Articles: connective-tissue-diseases.
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Am. J. Respir. Crit. Care Med. · Nov 2015
Randomized Controlled Trial Multicenter Study Comparative StudyComparison of Treatment Response in Idiopathic and Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.
Studies suggest that patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) have a poorer treatment response to therapies for PAH compared with patients with idiopathic PAH (IPAH), but individual randomized controlled trials (RCTs) have been underpowered to examine differences within these subgroups. ⋯ Treatment for PAH was less effective in CTD-PAH compared with IPAH in terms of increasing 6MWD and preventing clinical worsening. The heterogeneity of treatment response supports the need for identifying therapies that are more effective for CTD-PAH.
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Multicenter Study
Predictors of mortality in patients with interstitial lung disease treated with corticosteroids: results from a cohort study.
Interstitial lung disease (ILD) has a heterogeneous clinical presentation and establishing prognosis for these patients is challenging. We investigated the clinical characteristics and outcome of patients with idiopathic interstitial pneumonias (IIPs) and patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). We conducted a multicenter prospective study on 104 patients diagnosed with IIPs and 29 patients diagnosed with CTD-ILD, which were newly diagnosed and treated with corticosteroids initially. ⋯ Significant predictors for mortality in patients with IIPs at baseline were lower for performance status and definite usual interstitial pattern (UIP) on HRCT. Patients with UIP experienced worse survival than those with non-UIP. A definite UIP on HRCT and lower baseline performance status have important prognostic implications in patients with IIPs.
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Heart, lung & circulation · Aug 2014
Multicenter Study Clinical TrialPrevalence of venous thromboembolic events and diagnostic performance of the wells score and revised geneva scores for pulmonary embolism in patients with interstitial lung disease: a prospective study.
To determine the prevalence of venous thromboembolism (VTE) in patients with connective tissue disease-related interstitial lung diseases (CTD-ILD) and idiopathic interstitial pneumonia (IIP) and to further evaluate associated risk factors. To also examine the diagnostic performance of the Wells score and the revised Geneva scores for diagnosing pulmonary embolism (PE) in ILD patients. ⋯ VTE can be seen in approximately one fourth of patients with CTD-ILD or IIP and the Wells score and the revised Geneva score can be used for categorising PE risk.
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Arthritis Res. Ther. · Oct 2012
Multicenter StudyPredictors of mortality in connective tissue disease-associated pulmonary arterial hypertension: a cohort study.
Pulmonary arterial hypertension (PAH) is a major cause of mortality in connective tissue disease (CTD). We sought to quantify survival and determine factors predictive of mortality in a cohort of patients with CTD-associated PAH (CTD-PAH) in the current era of advanced PAH therapy. ⋯ In this cohort of CTD-PAH patients, three-year survival was 73%. Independent therapeutic predictors of survival included warfarin and combination PAH therapy. Our findings suggest that anticoagulation and combination PAH therapy may improve survival in CTD-PAH. This observation merits further evaluation in randomised controlled trials.
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Arthritis and rheumatism · Jan 2011
Multicenter StudyCombined pulmonary fibrosis and emphysema syndrome in connective tissue disease.
Connective tissue diseases (CTDs) are associated with several interstitial lung diseases. The aim of this study was to describe the recently individualized syndrome of combined pulmonary fibrosis and emphysema (CPFE) in a population of patients with CTD. ⋯ CPFE warrants inclusion as a novel, distinct pulmonary manifestation within the spectrum of CTD-associated lung diseases in smokers or former smokers, especially in patients with RA or SSc.