Home Contact Sitemap

eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease systemic scleroderma
Symptom C1619734|pulmonary arterial hypertension
Sentences 42
PubMedID- 23178295 Survival in incident systemic sclerosis patients with pulmonary arterial hypertension (pah).
PubMedID- 20044465 pulmonary arterial hypertension in systemic sclerosis: a distinctive endotheliopathy.
PubMedID- 20144930 pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class ii dyspnoea: mild symptoms but severe outcome.
PubMedID- 19955042 Objective: to assess the validation status of echocardiography with continuous doppler (echo-doppler) as an outcome measure in pulmonary arterial hypertension associated with systemic sclerosis (pah-ssc).
PubMedID- 22830207 Aim: to study survival of patients with a natural course of pulmonary arterial hypertension (pah) associated with systemic sclerosis (ss).
PubMedID- 21965635 Methods: studies using haq-di and/or sf-36 in patients with pulmonary arterial hypertension (pah) associated with systemic sclerosis (pah-ssc) were identified through a systematic literature review and assessed according to the outcome measures in rheumatology clinical trials (omeract) consensus group criteria.
PubMedID- 24992469 Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the reveal registry.
PubMedID- 20858146 Objectives: to describe the survival rate in a cohort of systemic sclerosis (ssc) patients with pulmonary arterial hypertension (pah) and to evaluate possible predictors for ssc-pah in a cohort of ssc patients.
PubMedID- 21888685 Microvascular disease is a prominent feature of systemic sclerosis (ssc) and leads to raynaud's phenomenon, pulmonary arterial hypertension, and scleroderma renal crisis.
PubMedID- 23812075 This is a rare case of rapidly progressive pulmonary arterial hypertension associated with systemic sclerosis that can be markedly improved with early diagnosis and treatment.
PubMedID- 20808962 Limited systemic sclerosis patients with pulmonary arterial hypertension show biomarkers of inflammation and vascular injury.
PubMedID- 20634241 Comparison of brain natriuretic peptide (bnp) and nt-probnp in screening for pulmonary arterial hypertension in patients with systemic sclerosis.
PubMedID- 25085432 Here we shows for the first time that an increase of nadph-derived reactive oxygen species production induced by sera from systemic sclerosis patients with pulmonary arterial hypertension drives collagen type i promoter activity in primary human pulmonary artery smooth muscle cells, suggesting that antioxidant-based therapies should be considered in the treatment of systemic sclerosis-associated vascular diseases.
PubMedID- 25596924 The performance characteristics of the three models for pah are presented in table 3.table 3comparison of the performance of detect versus esc/ers versus asig screening models for pulmonary arterial hypertension in patients with systemic sclerosispah prevalence set at 10%adetectesc/ersasigdetectesc/ersasign = 61n = 58n = 37n = 61n = 58n = 37positiveb49 (80.3)48 (82.8)25 (67.6)negativeb12 (19.7)10 (17.2)12 (32.4)true pah on rhcc27 (44.3)27 (46.55)15 (40.54)sensitivity100%96.3%100%100%96.3%100%(95% ci)(87.2-100)(81.0-99.9)(78.2-100)(54.1-100)(54.1-100)(39.8-100)specificity35.3%32.3%54.5%35.3%32.3%54.5%(95% ci)(19.7-53.5)(16.7-51.4)(32.2-75.6)(23.8-50.4)(15.6-41.0)(33.5-69.2)ppv55.1%55.3%60%14.7%13.6%19.6%(95% ci)(40.2-69.3)(40.1-69.8)(38.7-78.8)(5.6-29.2)(5.2-27.4)(5.7-43.7)npv100%90.9%100%100%98.7%100%(95% ci)(63.1-100)(58.7-99.8)(73.5-100)(83.2-100)(76.8-100)(80.5-100)arefer to additional file 1. bpositive or negative number screened by each of the algorithms.
PubMedID- 21119190 Early intervention in pulmonary arterial hypertension associated with systemic sclerosis: an essential component of disease management.
