AnatomyDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesDisciplines and OccupationsTechnology, Industry, AgricultureInformation ScienceHealth Care
Connective Tissue DiseasesMixed Connective Tissue DiseaseConnective TissueScleroderma, SystemicLung Diseases, InterstitialLung DiseasesAntibodies, AntinuclearCollagen DiseasesRaynaud DiseaseLupus Erythematosus, SystemicConnective Tissue Growth FactorAutoantibodiesLungSkin and Connective Tissue DiseasesSjogren's SyndromesnRNP Core ProteinsDermatomyositisHypertension, PulmonaryCREST SyndromeConnective Tissue CellsRheumatic DiseasesBreast ImplantsMyositisArthritis, RheumatoidRibonucleoprotein, U1 Small NuclearPolymyositisAutoantigensPulmonary FibrosisScleroderma, LocalizedPolyarteritis NodosaAutoimmune DiseasesMucinosesRibonucleoproteins, Small NuclearVasculitisRibonucleoproteinsIdiopathic Interstitial PneumoniasNailsSiliconesLung Diseases, ObstructiveMarfan SyndromePrednisoloneRespiratory Function TestsMicroscopic AngioscopyToesWegener GranulomatosisSurgery, PlasticEnzyme-Linked Immunosorbent AssayArthritisCryptogenic Organizing PneumoniaLung NeoplasmsRNA, Small CytoplasmicRheumatoid NoduleHydroxychloroquineImmunoglobulin GCounterimmunoelectrophoresisPericardial EffusionFatal OutcomeFluorescent Antibody TechniqueCranial Nerve DiseasesRheumatoid FactorBiological MarkersMicroscopic PolyangiitisTomography, X-Ray ComputedEsophageal Motility DisordersFingersFibroblastsScleritisHLA-DR4 AntigenNucleoproteinsRetrospective StudiesAntigens, NuclearSkinAntibody SpecificityCollagenLung TransplantationLung InjuryImmunosuppressive AgentsImmediate-Early ProteinsSkin DiseasesIntercellular Signaling Peptides and ProteinsImmunoblottingBehcet SyndromeMethylprednisoloneCardiac TamponadeCase-Control StudiesBiopsyChronic Disease
Rheumatoid arthritisFibrosisTransplantationMCTDSclerodermaBiopsyDisordersComputed tomographyIdiopathicOrgansPathogenesisComplicationsChronicInterstitial Lung Disease CSacs of the lungsPatientsCTDsDiagnosisRheumaticSymptomsImmune systemDermatomyositisAbstractPresence of autoantibodiesClinicalCyclophosphamideRheumatologyManifestationAnkylosing spondylitisShortness of breDecline in lung functionJointsTransplantRespiratoryInflammatoryDiffuseRestrictive lung diDrugsChestLupusMorbidityMusculoskeletal
Rheumatoid arthritis9
Fibrosis42
  • 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)
  • In the early stages of lung injury, the degree of inflammation of interstitial and alveolar spaces is associated with subsequent fibrosis. (medscape.com)
  • Following the initial insult, further activation of fibroblasts and myofibroblasts leads to lung fibrosis and scarring. (medscape.com)
  • 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)
  • There is emerging evidence that pirfenidone and nintedanib may work for people with fibrosis due to rheumatoid lung disease. (medlineplus.gov)
  • Pulmonary fibrosis is a condition in which the lungs become scarred over time. (wikipedia.org)
  • Lung sound in idiopathic pulmonary fibrosis velcro crackles on auscultation in a person with idiopathic pulmonary fibrosis Problems playing this file? (wikipedia.org)
  • Pulmonary fibrosis may be a secondary effect of other diseases. (wikipedia.org)
  • Diseases and conditions that may cause pulmonary fibrosis as a secondary effect include: Inhalation of environmental and occupational pollutants, such as metals in asbestosis, silicosis and exposure to certain gases. (wikipedia.org)
  • Smoking is a known cause of some types of lung fibrosis, such as the entity smoking-related interstitial fibrosis (SRIF). (wikipedia.org)
  • Such scar tissue causes an irreversible decrease in oxygen diffusion capacity, and the resulting stiffness or decreased compliance makes pulmonary fibrosis a restrictive lung disease. (wikipedia.org)
  • In contrast, quadriplegia and kyphosis are examples of causes of restrictive lung disease that do not necessarily involve pulmonary fibrosis. (wikipedia.org)
