Cardiac Autoantibodies from Patients Affected by a New Variant of Endemic Pemphigus Foliaceus in Colombia, South America
Cardiac Autoantibodies from Patients Affected by a New Variant of Endemic Pemphigus Foliaceus in Colombia, South America
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DOI：
10.1007/s10875-011-9574-y
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发表时间：
2011-12-01
影响因子：
9.1
通讯作者：
Dudley, Samuel C., Jr.
中科院分区：
文献类型：
--
作者：
Abreu-Velez, Ana Maria;Howard, Michael S.;Dudley, Samuel C., Jr.
在哥伦比亚巴格雷（El Bagre- epf），一些受地方性叶状天疱疮新变种影响的患者经历了猝死综合征，包括50岁以下的人。El Bagre-EPF患者与副肿瘤性天疱疮患者具有一些相同的自身抗原，例如对斑块的反应性。此外，副肿瘤天疱疮患者有心脏自身抗体。因此，我们利用心脏提取物作为抗原，使用直接和间接免疫荧光、共聚焦显微镜、免疫组织化学、免疫印迹和免疫电子显微镜检测了来自流行地区的15例El bagrei - epf患者和15例对照者对心脏组织的自身反应性。我们发现15例El Bagre患者中有7例对心脏的几个细胞连接表现出多克隆免疫反应，通常与已知标记共定位。这些共定位标记包括心脏组成区域的标记，如抗desmoplakins I和II；间隙连接的标记，如connexin 43；紧密连接标记，如ezrin和连接粘附分子A；并粘附连接点，如泛钙粘蛋白。我们还在血管、浦肯野纤维和心肌肌瘤中检测到患者抗体的共定位。我们得出结论，El bagrei - epf患者对多个心脏表位表现出自身反应，这种疾病可能类似于风湿性心炎患者的情况，此外，这种疾病的心脏病理生理学值得进一步评估。
Several patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF) have experienced a sudden death syndrome, including persons below the age of 50. El Bagre-EPF patients share several autoantigens with paraneoplastic pemphigus patients, such as reactivity to plakins. Further, paraneoplastic pemphigus patients have autoantibodies to the heart. Therefore, we tested 15 El Bagre-EPF patients and 15 controls from the endemic area for autoreactivity to heart tissue using direct and indirect immunofluorescence, confocal microscopy, immunohistochemistry, immunoblotting, and immunoelectron microscopy utilizing heart extracts as antigens. We found that 7 of 15 El Bagre patients exhibited a polyclonal immune response to several cell junctions of the heart, often colocalizing with known markers. These colocalizing markers included those for the area composita of the heart, such as anti-desmoplakins I and II; markers for gap junctions, such as connexin 43; markers for tight junctions, such as ezrin and junctional adhesion molecule A; and adherens junctions, such pan-cadherin. We also detected colocalization of the patient antibodies within blood vessels, Purkinje fibers, and cardiac sarcomeres. We conclude that El Bagre-EPF patients display autoreactivity to multiple cardiac epitopes, that this disease may resemble what is found in patients with rheumatic carditis, and further, that the cardiac pathophysiology of this disorder warrants further evaluation.