摘要
目的分析大动脉炎（TA）合并肺动脉高压（PAH）的临床特点，以提高临床早期诊治的水平。方法1987-2007年北京协和医院住院治疗的TA患者共191例，对其中合并PAH的12例患者的临床表现、实验室检查及治疗、预后进行回顾性分析。结果12例患者中女性10例，男性2例。年龄14．47岁，平均（27±10）岁。11例有肺血管受累的临床表现和（或）体征，7例以肺血管受累表现（活动后气短、咯血）为首发症状，其他首发表现分别为发热4例，乏力4例，四肢缺血表现（下肢间歇性跛行、上肢痛、肢体麻木）4例，头晕3例。12例患者中7例为Ⅰ＋Ⅳ型、1例为Ⅱ＋Ⅳ型、3例为Ⅲ＋Ⅳ型、1例为Ⅳ型。10例患者有炎性指标[红细胞沉降率（ESR）、C反应蛋白（CRP）]明显升高，ESR（66＋56）mm／1h。所有的患者均接受激素及免疫抑制剂治疗，1例行支架植入术，1例接受手术治疗。除1例因术后低心排出量死亡外，其余患者症状均好转。结论PAH为大动脉炎累及肺血管的晚期及严重并发症，常合并有全身其他部位血管受累。早期因起病隐匿不易引起重视，应行肺血管造影、CTA或心脏超声进行早期筛查，早期治疗。治疗上除激素、免疫抑制剂治疗原发病外，当血管重度狭窄时可行支架植入或血管扩张术。
Objective To investigate the clinical characteristics of Takayasu＇s arteritis （TA） with pulmonary hypertension （PAH） in order to improve the diagnosis and treatment earlier. Methods Twelve out of 191 patients with TA registered in Peking Union Medical College Hospital from 1987 to 2007 were diagnosed as PAH, the clinical data of 12 patients were analyzed. Results Ten patients were females. The range of age were from 14 to 47 years old, the average age was （27±10） years old. Eleven patients had the clinical manifestations or/and signs of pulmonary artery involvement. Seven patients presented with short breath after exercise or hemoptysis as the first manifestation, four patients with fatigue, four patients with intermittent elaudieation or pain or numbness of extremities, three patients with dizziness. Seven patients belonged to type I ＋IV, one patient to type Ⅱ ＋IV, three patients to type Ⅲ＋IV, one patient to type V. Elevated ESR/CRP was found in ten patients. All patients took the glucocorticoid and DMARDs, stent implantation in pulmonary artery was done in one patient, BentaU was operated in another patient .The symptoms of all patients improved except one patient died for low cardiac output after operation. Conclusion PAH is one of the severe complications in late stage of TA, and other arteries are usually involved too. Because it is difficult to observe PAH in TA patients in early stage, CTA or pulmonary angiography and UCG should be taken in early stage. The stent implantation or dilating the artery should he considered as a treatment, hut at on the same time, glucocorticoid and DMARDs should be taken to avoid the relapse.
出处
《中华风湿病学杂志》
CAS
CSCD
北大核心
2009年第9期612-615,共4页
Chinese Journal of Rheumatology