  • Common genes implicated in fibrosis are Transforming Growth Factor-Beta (TGF-β), Connective Tissue Growth Factor (CTGF), Epidermal Growth Factor Receptor (EGFR), Interleukin-13 (IL-13), Platelet-Derived Growth Factor (PDGF), Wnt/β-catenin signaling pathway. (wikipedia.org)
  • Idiopathic pulmonary fibrosis, or IPF, is a condition that causes progressive scarring of the lungs. (ucsfhealth.org)
  • UCSF offers specialized care for all types of interstitial lung disease, including idiopathic pulmonary fibrosis. (ucsfhealth.org)
  • Diagnosing idiopathic pulmonary fibrosis requires input from pulmonologists, radiologists and, in many cases, pathologists experienced in evaluating patients with interstitial lung disease. (ucsfhealth.org)
  • Disease-modifying treatment aims at inhibiting tissue fibrosis and vascular and immune system alterations, which are the three crucial components of disease pathogenesis. (medscape.com)
  • Supporting the FVC results, high-resolution computed tomography showed less progression of lung fibrosis with tocilizumab than with placebo. (medscape.com)
  • For example, trials of rituximab have yielded promising results, with improvement of skin fibrosis and prevention of worsening lung fibrosis. (medscape.com)
  • 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)
  • He is particularly interested in lung diseases leading to fibrosis, including those from exposures to foreign materials. (yalemedicine.org)
  • 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)
  • Human herpesvirus 8 (HHV-8) antibodies were detected in 1 of 33 patients with pulmonary hypertension (including in 1 of 16 with idiopathic pulmonary arterial hypertension), 5 of 29 with cystic fibrosis, and 3 of 13 with interstitial lung disease. (cdc.gov)
  • The 42 patients without PH included 29 patients with cystic fibrosis (PAP 21.1 ± 3.3 mm Hg) and 13 patients with interstitial lung disease (PAP 18 ± 4.6 mm Hg) (8 patients with idiopathic pulmonary fibrosis, 2 with sarcoidosis, 3 with pulmonary fibrosis secondary to bleomycin treatment). (cdc.gov)
  • 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)
  • 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)
  • Scleroderma is a relatively rare connective tissue disorder characterized by skin fibrosis, obliterative vasculopathy and distinct autoimmune abnormalities. (bvsalud.org)
  • Many other clinical conditions present substantial skin fibrosis and may be potentially confused with Scleroderma, sometimes leading to a wrong diagnosis (eosinophilic fasciitis, systemic amyloidosis, scleromyxedema, graft-versus-host disease, progeroid disorders, stiff skin syndrome). (bvsalud.org)
  • In most cases, even when the etiology is known or suspected, the precise pathogenetic mechanisms leading to skin and tissue fibrosis remain elusive. (bvsalud.org)
  • Jan 29 (Reuters) - Corbus Pharmaceuticals Holdings Inc said on Monday the U.S. Food and Drug Administration will evaluate its experimental cystic fibrosis drug without requiring proof the product improves lung function, dramatically cutting the time and cost it will take Corbus to develop the drug. (medscape.com)
  • Companies developing drugs for cystic fibrosis, a life-threatening genetic disorder that damages the lungs and affects about 30,000 Americans, have historically been required to show an improvement in lung function based on a measure known as forced expiratory volume in one second, or FEV1. (medscape.com)
  • The FDA has never allowed a reduction in disease flare-up by itself to be the main goal of a clinical trial for a cystic fibrosis drug. (medscape.com)
Transplantation17
  • Patients with connective tissues disease (CTD) are often on immunomodulatory agents before lung transplantation (LTx). (elsevierpure.com)
  • The International Society for Heart and Lung Transplantation-supported consensus document on LTx in patients with CTD addresses the risk and contraindications of perioperative and post-transplant management of the biologic disease-modifying antirheumatic drugs (bDMARD), kinase inhibitor DMARD, and biologic agents used for LTx candidates with underlying CTD, and the recommendations and management of non-gastrointestinal extrapulmonary manifestations, and esophageal disorders by medical and surgical approaches for CTD transplant recipients. (elsevierpure.com)
