eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | pulmonary arterial hypertension |
| Comorbidity | C0036421|systemic sclerosis |
| Sentences | 39 |
| PubMedID- 20144930 | pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class ii dyspnoea: mild symptoms but severe outcome. |
| PubMedID- 20634241 | Comparison of brain natriuretic peptide (bnp) and nt-probnp in screening for pulmonary arterial hypertension in patients with systemic sclerosis. |
| PubMedID- 23178295 | Survival in incident systemic sclerosis patients with pulmonary arterial hypertension (pah). |
| PubMedID- 21066870 | Interstitial lung disease was diagnosed, accompanied by pulmonary arterial hypertension (pah) associated with systemic sclerosis. |
| 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- 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- 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- 25181620 | Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis. |
| PubMedID- 20858146 | Survival in patients with pulmonary arterial hypertension associated with systemic sclerosis from a swedish single centre: prognosis still poor and prediction difficult. |
| PubMedID- 21510736 | pulmonary arterial hypertension associated with systemic sclerosis. |
| PubMedID- 22105781 | pulmonary arterial hypertension associated with systemic sclerosis in the czech republic. |
| 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- 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. |
| PubMedID- 26210131 | Monitoring and diagnostic approaches for pulmonary arterial hypertension in patients 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- 21816025 | Prognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in thailand. |
| PubMedID- 23687283 | Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the detect study. |
| PubMedID- 22830207 | [survival of patients with pulmonary arterial hypertension, associated with systemic sclerosis]. |
| PubMedID- 21119190 | Early intervention in pulmonary arterial hypertension associated with systemic sclerosis: an essential component of disease management. |
| PubMedID- 20808962 | Limited systemic sclerosis patients with pulmonary arterial hypertension show biomarkers of inflammation and vascular injury. |
| PubMedID- 21769843 | Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. |
| 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- 22177106 | The growing role of cardiac magnetic resonance imaging in assessment and follow-up of pulmonary arterial hypertension associated with systemic sclerosis. |
| PubMedID- 21048365 | Bosentan ameliorated exercise-induced pulmonary arterial hypertension complicated with systemic sclerosis. |
| 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- 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- 23744060 | Background: the impact of modern therapy on survival in pulmonary arterial hypertension (pah) associated with systemic sclerosis (ssc) is not clear. |
| 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- 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- 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- 20044465 | pulmonary arterial hypertension in systemic sclerosis: a distinctive endotheliopathy. |
| 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- 22174212 | pulmonary arterial hypertension in systemic sclerosis is associated with profound impairment of microvascular endothelium-dependent vasodilatation. |
| PubMedID- 21492463 | Introduction: systemic sclerosis (ssc) complicated by pulmonary arterial hypertension (pah) carries a poor prognosis, despite pulmonary vascular dilating therapy. |
| PubMedID- 22130820 | Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening. |
| PubMedID- 19158116 | Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis. |
| PubMedID- 20601197 | pulmonary arterial hypertension in systemic sclerosis. |
| PubMedID- 20576214 | Objectives: pulmonary arterial hypertension in patients with systemic sclerosis is a disease involving multiple organ systems. |
| PubMedID- 23671125 | systemic sclerosis patients with and without pulmonary arterial hypertension: a nailfold capillaroscopy study. |
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