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Fractional exhaled nitric oxide in idiopathic pulmonary arterial hypertension and mixed connective tissue disease complicating pulmonary hypertension
BackgroundFractional exhaled nitric oxide (FeNO) has been extensively studied in various causes of pulmonary hypertension (PH), but its utility as a...
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Saliva Production and Esophageal Motility Influence Esophageal Acid Clearance Related to Post-reflux Swallow-Induced Peristaltic Wave
BackgroundThe post-reflux swallow-induced peristaltic wave (PSPW) brings salivary bicarbonate to neutralize residual distal esophageal mucosal...
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Pulmonary hypertension in connective tissue diseases: epidemiology, pathogenesis, and treatment
AbstractPulmonary hypertension (PH) is a clinical condition characterized by increased pulmonary arterial pressure arising from a heterogeneous range...
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Macitentan in Pulmonary Arterial Hypertension Associated with Connective Tissue Disease (CTD-PAH): Real-World Evidence from the Combined OPUS/OrPHeUS Dataset
IntroductionData on real-world clinical practice and outcomes of patients with pulmonary arterial hypertension associated with connective tissue...
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Retrospective cohort study of pulmonary arterial hypertension associated with connective tissue disease effect on patients’ prognosis
ObjectiveThe objectives of this study are to clarify clinical characteristics and recognize prognostic factors of CTD-PAH patients.
Methods ...
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Mean pulmonary artery pressure estimated by echocardiogram rapidly exceeds 20 mmHg from the normal range in patients with connective tissue disease
Earlier intervention for pulmonary hypertension (PH) has been reported to improve the prognosis of patients with connective tissue disease (CTD)....
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The enigma of mixed connective tissue disease—challenges in routine care
ObjectivesAs a rare and heterogeneous disease, mixed connective tissue disease (MCTD) represents a challenge. Herein, we aimed to unravel potential...
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Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay
Connective tissue diseases (CTDs) include a spectrum of disorders that affect the connective tissue of the human body; they include autoimmune...
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Diversity of hemodynamic types in connective tissue disease associated pulmonary hypertension: more than a subgroup of pulmonary arterial hypertension
ObjectiveConnective tissue disease associated pulmonary hypertension (CTD-PH) is classified as a subgroup of WHO group 1 PH, also called pulmonary...
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Refractory systemic lupus erythematosus with chylous effusion successfully treated with sirolimus: a case report and literature review
Chylous effusion is a rare manifestation of systemic lupus erythematosus (SLE). When it does occur in SLE, it is generally well treated with standard...
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Is pulmonary hypertension protective against cardiac tamponade? A systematic review
The presence of pulmonary hypertension (PH) may affect whether cardiac tamponade physiology develops from a pericardial effusion. Specifically, the...
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The impact of hospitalization on mortality in patients with connective tissue disease-associated interstitial lung disease: a medical records review study
BackgroundInterstitial lung disease (ILD) remains one of the most important causes of morbidity and mortality in patients with Connective Tissue...
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Survival in systemic sclerosis associated pulmonary arterial hypertension in the current treatment era—results from a nationwide study
IntroductionPulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). This nationwide study aims to describe...
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Reintroduction of immunosuppressive medications in pediatric rheumatology patients with histoplasmosis: a case series
BackgroundChildren with rheumatic diseases (cRD) receiving immunosuppressive medications (IM) are at a higher risk for acquiring potentially lethal...
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Managing Symptoms of Systemic Sclerosis for the Allergist-Immunologist
Purpose of ReviewSystemic sclerosis (SSc) is a chronic, multisystem, autoimmune disease characterized by fibrosis, vasculopathy and immune system...
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Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension
The etiology of pulmonary arterial hypertension (PAH) is complex, especially the investigation of rare pathogeny is difficult. Congenital...
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Interstitielle Lungenerkrankungen und andere progressive pulmonale Fibrosen
A wide selection of medicinal treatment options are currently available for the treatment of patients with interstitial lung diseases (ILD), so that...