  • Journal of Heart and Lung Transplantation , 40 (11), 1279-1300. (elsevierpure.com)
  • In severe cases, lung transplantation can be considered. (medlineplus.gov)
  • Lung transplantation may occasionally be an option. (wikipedia.org)
  • For patients who get worse despite treatment, lung transplantation may be an option. (ucsfhealth.org)
  • Early recognition of these factors may guide decisions regarding more aggressive therapy, including consideration for lung transplantation. (ersjournals.com)
  • We retrospectively analyzed data from 75 patients referred to the Department of Cardiovascular and Respiratory Sciences of the University of Rome La Sapienza from January 2001 to February 2004 for clinical and serologic (hepatitis C virus, hepatitis B virus, HHV-8, and cytomegalovirus) evaluation for lung transplantation. (cdc.gov)
  • The 75 patients (38 male), who were candidates for lung transplantation, were all born and living in Italy. (cdc.gov)
  • Sirolimus Long-Term Tolerability and Impact on Kidney Function in Lung Transplantation: A Single-Center Experience. (ucsd.edu)
  • An Unexpected Cause of Lung Disease Identified After Lung Transplantation. (ucsd.edu)
  • Prone Positioning after Lung Transplantation Remains Prone to Debate. (ucsf.edu)
  • Management of primary graft dysfunction after lung transplantation with extracorporeal life support: an evidence-based review. (ucsf.edu)
  • The Association Between Frailty and Chronic Lung Allograft Dysfunction After Lung Transplantation. (ucsf.edu)
  • Chronic graft versus host disease (cGVHD) is the most common consequence of allogeneic bone marrow transplantation, and it is associated with morbidity and mortality. (bvsalud.org)
  • West Nile Virus transmission through tissue transplantation, for instance, skin, muscle, or connective tissues, has not been identified, and the risk for transmission by this route is not known. (cdc.gov)
  • In 2011, the CDC assisted state and local health departments in an investigation of a cluster of West Nile Virus disease transmitted through solid organ transplantation. (cdc.gov)
MCTD1
Scleroderma7
  • These studies provided support for the Scleroderma Lung Study II, in which MMF is being compared head-to-head with CYC in subjects with SSc-ILD. (jrheum.org)
  • A recent study found that mycophenolate mofetil is an effective alternative for the treatment of systemic scleroderma with interstitial lung disease, resulting in possible long-term improvement. (the-rheumatologist.org)
  • There are more than 200 of these conditions, including genetic disorders such as Ehlers-Danlos syndrome, autoimmune disorders such as scleroderma , and cancers such as soft tissue sarcomas . (medicalnewstoday.com)
  • PH can exist alone or be associated with other conditions such as connective tissue disorders (scleroderma, lupus and others), heart disease, HIV and COPD. (prweb.com)
  • They quickly diagnosed that I had a mixed connective tissue disease with overlapping lupus and scleroderma. (medlineplus.gov)
  • They also found I had pulmonary arterial hypertension and interstitial lung disease which resulted from the scleroderma. (medlineplus.gov)
  • I am taking methotrexate which has helped keep my lungs stable from the impact of scleroderma. (medlineplus.gov)
Biopsy10
  • 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)
  • Diagnosis may be based on symptoms, medical imaging, lung biopsy, and lung function tests. (wikipedia.org)
  • In adults, the procedure is most often used to take samples of (biopsy) suspicious lesions and for culturing specific areas in the lung. (mountsinai.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)
  • Surgical lung biopsy. (ucsfhealth.org)
  • A cardiothoracic surgeon performs the surgical lung biopsy, usually with small tools and cameras through one-inch-long incisions. (ucsfhealth.org)
  • Sometimes a biopsy of affected tissues may be required for diagnosis. (allergy.org.au)
Disorders9
