Publications by authors named "Elisabeth Bendstrup"

Background: Systemic juvenile idiopathic arthritis-related lung disease (sJIA-LD) is a severe complication in patients with treatment-refractory systemic juvenile idiopathic arthritis (sJIA). The objective of this study was to evaluate the effect of allogeneic haematopoietic stem-cell transplantation (HSCT) in a cohort of children with sJIA-LD.

Methods: This international, retrospective cohort study was performed in nine hospitals across the USA and Europe in children with sJIA-LD who had received allogeneic HSCT.

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Background: Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.

Aim: To characterize chILD-survivors and identify their impact on adult-ILD centers.

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Article Synopsis
  • - The study explored the risk factors and consequences of acute exacerbations in patients with progressive fibrosing interstitial lung diseases (ILDs), using data from the INBUILD trial, which involved treatments with nintedanib versus placebo.
  • - Results showed that 8.7% of patients experienced acute exacerbations, with lower lung function and older age being significant risk factors, while nintedanib treatment seemed to reduce the risk of these events.
  • - The analysis indicated that acute exacerbations are linked to high mortality rates, with approximately 19% of patients at risk of death within 30 days following such events.
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Background: Quality of life is impaired in patients with sarcoidosis. The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing health-related quality of life in patients with sarcoidosis, comprising subdomains of General Health Status (GHS), Lung, Medication, Skin and Eyes. The aim of this study was to enhance the validation of the KSQ, incorporating longitudinal validation and known-groups validity in a cohort with mild sarcoidosis.

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Interstitial lung disease (ILD) is a relevant cause of morbidity and mortality in patients with autoimmune rheumatic diseases (ARDs). In the last years, an acute exacerbation (AE) - defined as an acute, clinically significant respiratory deterioration characterized by evidence of new widespread alveolar abnormality - has been reported to occur in virtually all ILD types, including ARD-ILD. The aim of this review is to describe the available and investigational treatments in patients affected by AE-ARD-ILD in light of the very low quality of evidence available.

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Interstitial lung disease (ILD) is a frequent manifestation of rheumatoid arthritis (RA) that is associated with high mortality. RA-ILD may initially be asymptomatic, and lung function may be markedly impaired by the time it is diagnosed. The course of RA-ILD is highly variable, with some patients experiencing no discernable progression or a slow decline, whereas others experience more rapid deterioration.

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Article Synopsis
  • Pulmonary alveolar proteinosis (PAP) is a rare lung syndrome characterized by the accumulation of proteinaceous material, leading to symptoms like progressive dyspnea and hypoxemia, with various diagnostic methods such as CT scans, bronchoalveolar lavage, and genetic testing suggested for evaluation.
  • A European Respiratory Society Task Force, comprised of diverse experts, developed evidence-based guidelines for diagnosing and managing PAP using a systematic review of literature and the GRADE approach for assessing the strength of recommendations.
  • The Task Force provided specific management recommendations, including whole lung lavage, GM-CSF therapy, and potential treatments like rituximab, alongside diagnostic approaches involving GM-CSF antibody
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Introduction: Pulmonary lymphangitis carcinomatosa is a rare and severe manifestation of metastatic disease that causes pulmonary symptoms and radiologic patterns similar to interstitial lung diseases.

Case Presentation: We report a case of a 78-year-old woman who presented to our department with insidiously developed symptoms of fatigue, dry cough, and severe dyspnea for 3 months. Chest radiography showed bilateral interstitial changes.

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Background: Respirable crystalline silica is a well-known cause of silicosis but may also be associated with other types of interstitial lung disease. We examined the associations between occupational exposure to respirable crystalline silica and the risk of idiopathic interstitial pneumonias, pulmonary sarcoidosis and silicosis.

Methods: The total Danish working population was followed 1977-2015.

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Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis in the lungs. Activated fibroblasts play a central role in fibrogenesis and express fibroblast activation protein α. A truncated, soluble form (sFAP) can be measured in blood and is a potential novel biomarker of disease activity.

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Background: Organic dust is associated with hypersensitivity pneumonitis, and associations with other types of interstitial lung disease (ILD) have been suggested. We examined the association between occupational organic dust exposure and hypersensitivity pneumonitis and other ILDs in a cohort study.

Methods: The study population included all residents of Denmark born in 1956 or later with at least 1 year of gainful employment since 1976.

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In this review, we discuss a new definition and treatment options of allergic alveolitis (AA). AA is an immune-mediated interstitial lung disease triggered by inhaled antigens, it is defined as non-fibrotic (inflammatory) and/or fibrotic, and diagnosis relies on a multidisciplinary approach using clinical, radiological and sometimes histological assessments. Treatment involves early antigen elimination and may include corticosteroids or other immunosuppressants.

