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Management of interstitial lung disease associated with connective tissue disease

The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation (“lung dominant” CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome, and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD, such as idiopathic pulmonary fibrosis. Pulmonary hypertension (PH) can present as a primary vasculopathy in pulmonary arterial hypertension or in association with ILD (PH-ILD). Therefore, detailed history, physical examination, targeted serologic testing, and, occasionally, lung biopsy are needed to diagnose CTD-ILD, whereas both non-invasive and invasive assessments of pulmonary hemodynamics are needed to diagnose pulmonary hypertension. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. Furthermore, treatment strategies vary according to the clinical situation—for example, the treatment of a patient newly diagnosed as having CTD-ILD differs from that of someone with an acute exacerbation of the disease. Immunosuppression is indicated only in select cases of pulmonary arterial hypertension related to CTD; more commonly, selective pulmonary vasodilators are used. For both diseases, comorbidities such as sleep disordered breathing, symptoms of dyspnea, and cough should be evaluated and treated. Lung transplantation should be considered in patients with advanced disease but is not always feasible because of other manifestations of CTD and comorbidities. Clinical trials of novel therapies including immunosuppressive therapies are needed to inform best treatment strategies.  
feeds.bmj.com
over 3 years ago
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9

Management of interstitial lung disease associated with connective tissue disease

The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation (“lung dominant” CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome, and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD, such as idiopathic pulmonary fibrosis. Pulmonary hypertension (PH) can present as a primary vasculopathy in pulmonary arterial hypertension or in association with ILD (PH-ILD). Therefore, detailed history, physical examination, targeted serologic testing, and, occasionally, lung biopsy are needed to diagnose CTD-ILD, whereas both non-invasive and invasive assessments of pulmonary hemodynamics are needed to diagnose pulmonary hypertension. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. Furthermore, treatment strategies vary according to the clinical situation—for example, the treatment of a patient newly diagnosed as having CTD-ILD differs from that of someone with an acute exacerbation of the disease. Immunosuppression is indicated only in select cases of pulmonary arterial hypertension related to CTD; more commonly, selective pulmonary vasodilators are used. For both diseases, comorbidities such as sleep disordered breathing, symptoms of dyspnea, and cough should be evaluated and treated. Lung transplantation should be considered in patients with advanced disease but is not always feasible because of other manifestations of CTD and comorbidities. Clinical trials of novel therapies including immunosuppressive therapies are needed to inform best treatment strategies.  
feeds.bmj.com
over 3 years ago
Preview
0
0

Management of interstitial lung disease associated with connective tissue disease

The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation (“lung dominant” CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome, and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD, such as idiopathic pulmonary fibrosis. Pulmonary hypertension (PH) can present as a primary vasculopathy in pulmonary arterial hypertension or in association with ILD (PH-ILD). Therefore, detailed history, physical examination, targeted serologic testing, and, occasionally, lung biopsy are needed to diagnose CTD-ILD, whereas both non-invasive and invasive assessments of pulmonary hemodynamics are needed to diagnose pulmonary hypertension. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. Furthermore, treatment strategies vary according to the clinical situation—for example, the treatment of a patient newly diagnosed as having CTD-ILD differs from that of someone with an acute exacerbation of the disease. Immunosuppression is indicated only in select cases of pulmonary arterial hypertension related to CTD; more commonly, selective pulmonary vasodilators are used. For both diseases, comorbidities such as sleep disordered breathing, symptoms of dyspnea, and cough should be evaluated and treated. Lung transplantation should be considered in patients with advanced disease but is not always feasible because of other manifestations of CTD and comorbidities. Clinical trials of novel therapies including immunosuppressive therapies are needed to inform best treatment strategies.  
feeds.bmj.com
over 3 years ago
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0
2

Management of interstitial lung disease associated with connective tissue disease

The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation (“lung dominant” CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome, and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD, such as idiopathic pulmonary fibrosis. Pulmonary hypertension (PH) can present as a primary vasculopathy in pulmonary arterial hypertension or in association with ILD (PH-ILD). Therefore, detailed history, physical examination, targeted serologic testing, and, occasionally, lung biopsy are needed to diagnose CTD-ILD, whereas both non-invasive and invasive assessments of pulmonary hemodynamics are needed to diagnose pulmonary hypertension. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. Furthermore, treatment strategies vary according to the clinical situation—for example, the treatment of a patient newly diagnosed as having CTD-ILD differs from that of someone with an acute exacerbation of the disease. Immunosuppression is indicated only in select cases of pulmonary arterial hypertension related to CTD; more commonly, selective pulmonary vasodilators are used. For both diseases, comorbidities such as sleep disordered breathing, symptoms of dyspnea, and cough should be evaluated and treated. Lung transplantation should be considered in patients with advanced disease but is not always feasible because of other manifestations of CTD and comorbidities. Clinical trials of novel therapies including immunosuppressive therapies are needed to inform best treatment strategies.  
feeds.bmj.com
over 3 years ago
Www.bmj
0
0

Management of interstitial lung disease associated with connective tissue disease

