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Richard Hubbard - One of the best experts on this subject based on the ideXlab platform.
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surgical Lung biopsy for the diagnosis of Interstitial Lung Disease in england 1997 2008
European Respiratory Journal, 2016Co-Authors: John P Hutchinson, Tricia M Mckeever, Andrew W Fogarty, Vidya Navaratnam, Richard HubbardAbstract:Introduction: International guidelines and new targeted therapies for idiopathic pulmonary fibrosis have increased the need for accurate diagnosis of Interstitial Lung Disease, which may lead to more surgical Lung biopsies. This study aims to assess the risk of this procedure in patients from the United Kingdom. Methods: We used Hospital Episodes Statistics data from 1997-2008 to assess the frequency of surgical Lung biopsy for Interstitial Lung Disease in England. We identified cardiothoracic surgical patients using ICD-10 codes for Interstitial Lung Disease and OPCS-4 codes for surgical Lung biopsy. We excluded those with Lung resections or Lung cancer. We estimated in-hospital, 30-day and 90-day mortality following the procedure, and linked to cause of death using data from the Office of National Statistics. Results: We identified 2,820 patients with Interstitial Lung Disease undergoing surgical Lung biopsy during the 12 year period. The number of biopsies increased over the time period studied. In-hospital, 30-day and 90-day mortality were 1.7%, 2.4% and 3.9% respectively. Male sex, increasing age, increasing co-morbidity and open surgery were risk factors for mortality. Discussion: Surgical Lung biopsy for Interstitial Lung Disease has a similar mortality to lobectomy for Lung cancer, and clinicians and patients should understand the likely risks involved.
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in hospital mortality after surgical Lung biopsy for Interstitial Lung Disease in the united states 2000 to 2011
American Journal of Respiratory and Critical Care Medicine, 2016Co-Authors: John P Hutchinson, Tricia M Mckeever, Andrew W Fogarty, Richard HubbardAbstract:Rationale: Surgical Lung biopsy can help to determine a specific diagnosis in Interstitial Lung Disease but has associated risks. Most currently available mortality data are derived from case series and may not be generalizable to broader populations.Objectives: To assess in-hospital mortality after surgical Lung biopsy for Interstitial Lung Disease in a national secondary care dataset from the United States.Methods: Data were obtained from the 2000–2011 Nationwide Inpatient Sample. Cases were identified using International Classification of Diseases codes for Interstitial Lung Disease and surgical Lung biopsies. Lung resections and cases of Lung cancer were excluded. Weighted data were used to estimate numbers of biopsies nationwide and in-hospital mortality, and multivariable logistic regression was used to adjust for sex, age, geographic region, comorbidity, type of operation, and provisional diagnosis.Measurements and Main Results: We estimated there to be around 12,000 surgical Lung biopsies performe...
Aryeh Fischer - One of the best experts on this subject based on the ideXlab platform.
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Update on morbidity and mortality in systemic sclerosis–related Interstitial Lung Disease:
Journal of Scleroderma and Related Disorders, 2020Co-Authors: Elizabeth R. Volkmann, Aryeh FischerAbstract:Contemporary studies of systemic sclerosis consistently demonstrate that Interstitial Lung Disease is a leading cause of Disease-related death. This review summarizes morbidity and mortality outcomes in systemic sclerosis-related Interstitial Lung Disease patients from high-quality observational and interventional studies over the last 50 years. The data presented suggest a trend for improved morbidity and mortality outcomes among present day systemic sclerosis–associated Interstitial Lung Disease patients. Specifically, systemic sclerosis–associated Interstitial Lung Disease patients appear to be living longer from the time of the initial diagnosis. Despite improved survival, the number one cause of death for most systemic sclerosis–associated Interstitial Lung Disease patients remains respiratory failure from Interstitial Lung Disease. This review describes the most important demographic, clinical, and biological factors, which affect mortality in systemic sclerosis–associated Interstitial Lung Disease, and could be used to help stratify patients for closer monitoring and more aggressive initial treatment. The review concludes with an overview of future research needed to (1) understand how to personalize the care of systemic sclerosis–associated Interstitial Lung Disease patients to improve morbidity and mortality outcomes; and (2) investigate whether novel therapeutic interventions (e.g. anti-fibrotics, hematopoietic stem cell transplantation) offer any meaningful long-term survival advantage over the current standard of care.
