glomerular filtration rate (eGFR) and albuminuria can be used for diabetic kidney disease: the chronic kidney disease (CKD) risk classification and the Japanese classification of diabetic nephropathy. (go.jp)
- This system replaces the classification system published in 1986, which included only clinical disease criteria and which was developed before the widespread use of CD4+ T-cell testing (1). (cdc.gov)
Chronic5
- SSc, a chronic disease with multisystemic involvement, is characterized by widespread collagen deposition. (medscape.com)
- Systemic lupus erythematosus (SLE) is a chronic autoimmune connective tissue disorder, with a heterogeneous presentation. (lww.com)
- It may also be useful in follow-up of chronic obstructive pulmonary disease and looking at pathological findings in the lung tissue. (news-medical.net)
- Unlike obstructive lung diseases, such as asthma and chronic obstructive pulmonary disease (COPD), which show a normal or increased total lung capacity (TLC), restrictive disease are associated with a decreased TLC. (medscape.com)
- interstitial pneumonia: chronic lung disease affecting the interstitial tissue of the lungs. (medicalmarijuana.com)
Diffuse1
- Genetic testing for diffuse lung diseases in children. (cdc.gov)
Emphysema1
- 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)
Inflammatory3
- The collagen-induced arthritis (CIA) model was combined with the organic dust extract (ODE) airway inflammatory model to assess bone/joint-lung inflammatory outcomes. (cdc.gov)
- Data also support suppression of the lung inflammatory response, but increases in extracellular matrix protein deposition/interstitial disease in the setting of arthritis. (cdc.gov)
- Idiopathic inflammatory myopathies often affect extramuscular organs such as the skin, joints, lungs, heart and gastrointestinal tract ( 1 ). (frontiersin.org)
Presence of autoantibodies1
- As mentioned in the Introduction, the hallmark of mixed connective tissue disease is the presence of autoantibodies to U1 small nuclear ribonucleoproteins, in particular a 70-kDa U1 protein. (medscape.com)
NSIP1
Pathophysiology1
- Archivos de Bronconeumologia is a scientific journal that preferentially publishes prospective original research articles whose content is based upon results dealing with several aspects of respiratory diseases such as epidemiology, pathophysiology, clinics, surgery, and basic investigation. (archbronconeumol.org)
Hypersensitivity pneumonitis6
- 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)
Complications1
- Interstitial lung disease (ILD) is one of the most serious lung complications of connective tissue disease (CTD). (bvsalud.org)
Airway2
- The main blood vessels and airway of the new lung are sewn to your blood vessels and airway. (medlineplus.gov)
- Airway diseases are conditions that affect this network of tubes. (clevelandclinic.org)
Connective Tissue11
- Perform serologic testing to exclude connective-tissue disease. (medscape.com)
- Small series suggest mycophenolate mofetil (MMF) is well tolerated and may be an effective therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). (jrheum.org)
- ILD may arise as a result of a specific occupational or environmental exposure or as a manifestation of underlying connective tissue disease (CTD). (jrheum.org)
- Interstitial lung disease in connective tissue diseases: What are the important questions? (elsevierpure.com)
- Nucera V, Gerratana E, Giallanza M, La Corte L, Sangari D, Atzeni F. Efficacy of cyclophosphamide in treating connective tissue disease-related interstitial lung diseases. (zaphod.dev)
- SAN FRANCISCO-Lung involvement is a frequent and often life-threatening manifestation of the connective tissue diseases (CTDs) that are commonly encountered by rheumatologists. (the-rheumatologist.org)
- Connective tissue or autoimmune disease-related ILD (e.g. (nationaljewish.org)
- Lung disease caused by scleroderma and other connective tissue diseases . (clevelandclinic.org)
- The most frequent presentation of mixed connective tissue disease (MCTD) is a child with polyarthritis, general malaise, and Raynaud phenomenon. (medscape.com)
- Specific causes of mixed connective tissue disease remain undefined. (medscape.com)
- They quickly diagnosed that I had a mixed connective tissue disease with overlapping lupus and scleroderma. (medlineplus.gov)
Severe5
- Severe cases may risk permanent damage to the lungs and death from severe tachypnea. (wikipedia.org)