PubMedID- 25181620 Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis.
PubMedID- 23547391 There are data in the literature on the relationship of hyperuricemia with pulmonary arterial hypertension in patients with systemic sclerosis.
PubMedID- 22105781 pulmonary arterial hypertension associated with systemic sclerosis in the czech republic.
PubMedID- 22177106 The growing role of cardiac magnetic resonance imaging in assessment and follow-up of pulmonary arterial hypertension associated with systemic sclerosis.
PubMedID- 22130820 Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening.
PubMedID- 20576214 Objectives: pulmonary arterial hypertension in patients with systemic sclerosis is a disease involving multiple organ systems.
PubMedID- 21789477 pulmonary arterial hypertension (pah) when associated with systemic sclerosis (ssc) (ssc-pah) is one of the leading causes of mortality and is found in 10-15% of adult patients with ssc.
PubMedID- 26210131 Monitoring and diagnostic approaches for pulmonary arterial hypertension in patients with systemic sclerosis.
PubMedID- 24167351 Objectives: the aim of this study was to evaluate human leukocyte antigen (hla) involvement in the disease expression and poor prognostic clinical features (pulmonary fibrosis and pulmonary arterial hypertension) in patients diagnosed with systemic sclerosis (ssc) in a multiethnic population.
PubMedID- 19158116 Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis.
PubMedID- 20601197 pulmonary arterial hypertension in systemic sclerosis.
PubMedID- 21510736 pulmonary arterial hypertension associated with systemic sclerosis.
PubMedID- 23744060 Background: the impact of modern therapy on survival in pulmonary arterial hypertension (pah) associated with systemic sclerosis (ssc) is not clear.
PubMedID- 21769843 Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival.
PubMedID- 26324844 Objectives: despite the wide use of the 6 min walk distance (6mwd), no study has ever assessed its validity as a surrogate marker for haemodynamics and predictor of outcome in isolated pulmonary arterial hypertension associated with systemic sclerosis (ssc-pah).
PubMedID- 26320140 Application of the detect algorithm for detection of risk of pulmonary arterial hypertension in systemic sclerosis: data from a czech tertiary centre.
PubMedID- 21048365 Bosentan ameliorated exercise-induced pulmonary arterial hypertension complicated with systemic sclerosis.
PubMedID- 23687283 Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the detect study.
PubMedID- 22174212 pulmonary arterial hypertension in systemic sclerosis is associated with profound impairment of microvascular endothelium-dependent vasodilatation.
PubMedID- 26479414 Objective: to compare time to clinical worsening (ttcw) based on initial oral pah therapy in systemic sclerosis (ssc) patients with pulmonary arterial hypertension (pah).
PubMedID- 20598164 Background: pulmonary arterial hypertension (pah) in patients with systemic sclerosis is associated with a poor prognosis, but this can be improved by early disease detection.
PubMedID- 21816025 Prognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in thailand.
PubMedID- 21066870 Interstitial lung disease was diagnosed, accompanied by pulmonary arterial hypertension (pah) associated with systemic sclerosis.
PubMedID- 25372797 Clinical and laboratory characteristics of systemic sclerosis patients with pulmonary arterial hypertension in china.
PubMedID- 23671125 systemic sclerosis patients with and without pulmonary arterial hypertension: a nailfold capillaroscopy study.
PubMedID- 22084615 Bosentan is reported to have no effect on the six-minute walking distance in idiopathic pulmonary fibrosis,12 whereas prostacyclin derivatives improved six-minute walking distance test in systemic sclerosis with pulmonary arterial hypertension.12 these medications for pulmonary arterial hypertension did not improve cardiopulmonary function nor exercise capacity in our patient.
PubMedID- 21998119 Objective: the objective of this report is to compare baseline, management and survival characteristics in idiopathic pulmonary arterial hypertension (ipah) with systemic sclerosis-associated pulmonary arterial hypertension (ssc-apah) using data from the prospectively enrolled pah quality enhancement research initiative.

Page: 1