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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease scleroderma
Symptom C0020542|pulmonary hypertension
Sentences 13
PubMedID- 20335527 The presence of the hla-b35 allele has emerged as an important risk factor for the development of isolated pulmonary hypertension in patients with scleroderma, however the mechanisms underlying this association have not been fully elucidated.
PubMedID- 26210782 Objective: to determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (ssc)-pulmonary arterial hypertension (pah) enrolled in the pulmonary hypertension assessment and recognition of outcomes in scleroderma (pharos) registry.
PubMedID- 22836630 The present study was aimed at testing the prevalence of anti-endothelial cell antibodies (aeca) in systemic scleroderma (ssc) patients with and without pulmonary hypertension (ph) and in relation to the presence of pulmonary fibrosis.
PubMedID- 23042670 Methods: we used data from 179 patients enrolled in the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry with pre-ph (defined by criteria on pulmonary function tests and/or echocardiogram) or definite ph with mean pulmonary artery pressure >/=25 mm hg by right-sided heart catheterization within 6 months of enrollment.
PubMedID- 21597395 Objective: to detect coronary artery disease in asymptomatic patients with systemic lupus erythematosus and scleroderma associated with pulmonary hypertension, and to determine whether it is focal or diffuse ischemia.
PubMedID- 20973920 Flow cytometry confirmed decreased expression of il-7r on circulating cd4+ t-cells from scleroderma patients with pulmonary hypertension.
PubMedID- 20862337 The prevalence of pulmonary hypertension associated with scleroderma ranged from 4.9% to 38% as written in the accp guidelines.
PubMedID- 23130095 2 subsequently, a second epoprostenol trial for patients with pulmonary hypertension associated with scleroderma resulted in fda approval for that condition.
PubMedID- 21844142 The multicenter pharos registry (pulmonary hypertension assessment and recognition of outcomes in scleroderma) prospectively follows subjects with ssc at high risk for or with incident pulmonary hypertension (ph).
PubMedID- 25356253 Gastric antral vascular ectasia (gave) has been reported very rarely in imatinib-treated gastrointestinal stromal tumor (gist) and scleroderma patients treated with imatinib for pulmonary hypertension.
PubMedID- 22549387 Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension.
PubMedID- 21548946 Furthermore, it has been shown that gdf-15 serum levels are increased in scleroderma patients with pulmonary hypertension and gdf-15 protein was predominantly located in monocytes infiltrating the lung tissue .
PubMedID- 23983198 Methods: the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry is a prospective registry of ssc patients at high risk for pah or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment.

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