  • 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)
  • Examples include autoimmune disorders, viral infections and bacterial infection like tuberculosis which may cause fibrotic changes in both lung's upper or lower lobes and other microscopic injuries to the lung. (wikipedia.org)
  • Autoimmune diseases are a broad range of more than eighty related disorders, ranging from common to very rare. (allergy.org.au)
  • Less common autoimmune diseases include systemic lupus erythematosus (SLE), also known as lupus, and vasculitis disorders (inflammation of blood vessels). (allergy.org.au)
  • They can be broadly classified into rheumatological disease and vasculitis disorders. (allergy.org.au)
  • The Rheumatology clinical program enjoys a wealth of diverse clinical expertise, spanning the entire spectrum of rheumatological diseases from musculoskeletal disorders to systemic autoimmune conditions and other rheumatologic diseases. (houstonmethodist.org)
  • Overview of Autoimmune Disorders of Connective Tissue In an autoimmune disorder, antibodies or cells produced by the body attack the body's own tissues. (msdmanuals.com)
  • Many autoimmune disorders affect connective tissue and a variety of organs. (msdmanuals.com)
Computed tomography1
  • and no evidence of parenchymal pulmonary disease or vascular pathology in the image (chest X-ray and high-resolution computed tomography). (scirp.org)
Idiopathic4
  • Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? (nih.gov)
  • We hypothesized that idiopathic NSIP is an autoimmune disease and the lung manifestation of undifferentiated connective tissue disease (UCTD), a recently described, distinct entity. (nih.gov)
  • 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)
  • 1. Patients with diagnosed idiopathic pulmonary artery hypertension (IPAH)/drug-induced pulmonary artery hypertension (DPAH)/heritable pulmonary arterial hypertension (HPAH)/connective tissue disease (CTD) PAH who are stable for the past three months on PH-specific targeted therapy. (who.int)
Organs7
  • Connective tissue provides the framework and support for tissues throughout the body, including bones, muscles, blood vessels, and organs. (medicalnewstoday.com)
  • In autoimmune diseases the immune system produces antibodies that attack the body's own cells, tissues and organs, resulting in inflammation and damage. (allergy.org.au)
  • Whilst localised (organ specific) autoimmune diseases mainly affect a single organ or tissue, the effects frequently extend to other body systems and organs. (allergy.org.au)
  • Systemic autoimmune diseases can affect many body organs and tissues at the same time. (allergy.org.au)
  • An autoimmune disorder caused when antibodies-immune system cells that fight off bacteria and viruses-mistakenly attack healthy body tissues and organs. (lupus.org)
  • Skin changes Systemic sclerosis is a rare, chronic autoimmune connective tissue disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities. (msdmanuals.com)
  • Extra-articular involvement of organs such as the skin, heart, lungs, and eyes can be significant. (medscape.com)
Pathogenesis3
  • 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)
  • 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)
  • Proteomics: Potential techniques for discovering the pathogenesis of connective tissue diseases-interstitial lung disease. (bvsalud.org)
Complications4
Chronic10
  • SSc, a chronic disease with multisystemic involvement, is characterized by widespread collagen deposition. (medscape.com)
  • Ozasa M, Fuluka J, Smith ML. Chronic diffuse lung diseases. (medlineplus.gov)
  • Treatments include medication to slow the decline in lung function, care for other health problems that often affect patients with IPF, and a special exercise and education program designed for patients with chronic lung disease. (ucsfhealth.org)
  • Sixteen of them had IPAH (PAP 53.4 ± 17.1 mm Hg), whereas among the 17 patients with secondary PH, 7 patients had chronic thromboembolic PH (PAP 40.3 ± 7.8), and the remaining 10 patients (PAP 37.1 ± 12.2 mm Hg) had PH associated with connective tissue disease (4 patients), HIV infection (3 patients), and lung disease (3 patients). (cdc.gov)