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predictors of mortality in rheumatoid arthritis associated Interstitial Lung Disease
European Respiratory Journal, 2016Co-Authors: Joshua J Solomon, Aryeh Fischer, Evans R Fernandezperez, Jonathan H Chung, Gregory P Cosgrove, Kristen M Demoruelle, Stephen K Frankel, Stephen B Hobbs, Tristan J Huie, Jill KetzerAbstract:Interstitial Lung Disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and Disease behaviour over time in these patients.We identified rheumatoid arthritis-related Interstitial Lung Disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific Interstitial pneumonia (NSIP) or a "definite" or "possible" usual Interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model Disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, Disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.
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Interstitial Lung Disease in connective tissue disorders
The Lancet, 2012Co-Authors: Aryeh Fischer, Roland M. Du BoisAbstract:Summary Some of the most pressing challenges associated with Interstitial Lung Disease (ILD) are how best to define, diagnose, and treat connective tissue Disease-associated ILD (CTD-ILD)—disorders with potentially substantial morbidity and mortality. In this focused review, we address aspects of prognosis for CTD-ILD and what indices might predict outcome, together with lessons that can be learnt from clinical trials of systemic sclerosis-associated ILD and idiopathic pulmonary fibrosis and how these lessons might be applied to future studies of CTD-ILD.
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rheumatoid arthritis Interstitial Lung Disease associated mortality
American Journal of Respiratory and Critical Care Medicine, 2011Co-Authors: Amy L Olson, Jeffrey J Swigris, David Sprunger, Aryeh Fischer, Evans R Fernandezperez, Josh Solomon, James R Murphy, Marc Cohen, Ganesh Raghu, Kevin K BrownAbstract:Rationale: Mortality rates from rheumatoid arthritis–associated Interstitial Lung Disease (RA-ILD) are largely unknown.Objectives: We sought to determine mortality rates from rheumatoid arthritis–associated Interstitial Lung Disease in the United States from 1988 through 2004.Methods: Using data from the National Center for Health Statistics, we calculated age-adjusted mortality rates from the deaths of persons with rheumatoid arthritis–associated Interstitial Lung Disease, determined the prevalence of Interstitial Lung Disease in all decedents with rheumatoid arthritis, and compared the age and underlying cause of death in these two cohorts of decedents.Measurements and Main Results: From 1988 to 2004, there were 39,138,394 deaths in U.S. residents and 162,032 rheumatoid arthritis–associated deaths. Of these deaths, 10,725 (6.6%) met criteria for rheumatoid arthritis–associated Interstitial Lung. Mortality rates from rheumatoid arthritis fell over the course of this study in both women and men. However, ...
John P Hutchinson - One of the best experts on this subject based on the ideXlab platform.
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surgical Lung biopsy for the diagnosis of Interstitial Lung Disease in england 1997 2008
European Respiratory Journal, 2016Co-Authors: John P Hutchinson, Tricia M Mckeever, Andrew W Fogarty, Vidya Navaratnam, Richard HubbardAbstract:Introduction: International guidelines and new targeted therapies for idiopathic pulmonary fibrosis have increased the need for accurate diagnosis of Interstitial Lung Disease, which may lead to more surgical Lung biopsies. This study aims to assess the risk of this procedure in patients from the United Kingdom. Methods: We used Hospital Episodes Statistics data from 1997-2008 to assess the frequency of surgical Lung biopsy for Interstitial Lung Disease in England. We identified cardiothoracic surgical patients using ICD-10 codes for Interstitial Lung Disease and OPCS-4 codes for surgical Lung biopsy. We excluded those with Lung resections or Lung cancer. We estimated in-hospital, 30-day and 90-day mortality following the procedure, and linked to cause of death using data from the Office of National Statistics. Results: We identified 2,820 patients with Interstitial Lung Disease undergoing surgical Lung biopsy during the 12 year period. The number of biopsies increased over the time period studied. In-hospital, 30-day and 90-day mortality were 1.7%, 2.4% and 3.9% respectively. Male sex, increasing age, increasing co-morbidity and open surgery were risk factors for mortality. Discussion: Surgical Lung biopsy for Interstitial Lung Disease has a similar mortality to lobectomy for Lung cancer, and clinicians and patients should understand the likely risks involved.