- Children under the age of 2 may present with signs of interstitial lung disease and be diagnosed with NEH following severe progression. (wikipedia.org)
- Lung transplants may be recommended for people under age 65 who have severe lung disease. (medlineplus.gov)
- Nearly 100% of people with severe mitral valve disease . (clevelandclinic.org)
- Studies of the natural history of HIV infection have documented a wide spectrum of disease manifestations, ranging from asymptomatic infection to life-threatening conditions characterized by severe immunodeficiency, serious opportunistic infections, and cancers (4-13). (cdc.gov)
Lesions2
- CONCLUSIONS: Vesiculopustular lesions are common in congenital/neonatal LCH, but the morphologic characteristics of lesions are not helpful in predicting the extent of disease. (thedoctorsdoctor.com)
- Lund University is one of the 21 sites within the consortium named TRISTAN (Translational Imaging in Drug Safety Assessment), that perform preclinical models and apply imaging techniques such as MRI, CT and PET to assess lesions in the lung tissue caused by drug administration. (lu.se)
Acute1
- 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)
Restrictive3
- Typical results are restrictive physiology, with reduced lung volumes and diffusion capacity. (merckmanuals.com)
- Restrictive lung diseases are characterized by reduced lung volumes, either because of an alteration in lung parenchyma or because of a disease of the pleura, chest wall, or neuromuscular apparatus. (medscape.com)
- Restrictive lung diseases are characterized by a reduction in FRC and other lung volumes because of pathology in the lungs, pleura, or structures of the thoracic cage. (medscape.com)
Respiratory failure1
- Interstitial Lung Disease (ILD) is a group of respiratory conditions affecting the lung interstitium often associated with progressive respiratory failure. (springer.com)
Diagnostic1
- Our specialists use robotic-assisted bronchoscopy to perform minimally invasive biopsies of the lung, which allows for greater precision during the important diagnostic procedure. (nortonhealthcare.com)
Arteries4
- Pulmonary hypertension is high blood pressure in your pulmonary arteries, which carry oxygen-poor blood from your heart to your lungs. (clevelandclinic.org)
- High blood pressure in your pulmonary arteries forces your heart to work harder to send oxygen-poor blood to your lungs. (clevelandclinic.org)
- Certain lung problems cause the arteries in your lungs to tighten. (clevelandclinic.org)
- Less blood can flow through your lungs, raising the pressure in your pulmonary arteries. (clevelandclinic.org)
Chest9
- Chest CT gives a better look at the lungs to see signs of pneumonia. (wikipedia.org)
- For single lung transplants, the cut is made on the side of your chest where the lung will be transplanted. (medlineplus.gov)
- For double lung transplants, the cut is made below the breast and reaches to both sides of the chest. (medlineplus.gov)
- Chest tubes are inserted to drain air, fluid, and blood out of the chest for several days to allow the lungs to fully re-expand. (medlineplus.gov)
- Chest CT is the imaging method of choice for analyzing lung disease and tracking changes over time. (news-medical.net)
- Researchers in Germany and the Netherlands developed a chest imaging protocol that yields information on structure and function of the lungs as a one-stop-shop procedure. (news-medical.net)
- The volume of FRC is determined by the balance of the inward elastic recoil of the lungs and the outward elastic recoil of the chest wall. (medscape.com)
- Therefore, it is influenced by any disease of the lungs, pleura, or chest wall. (medscape.com)
- It is conditionally recommended that providers do not use 6-minute walk test distance, chest radiography, or bronchoscopy for screening or monitoring disease. (medscape.com)
Drug-induced1
Pneumonitis2
- dNonspecific interstitial pneumonitis. (medscape.com)
- Giant cell interstitial pneumonitis (GIP) is now considered a hard metal pneumoconiosis. (medicalmarijuana.com)
HRCT1
- HRCT, which distinguishes airspace from interstitial disease, is the most useful test and is always done. (merckmanuals.com)
Exercise intolerance1
- Tachypnea Hypoxemia retractions crackles (in lungs) exercise intolerance There is not cause of Neuroendocrine hyperplasia, however some known causes are a rapid increase of pulmonary endocrine cells in the lungs in children under the age of 2. (wikipedia.org)