  • Tolerability and Feasibility of the Upper Esophageal Sphincter Assist Device in Preventing Acute and Chronic Allograft Rejection Among Lung Transplant Recipients. (ucsd.edu)
  • Short Airway Telomeres are Associated with Primary Graft Dysfunction and Chronic Lung Allograft Dysfunction. (ucsf.edu)
  • Incidence of first cardiovascular event in spanish patients with chronic inflammatory rheumatic diseases. (unican.es)
  • RA is a chronic systemic inflammatory disease of unknown cause. (medscape.com)
  • Clinical manifestations of chronic GVHD are similar to autoimmune collagen vascular disease and the two main types of cutaneous chronic GVHD are lichenoid and sclerodermatous. (bvsalud.org)
  • Chronic graft-vs-host disease (cGVHD) is the most common complication following allogeneic BMT [10]. (bvsalud.org)
Interstitial Lung Disease C1
  • The National Jewish Health Interstitial Lung Disease Center for Patient Care, Education, Discovery and Innovation is one of the largest interstitial lung disease (ILD) centers in the country. (nationaljewish.org)
Sacs of the lungs1
  • which affect the tissue around the air sacs of the lungs (alveoli), are the most common lung problems. (msdmanuals.com)
Patients35
  • [ 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)
  • 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)
  • 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)
  • 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)
  • ABSTRACT Chagas disease (CD) is a neglected tropical disease associated with poverty in which patients are surrounded by stigma. (scielo.br)
  • There are no proven risk factors for IPF, but a minority of patients have a family history of lung scarring. (ucsfhealth.org)
  • Some patients with these connective tissue diseases develop lung problems before the more typical signs and symptoms appear. (ucsfhealth.org)
  • After 48 weeks, decline in lung function from baseline, as defined by changes in predicted forced vital capacity (FVC), had occurred 50.5% of patients in the tocilizumab arm, versus 70.3% of those in the placebo arm ( P = 0.015). (medscape.com)
  • In patients with systemic sclerosis-related interstitial lung disease, a clinically meaningful decline of 10% or more in lung function was seen in 24.5% of placebo recipients, compared with 8.6% of tocilizumab recipients. (medscape.com)
  • Patients often require hospitalisation during the course of their disease, typically for bouts of right heart failure (RHF). (ersjournals.com)
  • We investigated if signs of immune activation are present in bronchial biopsies and bronchoalveolar lavage (BAL) of patients with early-untreated RA without clinical signs of lung involvement. (bmj.com)
  • Conclusions Signs of immune cell accumulation and activation are present both in the bronchial tissue and in BAL of untreated patients with early RA without concomitant lung disease, strengthening the role of the lung compartment as an important player in ACPA-positive RA. (bmj.com)
  • Presence of anticitrullinated proteins antibodies (ACPA) in approximately two-thirds of the patients with RA defines a more aggressive disease phenotype. (bmj.com)
  • 5 Smoking increases expression of citrullinated proteins and peptidylarginine deiminase type 2 (PAD2) in the lungs of healthy smokers, 6 , 7 and we have recently confirmed this finding in patients with early untreated RA as well. (bmj.com)
  • Identification of shared citrullinated epitopes in the lungs and joints of patients with RA 9 together with local enrichment of ACPA in the bronchoalveolar lavage (BAL) of patients with early untreated RA and in the induced sputum of arthritis-free individuals at risk for developing RA 10 support the notion that RA-associated autoimmunity might indeed initiate in the lung compartment. (bmj.com)
  • 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)
  • Human herpesvirus 8 (HHV-8) has been detected in patients with Kaposi sarcoma, primary-effusion B-cell lymphomas, and Castleman disease ( 1 ). (cdc.gov)