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in hospital mortality after surgical Lung biopsy for Interstitial Lung Disease in the united states 2000 to 2011
American Journal of Respiratory and Critical Care Medicine, 2016Co-Authors: John P Hutchinson, Tricia M Mckeever, Andrew W Fogarty, Richard HubbardAbstract:Rationale: Surgical Lung biopsy can help to determine a specific diagnosis in Interstitial Lung Disease but has associated risks. Most currently available mortality data are derived from case series and may not be generalizable to broader populations.Objectives: To assess in-hospital mortality after surgical Lung biopsy for Interstitial Lung Disease in a national secondary care dataset from the United States.Methods: Data were obtained from the 2000–2011 Nationwide Inpatient Sample. Cases were identified using International Classification of Diseases codes for Interstitial Lung Disease and surgical Lung biopsies. Lung resections and cases of Lung cancer were excluded. Weighted data were used to estimate numbers of biopsies nationwide and in-hospital mortality, and multivariable logistic regression was used to adjust for sex, age, geographic region, comorbidity, type of operation, and provisional diagnosis.Measurements and Main Results: We estimated there to be around 12,000 surgical Lung biopsies performe...
Christopher P. Denton - One of the best experts on this subject based on the ideXlab platform.
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tocilizumab prevents progression of early systemic sclerosis associated Interstitial Lung Disease
Arthritis & Rheumatism, 2021Co-Authors: David Roofeh, Christopher P. Denton, Grace Kim, Jonathan G. Goldin, Celia J F Lin, Daniel E Furst, Suiyuan Huang, Dinesh KhannaAbstract:OBJECTIVE Tocilizumab has demonstrated Lung function preservation in two randomized controlled trials in early systemic sclerosis (SSc). This effect has yet to be characterized in terms of quantitative radiographic Lung involvement. In this post-hoc analysis, we assess tocilizumab's impact on Lung function preservation, stratifying treatment arms by the degree of radiographic Lung involvement. METHODS The focuSSced trial was a phase 3, randomized placebo-controlled trial of tocilizumab in patients with SSc and progressive skin Disease. Participants had baseline and serial spirometry along with high resolution chest CT at baseline and week 48. Quantitative Interstitial Lung Disease and fibrosis were derived using computer software. We divided quantitative Interstitial Lung Disease in mild (5-10%), moderate (>10-20%), or severe (>20%) categories. RESULTS Of 210 participants recruited in the trial, 136 [65%] had Interstitial Lung Disease. The majority of these participants had moderate-to-severe involvement defined by >10% Lung involvement (77%). The tocilizumab arm demonstrated preservation of forced vital capacity over 48 weeks (least squared mean change in %predicted = -0.1) compared to placebo (-6.3%). For mild, moderate, and severe QILD, the mean decline in the %pFVC in the tocilizumab arm at 48 weeks were -4.1, 0.7, and 2.1, and in the placebo group were -10.0, -5.7, and -6.7, respectively. Similar treatment-related preservation findings were seen independent of fibrosis severity. CONCLUSION Tocilizumab in early SSc- associated Interstitial Lung Disease with progressive skin Disease stabilized forced vital capacity over 48 weeks, independent of the extent of quantitative radiographic Interstitial Lung Disease or fibrosis.
