eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | systemic scleroderma |
| Symptom | C0020538|hypertension |
| Sentences | 70 |
| PubMedID- 24949025 | Described a higher frequency of gstm1 and gstt1-null genotypes (especially gstt1-nulls) among systemic sclerosis patients with hypertension and pulmonary involvement. |
| 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- 22130821 | Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus. |
| PubMedID- 25284845 | Another report mentions the efficacy of infliximab treatment in a patient with lung fibrosis and pulmonary hypertension associated with advanced systemic sclerosis, refractory to conventional therapies. |
| PubMedID- 20658290 | Significance of serum uric acid in pulmonary hypertension due to systemic sclerosis: a pilot study. |
| PubMedID- 23292818 | Epidemiology and risk factors for pulmonary hypertension in systemic sclerosis. |
| PubMedID- 21047671 | Pulmonary hypertension in systemic sclerosis. |
| PubMedID- 23372926 | Fra-2 transgenic mice as a novel model of pulmonary hypertension associated with systemic sclerosis. |
| PubMedID- 23740875 | Submaximal heart and pulmonary evaluation: a novel noninvasive test to identify pulmonary hypertension in patients with systemic sclerosis. |
| PubMedID- 20808962 | Limited systemic sclerosis patients with pulmonary arterial hypertension show biomarkers of inflammation and vascular injury. |
| PubMedID- 22001478 | Thrombotic thrombocytopenic purpura with severe hypertension in a patient with systemic sclerosis sine scleroderma and polymyositis. |
| PubMedID- 23812075 | Rapidly progressive pulmonary arterial hypertension associated with systemic sclerosis : a case report. |
| PubMedID- 25705389 | A retrospective study of 70 systemic sclerosis patients with ild-associated pulmonary hypertension was unable to demonstrate any clear benefit from pah-specific therapy. |
| PubMedID- 23178295 | Survival in incident systemic sclerosis patients with pulmonary arterial hypertension (pah). |
| PubMedID- 23910607 | Pulmonary hypertension in systemic sclerosis: prevalence, incidence and predictive factors in a large multicentric italian cohort. |
| PubMedID- 25332860 | Potential effect of sildenafil beyond pulmonary hypertension in a patient with diffuse systemic sclerosis and cryoglobulinemic vasculitis. |
| PubMedID- 24992469 | Background: patients with pulmonary arterial hypertension (pah) associated with systemic sclerosis (ssc-apah) experience higher mortality rates than patients with idiopathic disease and those with other connective tissue diseases (ctd-apah). |
| PubMedID- 21048365 | Bosentan ameliorated exercise-induced pulmonary arterial hypertension complicated with systemic sclerosis. |
| 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- 22177106 | The growing role of cardiac magnetic resonance imaging in assessment and follow-up of 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- 21769843 | Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. |
| PubMedID- 21510736 | Pulmonary arterial hypertension associated with systemic sclerosis. |
| PubMedID- 20601197 | Pulmonary arterial hypertension in systemic sclerosis. |
| PubMedID- 22518163 | Accurate blood pressure (bp) measurement is essential to the diagnosis and management of hypertension in patients with systemic sclerosis (ssc) to help prevent renal and cardiovascular complications. |
| 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- 22830207 | Survival of patients with pulmonary arterial hypertension, associated with systemic sclerosis. |
| PubMedID- 19101049 | Pulmonary hypertension in systemic sclerosis determines cardiac autonomic dysfunction assessed by heart rate turbulence. |
| PubMedID- 26402988 | Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in 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- 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- 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- 26320140 | Application of the detect algorithm for detection of risk of pulmonary arterial hypertension in systemic sclerosis: data from a czech tertiary centre. |
| 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- 19158116 | Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis. |
| PubMedID- 22174212 | Pulmonary arterial hypertension in systemic sclerosis is associated with profound impairment of microvascular endothelium-dependent vasodilatation. |
| PubMedID- 25146407 | Objective: to study the clinical, cardiopulmonary functional and hemodynamic profiles of systemic sclerosis patients with pulmonary hypertension (ssc-pah) compared with those of idiopathic pulmonary hypertension (ipah). |
| 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- 25104236 | Influence of pulmonary vascular reserve on exercise-induced pulmonary hypertension in patients with systemic sclerosis. |
| PubMedID- 22523431 | Fra-2 transgenic mice as a novel model of pulmonary hypertension associated with systemic sclerosis. |
| PubMedID- 21816025 | Prognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in thailand. |
| PubMedID- 20576214 | Objectives: pulmonary arterial hypertension in patients with systemic sclerosis is a disease involving multiple organ systems. |
| PubMedID- 25372797 | Clinical and laboratory characteristics of systemic sclerosis patients with pulmonary arterial hypertension in china. |
| 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- 20634241 | Comparison of brain natriuretic peptide (bnp) and nt-probnp in screening for pulmonary arterial hypertension in patients with systemic sclerosis. |
| PubMedID- 23744060 | Survival in systemic sclerosis with pulmonary arterial hypertension has not improved in the modern era. |
| PubMedID- 26210131 | Monitoring and diagnostic approaches for pulmonary arterial hypertension in patients with systemic sclerosis. |
| 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- 26489821 | Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient. |
| PubMedID- 20722025 | Exercise-induced pulmonary hypertension associated with systemic sclerosis: four distinct entities. |
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