  • lung tissue from 1 of these patients was positive for latency-associated nuclear antigen-1 ( 3 ). (cdc.gov)
  • HHV-8 latency-associated nuclear antigen-1 and HHV-8 viral cyclin gene were identified in the lung tissue of 10 (62.5%) of 16 patients with IPAH, whereas only 1 (7.1%) of 14 patients with associated pulmonary hypertension (PH) had HHV-8 gene sequences in lung tissue ( 2 ). (cdc.gov)
  • 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)
  • There is no definitive therapy, while corticosteroids may decrease symptoms and improve lung function in some patients. (scirp.org)
  • The objective of this study was to describe the main imaging findings in Shrinking Lung, an important pulmonary alteration in lupus patients. (scirp.org)
  • Serial CXRs demonstrate steadily declining lung volumes as SLS patients become more dyspneic, rendering the impression that the lungs are vanishing. (scirp.org)
  • She performs lung transplant surgery and lung volume reduction surgery for emphysema, and treats patients with tumors of the chest, including lung cancer, esophageal cancer, mediastinal tumors and mesothelioma. (ucsf.edu)
  • 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)
  • Abatacetp in spanish patients with arthritis rheumatoid and interstitial lung disease. (unican.es)
  • However, asbestos-associated diseases typically have long latency periods so many patients exposed to asbestos are asymptomatic for years before any clinically apparent asbestos-related disease develops. (cdc.gov)
  • Use of a Genomic Classifier in Patients with Interstitial Lung Disease: A Systematic Review. (cdc.gov)
  • [ 3 ] Anti-RA33 antibodies are found in about 30% of sera from RA patients but only occasionally in sera from patients with other connective tissue diseases. (medscape.com)
  • To show lung function improvement Cohen estimated the company would have had to enroll more than 1,500 patients and run the trial for a year. (medscape.com)
CTDs1
Diagnosis4
  • 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)
  • With early, accurate diagnosis, treatments can extend and improve the quality of life for many people living with the disease. (prweb.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)
  • Diagnosis of Behçet's Disease: Comparison of Two Sets of Classification Criteria. (unican.es)
Rheumatic1
  • 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)
Symptoms7
Immune system3
Dermatomyositis1
Abstract1
  • ABSTRACT Visceral leishmaniasis (VL) has shown endemic pattern and epidemic episodes in urban and rural areas, however, there are still gaps in knowledge with regards to disease transmission. (scielo.br)
Presence of autoantibodies1
Clinical9
  • Purpose: Progressive fibrosing interstitial lung disease (PF-ILD) is a phenotype defined by rapid clinical progression towards respiratory failure. (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)
  • 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)
  • 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)
  • Early clinical experience of bacteriophage therapy in 3 lung transplant recipients. (ucsd.edu)
  • Autoimmune diseases are usually diagnosed using a combination of clinical history, blood tests (autoantibodies, inflammation, organ function) and other investigations such as x-rays. (allergy.org.au)
  • These diseases are often managed by clinical immunology/allergy specialists and/or rheumatologists. (allergy.org.au)
  • Systemic Treatment in Behçet's Disease According to Clinical Phenotypes. (unican.es)
  • Although NHANES I provides a wealth of information on the prevalence of health conditions and risk factors, the cross-sectional nature of the original survey limits its usefulness in studying the effects of clinical, environmental, and behavioral factors and in tracing the natural history of disease. (cdc.gov)
Cyclophosphamide1
Rheumatology1
Manifestation1
  • 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)
Ankylosing spondylitis1
  • Subclinical atherosclertoic disease in ankylosing spondylitis and non-radiographic axial spondyloarthritis. (unican.es)