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Interstitial Lung Disease in connective tissue Disease—mechanisms and management
Nature Reviews Rheumatology, 2014Co-Authors: Athol U. Wells, Christopher P. DentonAbstract:Pulmonary complications are an important extra-articular feature of autoimmune rheumatic Diseases and a major cause of mortality. The underlying pathogenesis probably involves multiple cellular compartments, including the epithelium, Lung fibroblasts, and the innate and adaptive immune system. Heterogeneity in the extent and progression of Lung fibrosis probably reflects differences in underlying pathogenic mechanisms. Growing understanding of the key pathogenic drivers of Lung fibrosis might lead to the development of more effective targeted therapies to replicate the treatment advances in other aspects of these Diseases. Interstitial Lung Disease (ILD) in connective tissue Disease (CTD) is characterized using the classification of the idiopathic Interstitial pneumonias. Systemic sclerosis is most frequently associated with ILD and, in most of these patients, ILD manifests as a histological pattern of nonspecific Interstitial pneumonia. Conversely, in rheumatoid arthritis, the pattern of ILD is most often usual Interstitial pneumonia. The key goals of clinical assessment of patients with both ILD and CTD are the detection of ILD and prognostic evaluation to determine which patients should be treated. Data from treatment trials in systemic sclerosis support the use of immunosuppressive therapy, with the treatment benefit largely relating to the prevention of progression of Lung Disease. Extra-articular features, particularly pulmonary complications, are a major cause of mortality in autoimmune rheumatic Disease Interstitial Lung Disease (ILD) is an important pulmonary complication in a variety of connective tissues Diseases, particularly systemic sclerosis Despite advances in understanding of Disease mechanisms, more work needs to be done to identify cardinal pathogenetic pathways in order to develop more efficacious therapies The most difficult clinical decision in ILD associated with connective tissue Disease is to determine when treatment should be initiated and when meticulous observation without intervention is appropriate The initiation of treatment is warranted when Disease progression is probable, based on pulmonary Disease severity, evidence of ongoing progression and short duration of systemic Disease Current treatment is essentially based on immunosuppression: it is imperative that novel immunomodulatory and antifibrotic agents be evaluated in multicentre treatment trials Pulmonary complications are an important extra-articular manifestation of connective tissue Disease (CTD) and a major cause of mortality. Here, the authors provide a broad overview of Interstitial Lung Disease in the context of CTD, providing insights into pathogenesis, classification and management of the Disease.
Sonye K Danoff - One of the best experts on this subject based on the ideXlab platform.
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Interstitial Lung Disease associated with the idiopathic inflammatory myopathies what progress has been made in the past 35 years
Chest, 2010Co-Authors: Geoffrey R Connors, Lisa Christopherstine, Chester V Oddis, Sonye K DanoffAbstract:Interstitial Lung Disease is commonly associated with the autoimmune inflammatory myopathies dermatomyositis and polymyositis and accounts for significant morbidity and mortality in these conditions. In the 35 years since the association between inflammatory myopathy and Interstitial Lung Disease was initially described, there has been progress in diagnosing and treating this dis-order. Nevertheless, there remains much about pathogenesis and therapeutics to be learned. This review examines the changes in the understanding of this complex condition, highlighting recent advances and areas deserving of further study.
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ascertainment of collagen vascular Disease in patients presenting with Interstitial Lung Disease
Respiratory Medicine, 2009Co-Authors: Shikha Mittoo, Lisa Christopherstine, Allan C Gelber, Maureen R Horton, Noah Lechtzin, Sonye K DanoffAbstract:Summary Introduction Previous studies of Interstitial Lung Disease (ILD) suggest that prognosis and therapeutic response are influenced by the presence of underlying collagen vascular Disease (CVD). Yet, what proportion of patients presenting with ILD have CVD is largely unknown. We sought to determine the frequency of a new CVD diagnosis in an ILD referral population. Materials/patients and methods We retrospectively studied 114 consecutive patients evaluated at the Johns Hopkins Interstitial Lung Disease Clinic for the development of CVD. Results In this retrospective cohort, nearly one-third of the 114 patients with confirmed ILD satisfied published criteria for a CVD diagnosis. Seventeen (15%) patients were diagnosed with a new CVD as a direct consequence of their ILD evaluation. Patients with new CVD diagnosis were younger than those without new CVD diagnosis: 51.4years (95% CI 45–58years) and 60years (95% CI 57–63), respectively ( p =0.01). Moreover, an ANA≥1:640 ( p =0.03) and elevated levels of creatine phosphokinase (CPK) or aldolase ( p Conclusions Unrecognized collagen vascular Disease may be more common than previously appreciated among patients referred with ILD. High titer ANA and an elevated CPK or aldolase are associated with a CVD diagnosis in this referral population.
