ANTINUCLEAR ANTIBODIES IN PRIMARY PULMONARY-HYPERTENSION
ANTINUCLEAR ANTIBODIES IN PRIMARY PULMONARY-HYPERTENSION
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DOI：
10.1016/s0735-1097(86)80301-1
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发表时间：
1986-12-01
影响因子：
24
通讯作者：
BRUNDAGE, BH
中科院分区：
文献类型：
--
作者：
RICH, S;KIERAS, K;BRUNDAGE, BH
本文对43例原发性肺动脉高压患者及16例继发性肺动脉高压患者的临床及血流动力学特征与抗核抗体阳性的关系进行了研究。每位患者使用KB细胞底物免疫荧光试验测定抗核抗体。原发性肺动脉高压患者中，40%的抗核抗体阳性，滴度为1:80或更高。抗核抗体阳性和抗核抗体阴性的原发性肺动脉高压患者在临床和血流动力学方面没有差异。继发性肺动脉高压患者抗核抗体的发生率为6%，与正常人群相似。原发性肺动脉高压患者与不明原因肺动脉高压合并胶原血管病变患者的临床、血流动力学、血清学和组织学相似性提示，一些患者的原发性肺动脉高压可能是一种局限于肺部的胶原血管疾病。抗核抗体测试阳性的频率将原发性肺动脉高压置于胶原血管疾病谱中的类风湿关节炎和硬皮病之间。然而，在期望免疫抑制剂对这些患者有益之前，还需要进一步的研究。
The association of positive antinuclear antibodies with the clinical and hemodynamic features of 43 patients with primary pulmonary hypertension and 16 patients with secondary pulmonary hypertension was investigated. Each patient had determinations of antinuclear antibodies using a KB cell substrate immunofluorescent test. Of the patients with primary pulmonary hypertension, 40% had positive antinuclear antibodies at titers of 1:80 dilutions or greater. There were no differences between patients with primary pulmonary hypertension and positive antinuclear antibodies compared with those with negative antinuclear antibodies in relation to clinical or hemodynamic status. A 6% incidence rate of antinuclear antibodies was found in patients with secondary pulmonary hypertension, similar to that in the normal population. The clinical, hemodynamic, serologic and histologic similarity between patients with primary pulmonary hypertension and those with unexplained pulmonary hypertension associated with collagen vascular disorders suggests that primary pulmonary hypertension in some patients may represent a collagen vascular disease confined to the lungs. The frequency of positive antinuclear antibody tests would place primary pulmonary hypertension between rheumatoid arthritis and scleroderma in the spectrum of collagen vascular diseases. Further studies are necessary, however, before one might expect that immunosuppressive would be beneficial to these patients.