Targets of anti-endothelial cell antibodies in pulmonary hypertension and scleroderma

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Targets of anti-endothelial cell antibodies in pulmonary hypertension and scleroderma.

Anti-endothelial cell antibodies (AECAs) have been identified in patients with systemic sclerosis (SSc) with and without pulmonary arterial hypertension (PAH) and in patients with idiopathic pulmonary arterial hypertension (iPAH). However, their target antigens remain poorly identified. Sera from 24 patients with SSc without PAH, 20 patients with SSc with PAH, 30 with iPAH and 12 healthy contro...

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Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension.

The aim of the present study was to investigate the presence of anti-fibroblast antibodies in patients with idiopathic or scleroderma-associated pulmonary arterial hypertension (PAH) and healthy controls. PAH was documented by right-heart catheterisation (mean pulmonary artery pressure at rest >25 mmHg). Serum immunoglobulin (Ig)G and IgM reactivities of patients with idiopathic PAH (n = 35), s...

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Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension.

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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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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Antigenic targets and pathogenicity of anti-aortic endothelial cell antibodies in Takayasu arteritis.

OBJECTIVE Anti-endothelial cell antibodies are considered to have an important role in the pathogenesis of Takayasu arteritis (TA). Previously, these antibodies were detected using human umbilical vein endothelial cells, which do not completely represent the antigenicity/functions of aortic endothelial cells, the specific targets in TA. To delineate the precise role of antigenic targets, we inv...

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ژورنال

عنوان ژورنال: European Respiratory Journal

سال: 2011

ISSN: 0903-1936,1399-3003

DOI: 10.1183/09031936.00181410