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Objectives: The association between asbestos exposure and asbestosis in high-exposed industrial cohorts is well-known, but there is a lack of knowledge about the exposure-response relationship for asbestosis in a general working population setting. We examined the exposure-response relationship between occupational asbestos exposure and asbestosis in asbestos-exposed workers of the Danish general working population.

Methods: We followed all asbestos-exposed workers from 1979 to 2015 and identified incident cases of asbestosis using the Danish National Patient Register.

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Systemic Lupus Erythematosus (SLE) is a multifaceted, multisystem autoimmune disorder with diverse clinical expressions. While prevalence reports vary widely, pulmonary involvement accounts for significant morbidity and mortality in SLE. This comprehensive review explores the spectrum of pulmonary disease in SLE, including upper airway manifestations (e.

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Lymphangioleiomyomatosis (LAM) is a rare lung disease predominantly affecting women, and it is characterized by the proliferation of smooth muscle cells and cystic lung destruction. LAM diagnosis is challenging due to varied clinical presentations and resemblance to common conditions such as asthma. We present two female cases where LAM was initially misdiagnosed.

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Chronic Obstructive Pulmonary Disease (COPD) exacerbation is known for its substantial impact on morbidity and mortality among affected patients, creating a significant healthcare burden worldwide. Coagulation abnormalities have emerged as potential contributors to exacerbation pathogenesis, raising concerns about increased thrombotic events during exacerbation. The aim of this study was to explore the differences in thrombelastography (TEG) parameters and coagulation markers in COPD patients during admission with exacerbation and at a follow-up after discharge.

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Article Synopsis
  • SpO measured at the fingers in patients with systemic sclerosis (SSc) can be inaccurate due to conditions like Raynaud's phenomenon, making more central body sensor locations (like the forehead and earlobe) preferable.
  • A study assessing the reliability of SpO measurements during the 6-Minute Walk Test (6MWT) involved 82 SSc patients and found significant differences in accuracy, with earlobe and forehead sensors performing better than finger sensors.
  • The study revealed the finger sensor had the lowest re-test reliability, suggesting that earlobe or forehead sensors should be used for a more accurate assessment of oxygen saturation in SSc patients during physical tests.
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Background: Many patients with chronic obstructive pulmonary disease and fibrotic interstitial lung disease suffer from severe dyspnea and reduced quality of life, despite receiving optimal disease-modifying treatment for their illness. Studies have suggested that these patients may benefit from treatment with low-dose opioids. However, many patients decline opioid treatment.

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Article Synopsis
  • Interstitial lung abnormalities (ILA) are specific findings on CT scans of patients not suspected to have interstitial lung disease (ILD).
  • If ILA shows signs of fibrosis in more than 5% of the lungs, patients should see a pulmonologist for further evaluation.
  • Early detection of ILA and understanding the associated risks can help prevent progression to ILD and improve survival rates.
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Neurosarcoidosis is a rare and serious condition. Rapid diagnosis and treatment are crucial to prevent morbidity and mortality. When neurological symptoms are not present at the time of diagnosis, CNS involvement can be undetected.

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Usual Interstitial Pneumonia (UIP) is characterized by progression of lung parenchyma that may be observed in various autoimmune rheumatic diseases (ARDs), including rheumatoid arthritis and connective tissue diseases. From a diagnostic point of view, a UIP pattern related to ARDs may display imaging and pathological features able to distinguish it from that related to IPF, such as the "straight-edge" sign at HRCT and lymphoplasmacytic infiltrates at histologic specimens. Multidisciplinary approach (MDD), involving at least pulmonologist, rheumatologist and radiologist, is fundamental in the differential diagnosis process, but MDD is also required in the evaluation of severity, progression and response to treatment, that is based on the combination of changes in symptoms, pulmonary function trends, and, in selected patients, serial CT evaluation.

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Objectives: Endobronchial valve (EBV) treatment is a treatment option for selected patients with severe chronic obstructive pulmonary disease (COPD) to reduce dyspnea and improve quality of life. However, the procedure is associated with risk of complications, and not all patients achieve the expected outcomes. The present study explores patients' expectations while waiting for EBV treatment.

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Background And Objective: Surrogate endpoints enable determination of meaningful treatment effects more efficiently than applying the endpoint of ultimate interest. We used data from trials of nintedanib in subjects with pulmonary fibrosis to assess decline in forced vital capacity (FVC) as a surrogate for mortality.

Methods: Data from the nintedanib and placebo groups of trials in subjects with idiopathic pulmonary fibrosis, other forms of progressive pulmonary fibrosis, and pulmonary fibrosis due to systemic sclerosis (NCT00514683, NCT01335464, NCT01335477, NCT01979952, NCT02999178, NCT02597933) were pooled.

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