Prevalence of anti-endothelial cell antibodies in idiopathic pulmonary arterial hypertension
نویسندگان
چکیده
منابع مشابه
Prevalence of anti-endothelial cell antibodies in idiopathic pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is a rare disease often resulting in right-sided heart failure and premature death [1]. PAH is idiopathic (IPAH), heritable, or related to conditions such as connective tissue diseases (CTD) [2]. IPAH prognosis remains poor despite improved patient survival with current treatment options. Therefore, elucidating the pathophysiology of IPAH is important for t...
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BACKGROUND It has previously been shown that IgG antibodies from patients with limited cutaneous systemic sclerosis (SSc) bind to specific microvascular endothelial cell antigens. Since patients with limited cutaneous SSc are prone to develop pulmonary arterial hypertension (PAH), and since endothelial cell activation is involved in the pathogenesis of idiopathic PAH (IPAH), a study was underta...
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IN THIS ISSUE OF AJP-LUNG, Masri and colleagues (11) report that pulmonary artery endothelial cells (PAECs) isolated from patients with idiopathic pulmonary arterial hypertension (IPAH) exhibit an unusual hyperproliferative potential, with decreased susceptibility to apoptosis. Interestingly, although the PAECs from IPAH patients grew rapidly when placed in Matrigel, they generated disorganized...
متن کاملCirculating plasmablasts are elevated and produce pathogenic anti-endothelial cell autoantibodies in idiopathic pulmonary arterial hypertension.
Idiopathic pulmonary arterial hypertension (IPAH) is a devastating pulmonary vascular disease in which autoimmune and inflammatory phenomena are implicated. B cells and autoantibodies have been associated with IPAH and identified as potential therapeutic targets. However, the specific populations of B cells involved and their roles in disease pathogenesis are not clearly defined. We aimed to as...
متن کاملidiopathic pulmonary arterial hypertension
cytometry analysis of BAL cells. Table 1 integrates information given in our published paper, clearly demonstrating that the number of BAL neutrophils was fair in our case series. This unfortunately prevented a definitive evaluation of whether polymorph nucleates represent a source of IL-17. Nonetheless, as shown in figure 1, in selected cases with a significant number of BAL neutrophils (two s...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2010
ISSN: 0903-1936,1399-3003
DOI: 10.1183/09031936.00164209