抗磷脂综合征合并慢性血栓栓塞性肺高血压22例临床资料分析

The clinical characteristics of antiphospholipid syndrome associated with chronic thromboembolic pulmonary hypertension

目的 通过分析抗磷脂综合征(APS)合并慢性血栓栓塞性肺高血压(CTEPH)患者的临床资料,旨在提高临床医生对其的认识与理解,从而实现早期识别,改善预后.方法 回顾性分析2012年1月-2018年8月北京协和医院风湿免疫科收治的22例APS合并CTEPH患者的人口统计学资料、临床表现、实验室检查及影像学资料、治疗和预后.结果 APS合并CTEPH患者22例,男7例,女15例,年龄19~51岁.主要临床症状,胸痛6例,劳力性呼吸困难22例,咳嗽6例,咯血9例.12例患者狼疮抗凝物(LA)、抗心磷脂抗体(ACL)、抗β2糖蛋白Ⅰ(抗β2GP Ⅰ)抗体均阳性,2种抗体阳性者5例,1种抗体阳性者5例.22例患者行超声心动图检查提示不同程度肺高血压.9例患者行肺通气/血流灌注显像筛查,见多发血流灌注缺损;20例患者行计算机断层摄影肺血管造影(CTPA)检查,见多发肺栓塞,肺动脉主干增宽;9例患者经右心导管检查证实CTEPH.所有患者均接受长期抗凝治疗.9例患者行肺动脉血栓内膜剥脱术(PTE).13例患者未行PTE,仅口服药物保守治疗,其中11例患者症状好转,3例患者在随访中症状反复,3例患者因右心衰竭而死亡.结论 肺栓塞是APS患者常见的血栓事件之一,如未能及时发现,易进展为CTEPH.早期规律抗凝治疗可有效改善CTEPH症状,但无法逆转肺高血压进程;部分患者经抗凝治疗及心外科评估后可通过PTE长期改善预后.

Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) patients with chronic thromboembolic pulmonary hypertension (CTEPH).Methods A total of 22 APS patients with CTEPH were enrolled in our study,who were admitted in Peking Union Medical College Hospital from January 2012 to August 2018.Diagnoses were confirmed by computed tomographic pulmonary angiography (CTPA),or pulmonary angiography.Demographic characteristics,clinical manifestations,laboratory tests,therapy,World Health Organization (WHO) functional class were retrospectively collected.Results There were 15 females and 7 males with a median age of 29-year-old.Chest pain (6 cases),dyspnea on exertion (22 cases),cough (6 cases) and hemoptysis (9 cases) were the most common clinical manifestations.Lupus anticoagulant (LA),anticardiolipin (ACL) antibodies and anti-beta 2 glycoprotein Ⅰ (anti-β2 GP Ⅰ) antibodies were all positive in 12 patients,two of three antibodies positive in 5 patients,only one positive in 5 patients.The WHO functional classes were Ⅱ-Ⅳ before treatment.Anticoagulants were administrated in all patients.After multidisciplinary evaluation,9 patients underwent pulmonary thromboendarterectomy (PTE),who all had a good outcome.Symptoms in eleven over thirteen patients with only anticoagulants improved.Three patients developed cardiac deterioration while other 3 patients died of right heart failure during follow-up.Conclusion Pulmonary embolism is one of the most common thrombotic events in APS patients.It is important to recognize symptoms and signs related to pulmonary embolism and start anticoagulation as soon as possible.Standard anticoagulation improves symptoms but can't reverse the process of pulmonary hypertension.Some patients may benefit from PTE after anticoagulation and multidisciplinary evaluation.