The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation (“lung dominant” CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome, and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD, such as idiopathic pulmonary fibrosis. Pulmonary hypertension (PH) can present as a primary vasculopathy in pulmonary arterial hypertension or in association with ILD (PH-ILD). Therefore, detailed history, physical examination, targeted serologic testing, and, occasionally, lung biopsy are needed to diagnose CTD-ILD, whereas both non-invasive and invasive assessments of pulmonary hemodynamics are needed to diagnose pulmonary hypertension. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. Furthermore, treatment strategies vary according to the clinical situation—for example, the treatment of a patient newly diagnosed as having CTD-ILD differs from that of someone with an acute exacerbation of the disease. Immunosuppression is indicated only in select cases of pulmonary arterial hypertension related to CTD; more commonly, selective pulmonary vasodilators are used. For both diseases, comorbidities such as sleep disordered breathing, symptoms of dyspnea, and cough should be evaluated and treated. Lung transplantation should be considered in patients with advanced disease but is not always feasible because of other manifestations of CTD and comorbidities. Clinical trials of novel therapies including immunosuppressive therapies are needed to inform best treatment strategies.  
feeds.bmj.com
over 3 years ago
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0

Interstitial Lung Abnormalities Tied to Increased Mortality

Interstitial lung abnormalities were associated with increased risk for all-cause mortality, suggesting they might be mild or early idiopathic pulmonary fibrosis.  
medscape.com
over 3 years ago
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0

Interstitial lung abnormalities are linked to increased risk of death

Interstitial lung abnormalities—which are often undiagnosed and asymptomatic—are associated with a greater risk of all cause mortality, US research published in JAMA has shown.1  
feeds.bmj.com
over 3 years ago
Www.bmj
0
0

Interstitial lung abnormalities are linked to increased risk of death

Interstitial lung abnormalities—which are often undiagnosed and asymptomatic—are associated with a greater risk of all cause mortality, US research published in JAMA has shown.1  
feeds.bmj.com
over 3 years ago
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7

Imaging Case of the Week 190 Answer

The chest x-ray shows a linear density along the left lung field due to a skin fold, which can sometimes be mistaken for a pneumothorax.  
emergucate.com
over 3 years ago
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4

Cherry-Flavored e-Cigarettes Linked to Lung Irritant

Flavored e-cigarettes, particularly cherry-flavored, expose users to benzaldehyde, a compound that can irritate the airways, at levels often greater than those seen with conventional cigarettes.  
medscape.com
over 3 years ago
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2

Which antibiotics are best to treat worsening symptoms in people with cystic fibrosis with persistent Burkholderia cepacia complex lung infection? | Cochrane

We looked for evidence of which antibiotics are best to treat a flare up of symptoms in people with cystic fibrosis with persistent Burkholderia cepacia complex lung infection.  
cochrane.org
over 3 years ago
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2

Can smoking cessation interventions among adults with pulmonary tuberculosis improve their tuberculosis treatment outcomes? | Cochrane

Do treatments to help people with tuberculosis (TB) of the lungs to stop smoking also improve how they respond to treatment for their TB?  
cochrane.org
over 3 years ago
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Using samples obtained by bronchoscopy to decide how to treat lung infections in people with cystic fibrosis | Cochrane

We reviewed the evidence about whether to use samples obtained by bronchoscopy when deciding how to treat lung infections in people with cystic fibrosis.  
cochrane.org
over 3 years ago
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0

Does adding tiotropium, a long-acting muscarinic antagonist (LAMA), to combination therapy (LABA/ICS) help to control asthma? | Cochrane

Adding the LAMA tiotropium Respimat inhaler to combination LABA/ICS inhaler may reduce the need for rescue oral steroids. A noticeable benefit on quality of life is unlikely, and we couldn't tell if it reduced hospital admissions, but adding tiotropium has some benefit on lung function, asthma control, and non-serious side effects.  
cochrane.org
over 3 years ago
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2

Inhaled non-steroid anti-inflammatories (NSAIDs) for children and adults with bronchiectasis | Cochrane

People with bronchiectasis experience chronic inflammation of the lungs. Anti-inflammatory effects of inhaled non-steroid anti-inflammatory drugs (NSAIDs) may be beneficial for patients with bronchiectasis. However, short-term and long-term benefits in adults and children must be investigated, in addition to potential side effects of NSAIDs used over the long term.  
cochrane.org
over 3 years ago
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0

Prenatal Vitamin D Has a Weak Effect on Wheezing

Women did not significantly reduce the risk of wheezing in their children by taking vitamin D during their pregnancies in two new studies, but they did reduce the risk for some other lung problems.  
medscape.com
over 3 years ago
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Imaging Case of the Week 188 Answer

The chest x-ray exhibits a well circumscribed opacity in the left upper lobe with ‘popcorn’ calcification. This is a feature of pulmonary hamartoma, which is a benign neoplasm of the lung.  
emergucate.com
over 3 years ago
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LUNG US AND BNP

A small study in Ann Intensive Care reminds me that lung ultrasound is  good at detecting heart failure and differentiating against other causes of acute dyspnoea.  
scancrit.com
over 3 years ago
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0

Imaging Case of the Week 188

The following chest x-ray is from a 68 year old with history of chest pain. What abnormality can be seen in the left lung field?  
emergucate.com
over 3 years ago
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Imaging Case of the Week 187 Answer

The chest x-rays shows an expanded medial end of the right clavicle with irregular margins. The lung fields and rest of the bony skeleton appears normal.  
emergucate.com
over 3 years ago