Shortness of bre1
  • In some people, abnormal lung function is the most serious problem, causing shortness of breath during exertion. (msdmanuals.com)
Decline in lung function1
  • Extracorporeal photopheresis to attenuate decline in lung function due to refractory obstructive allograft dysfunction. (ucsd.edu)
Joints2
  • 1 ACPAs develop prior to the onset of symptomatic joint disease 2 , 3 and associate with smoking, 4 suggesting that the RA-associated autoimmune responses are initially generated outside the joints, and perhaps at a mucosal site. (bmj.com)
  • Almost everyone with mixed connective tissue disease has aching joints. (msdmanuals.com)
Transplant8
  • 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)
  • UCSF is home to a high-performing lung transplant program with the expertise to handle the most complex, challenging cases. (ucsfhealth.org)
  • Development of Host Immune Response to Bacteriophage in a Lung Transplant Recipient on Adjunctive Phage Therapy for a Multidrug-Resistant Pneumonia. (ucsd.edu)
  • Heart and Lung Transplant for Mixed Connective Tissue Disease: A Case Report and Implications for Pretransplant Testing. (ucsd.edu)
  • Low dose posaconazole delayed release tablets for fungal prophylaxis in lung transplant recipients. (ucsd.edu)
  • Dr. Jasleen Kukreja is a cardiothoracic surgeon and director of the Lung Transplant Program at UCSF Medical Center. (ucsf.edu)
  • Kukreja is the associate director of UCSF's lung transplant research division of cardiothoracic surgery. (ucsf.edu)
  • J Heart Lung Transplant. (ucsf.edu)
Respiratory2
  • 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)
  • The term identifies a constellation of respiratory manifestations including dyspnea, reduced lung volumes and/or elevated hemidiaphragms on chest radiography (CXR), and a restrictive ventilatory defect assessed by pulmonary function tests (PFTs). (scirp.org)
Inflammatory3
  • Ocular Manifestations in Inflammatory Bowel Disease. (unican.es)
  • Ocular Scleral Pathology and Immune-Mediated Inflammatory Diseases. (unican.es)
  • Treatment with Certolizumab Pegol in Refractory Uveitis Secondary to Inmune-Mediated Inflammatory Diseases. (unican.es)
Diffuse1
Restrictive lung di1
  • It is the main cause of restrictive lung disease that is intrinsic to the lung parenchyma. (wikipedia.org)
Drugs3
  • PAH associated with drugs/toxins) and connective tissue disease (CTD)-associated PAH, thus excluding other causes of WHO group 1 PAH, such as portopulmonary hypertension, haemoglobinopathies or HIV. (ersjournals.com)
  • 1 year and naïve to disease-modifying antirheumatic drugs were subjected to bronchoscopy where BAL and mucosal bronchial biopsies were retrieved. (bmj.com)
  • Other factors such as infections and some drugs may also play a role in triggering autoimmune diseases. (allergy.org.au)
Chest3
  • 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)
  • Chest X-rays usually show small, diaphragmatic lungs. (scirp.org)
  • Chest tomography usually shows reduced lung volumes with diaphragmatic elevation, occasional basal atelectasis, without severe pulmonary or pleuropulmonary disease. (scirp.org)
Lupus3
  • Shrinking lung refers to a rare complication of systemic lupus erythematosus and is characterized by unexplained dyspnea, a restrictive pattern in lung function tests, and elevation of the diaphragmatic hemicuples. (scirp.org)
  • [ 1 ] Anti-RA33 positivity may also be associated with systemic lupus erythematosus and mixed connective-tissue disease. (medscape.com)
  • The condition often shares the features of a variety of autoimmune diseases, such as lichen planus, Sjögren's syndrome, systemic lupus erythematosus or primary biliary cirrhosis [12, 13]. (bvsalud.org)
Morbidity2
  • Pulmonary arterial hypertension (PAH) remains a disease with high morbidity and mortality rates despite recent advances in therapy and overall improved survival. (ersjournals.com)
  • Shrinking lung can cause significant morbidity and occasional mortality. (scirp.org)
Musculoskeletal1