eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | systemic scleroderma |
| Comorbidity | C0020538|hypertension |
| Sentences | 68 |
| 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- 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- 20722025 | Exercise-induced pulmonary hypertension associated with systemic sclerosis: four distinct entities. |
| 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- 23042670 | Dyspnea assessment and pulmonary hypertension in patients with systemic sclerosis: utility of the university of california, san diego, shortness of breath questionnaire. |
| 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- 26210131 | Monitoring and diagnostic approaches for pulmonary arterial hypertension in patients with systemic sclerosis. |
| PubMedID- 21906161 | Echo doppler predictors of pulmonary artery hypertension in patients with systemic sclerosis. |
| PubMedID- 23740875 | Submaximal heart and pulmonary evaluation: a novel noninvasive test to identify pulmonary hypertension in patients 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- 21547892 | Objective: to develop a score to estimate the risk of developing pulmonary hypertension (ph) in patients with systemic sclerosis (ssc). |
| PubMedID- 25332860 | Potential effect of sildenafil beyond pulmonary hypertension in a patient with diffuse systemic sclerosis and cryoglobulinemic vasculitis. |
| PubMedID- 20526641 | Early detection of pulmonary hypertension (ph) in patients with systemic sclerosis (ssc) is essential as it leads to substantial morbidity and mortality irrespective of its etiology. |
| PubMedID- 26489821 | Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient. |
| PubMedID- 25181620 | Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis. |
| PubMedID- 22177106 | The growing role of cardiac magnetic resonance imaging in assessment and follow-up of pulmonary arterial 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- 22105781 | Pulmonary arterial hypertension associated with systemic sclerosis in the czech republic. |
| PubMedID- 22523431 | Fra-2 transgenic mice as a novel model of pulmonary hypertension associated with systemic sclerosis. |
| PubMedID- 21119190 | Early intervention in pulmonary arterial hypertension associated with systemic sclerosis: an essential component of disease management. |
| PubMedID- 22830207 | [survival of patients with pulmonary arterial hypertension, associated with systemic sclerosis]. |
| PubMedID- 20598164 | Echocardiography may help detect pulmonary vasculopathy in the early stages of pulmonary artery hypertension associated with systemic sclerosis. |
| PubMedID- 22130821 | Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus. |
| PubMedID- 21769843 | Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. |
| PubMedID- 25104236 | Influence of pulmonary vascular reserve on exercise-induced pulmonary hypertension in patients with 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- 24949025 | Described a higher frequency of gstm1 and gstt1-null genotypes (especially gstt1-nulls) among systemic sclerosis patients with hypertension and pulmonary involvement. |
| PubMedID- 23499807 | N-terminal pro-brain natriuretic peptide (nt-probnp), a pro-hormone secreted by the myocardium in response to various stimuli, was found to be correlated with several hemodynamic parameters in pulmonary hypertension associated with systemic sclerosis. |
| PubMedID- 20144930 | Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class ii dyspnoea: mild symptoms but severe outcome. |
| 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- 22001478 | We present the first documented case of thrombotic thrombocytopenic purpura (ttp) with severe hypertension complicated by polymyositis and systemic sclerosis sine scleroderma. |
| PubMedID- 26402988 | Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in systemic sclerosis. |
| PubMedID- 25036561 | Incidence of pulmonary hypertension in patients with systemic sclerosis and no pulmonary symptoms: is annual echocardiographic screening necessary. |
| PubMedID- 23292818 | Epidemiology and risk factors for pulmonary hypertension in systemic sclerosis. |
| PubMedID- 20382500 | Conclusion: hrct-determined severity and extent of pulmonary fibrosis may be helpful in screening for pulmonary hypertension in patients with systemic sclerosis. |
| PubMedID- 19158116 | Overlap of coronary disease and pulmonary arterial hypertension in 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- 21670950 | systemic sclerosis (ssc) is complicated by pulmonary hypertension and right ventricle (rv) failure in approximately 10% of the patients. |
| PubMedID- 20425528 | Diagnosis and management of pulmonary hypertension in 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- 19101049 | Pulmonary hypertension in systemic sclerosis determines cardiac autonomic dysfunction assessed by heart rate turbulence. |
| PubMedID- 23283858 | Pulmonary artery hypertension associated with systemic scleroderma: correlation among lung pulmonary blood volume, lung perfusion single photon emission computed tomography, and chest computed tomography images. |
| 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- 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- 20576214 | Objectives: pulmonary arterial hypertension in patients with systemic sclerosis is a disease involving multiple organ systems. |
| 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- 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- 20634241 | Comparison of brain natriuretic peptide (bnp) and nt-probnp in screening for pulmonary arterial hypertension in patients with systemic sclerosis. |
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