AUTOANTIBODIES IN PATIENTS WITH PRIMARY PULMONARY-HYPERTENSION - ASSOCIATION WITH ANTI-KU
AUTOANTIBODIES IN PATIENTS WITH PRIMARY PULMONARY-HYPERTENSION - ASSOCIATION WITH ANTI-KU
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DOI：
10.1016/0002-9343(92)90238-7
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发表时间：
1992-09-01
影响因子：
5.9
通讯作者：
ARNETT, FC
中科院分区：
文献类型：
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作者：
ISERN, RA;YANEVA, M;ARNETT, FC
目得：原发性肺动脉高压（PPH）患者常伴有雷诺现象、血清抗核抗体（ANA）和/或肺血管病变，与某些结缔组织疾病（尤其是硬皮病）相似。 在结缔组织疾病中已经描述了一些相对疾病特异性的自身抗体，但尚未在PPH患者中进行研究。因此，研究了PPH患者血清中的各种自身抗体，寻找这种肺部疾病和结缔组织疾病之间的可能联系。患者和方法：研究了来自31名PPH患者和24名继发性肺动脉高压（SPH）患者的血清中的以下自身抗体：抗着丝点（Hep-2细胞的间接免疫荧光），通过免疫印迹和使用克隆的CENP-B融合蛋白的酶免疫测定（EIA）的抗CENP-B，抗拓扑异构酶I（Scl-70），结果：PPH组抗Ku抗体阳性率为23%，SPH组为4%，正常对照组为0（PPH与SPH，p = 0.06; PPH与对照组，p = 0.01）。 CENP-B抗体在PPH和SPH各1例患者中发现，抗拓扑异构酶I在SPH 1例患者中发现，抗Ro（SS-A）和La（SS-B）在PPH 1例患者中发现。 总体而言，12例PPH患者（39%）雷诺现象或阳性ANA结果，9（29%）有更多的特异性自身抗体与结缔组织diseases.CONCLUSIONS：这些结果进一步表明至少一个亚组的PPH患者和自身免疫性结缔组织疾病之间的联系，抗Ku抗体是一种可能的新的血清学标志物。
PURPOSE: Patients with primary pulmonary hypertension (PPH) frequently have Raynaud's phenomenon, serum antinuclear antibodies (ANAs), and/or pulmonary vascular lesions similar to those seen in certain connective tissue diseases, especially scleroderma. A number of relatively disease-specific autoantibodies have been described in connective tissue diseases but have not been studied in patients with PPH. Therefore, sera from PPH patients were studied for a variety of autoantibodies, seeking a possible link between this pulmonary disorder and connective tissue diseases.PATIENTS AND METHODS: Sera from 31 patients with PPH and 24 with secondary pulmonary hypertension (SPH) were studied for the following autoantibodies: anti-centromere (indirect immunofluorescence of Hep-2 cells), anti-CENP-B by immunoblotting and enzyme immunoassay (EIA) using cloned CENP-B fusion protein, anti-topoisomerase I(Scl-70), anti-Ku using immunoblotting of affinity purified antigens, anti-cardiolipin using EIA, and anti-Ro (SS-A), La (SS-B), Sm, nRNP, Jo-1, PM-Scl, and Mi-2 by counter-current immunoelectrophoresis.RESULTS: Anti-Ku antibodies were found in 23% of patients with PPH, 4% with SPH, and none of 24 normal controls (PPH versus SPH, p = 0.06; PPH versus controls, p = 0.01). Antibodies to CENP-B were found in one patient each with PPH and SPH, anti-topoisomerase I in one with SPH, and anti-Ro (SS-A) and La (SS-B) in one with PPH. Overall, 12 patients (39%) with PPH had Raynaud's phenomenon or positive ANA results, with 9 (29%) having more specific autoantibodies associated with connective tissue diseases.CONCLUSIONS: These results further suggest a link between at least a subgroup of patients with PPH and autoimmune connective tissue diseases, with anti-Ku antibodies being a possible new serologic marker.