原发性干燥综合征并亚临床甲状腺功能减退相关肺动脉高压——1例报告及文献复习.pdfVIP

临床论著 原发性干燥综合征并亚临床甲状腺功能减退相关肺 动脉高压一一1例报告及文献复习 史永红 王婉瑜 杨坤旺 福建医科大学附属厦门第一医院呼吸科， 福建 厦门 361 003 【摘要】目的 提高对原发性干燥综合征相关肺动脉高压临床特征认识。 方法 结合1例原发性干燥综合征征相关肺动脉高 压临床资料进行文献复习。 结果 患者，女，1 6岁，因运动性呼吸困难1年住院，诊断：1．肺动脉高压；2．原发性干燥综合 征；3．亚临床甲状腺功能减退，免疫抑制治疗有效。结论 原发性干燥综合征相关肺动脉高压是一种少见、严重的并发症。多 普勒超声心动图对肺动脉高压早期诊断是必需的，免疫机制在原发性干燥综合征相关肺动脉高压发病中期起重要作用，积极 免疫抑制治疗能改善原发性干燥综合征相关肺动脉高压预后。 【关键词】肺动脉高压；原发性干燥综合征；亚临床甲状腺功能减退 【中图分类号】R543．2 【文献标识码】A 【文章编号】1 009-3257(2008)0l一0026-03 Pulmonary arterial hypertension associated with primary Sjogren’s syndrome and subclinical SHl Yong-hong，WANG Wan-yu，YANG Kun-wang，Department of Respiration，the First Affiliated Hospital of Fuiian Medical University，Xiamen 36Ioo3，ChiFla [Abstract] Objecti-， To improve the understanding of the clinical characteristics of pulmonary arterial hypertension (PAH)associated with primary Sjogren’S syndrome(PSS)and subclinical hypothyroidism． Me Ioars A case of PAH associated with PSS and subclinical hypothyroidism was reported and the literature was reviewed．Resu／／s A 16-year old female was admitted to this hospital because of exertional dyspnea for 1 year．Her disease was diagnosed as PAH，PSS and subc cal hypothyroidism． Conclusion PAH associated with PSS is a rare fatal complicatioD．It is necessary to Use Doppler echOcardiOgraphy to detect PAH early． Immunologicalmechanisms may play aD important role in PAH and active immunosuppressive therapy can improve the prognostic PAH associated with PSS． [Key words]pulmonary arterial hypertension l primary Sjogren’S syndrome l subclinical hypothyroidism 原发性干燥综合征(Primary Sjogren’S syndrome 种少见、严重并发症，有报道发病率仅约4．3％t 1，PAH PsS)是一种较常见的结缔组织病。在我国Pss相关肺动 的发生与死亡率呈正相关。我院收治一例以呼吸困难为 脉高压(Pulmonary arterial hypertension PAH)是一 首发表现原发性干燥综合征并亚临床甲状腺功能减退相 中的应用[J]．中国医刊，2006，41(2)：36—37． 9．Pennington DG，Swartz M，Godd JE，et a1．Intra-aortic bal 1oon 7．赵铁夫，马涵英，孟旭，等．心脏手术围术期主动脉内球囊反 pumping in card