以肺高血压首诊的POEMS综合征患者的临床特征分析  

Clinical features of POEMS syndrome initially diagnosed as pulmonary hypertension

作　　者：刘红 吴文汇[2] 邱宏玲 罗祠君[2] 李慧婷 姜蓉[2] 何晶[2] 宫素岗[2] 刘锦铭[2] 王岚[2] 赵勤华[2] Liu Hong;Wu Wenhui;Qiu Hongling;Luo Cijun;Li Huiting;Jiang Rong;He Jing;Gong Sugang;Liu Jinming;Wang Lan;Zhao Qinhua(Department of Respiratory and Critical Care Medicine,Yixing People′s Hospital,Yixing 214200,China;Department of Cardio-Pulmonary Circulation,Shanghai Pulmonary Hospital,Tongji University School of Medicine,Shanghai 200433,China)

机构地区：[1]江苏省宜兴市人民医院呼吸与危重症医学科,宜兴214200 [2]同济大学附属上海市肺科医院肺循环科,上海200433

出　　处：《中华全科医师杂志》2023年第9期915-920,共6页Chinese Journal of General Practitioners

基　　金：国家自然科学基金(81900050,82000059);上海市科学技术委员会自然科学基金(22ZR1452400);上海市浦江人才计划(22PJD064);上海市卫生健康委员会卫生行业临床研究专项青年项目(20204Y0384);上海市肺科医院临床研究项目(FKLY20005,fk18003)。

摘　　要：目的探讨以肺高血压首诊的POEMS综合征患者的临床特征。方法该研究为回顾性病例系列研究。回顾性收集2013年5月至2021年11月同济大学附属上海市肺科医院收治的以肺高血压首诊的POEMS综合征患者的临床资料,包括一般临床资料以及实验室检查、超声心动图、右心导管检查、治疗方法及随访结果,并分析其特征。结果共入选患者7例,其中男性3例,年龄44~62(55±9)岁。7例患者均以超声心动图提示肺动脉压力升高就诊,首发症状主要为胸闷气促(7例)、乏力(6例)和下肢水肿(4例)。POEMS综合征相关症状主要为多发性周围神经病变(7例)、多浆膜腔积液(6例)、脏器肿大(5例)、皮肤病变(5例)和内分泌病变(4例)。7例患者血清血管内皮生长因子水平均明显升高。7例患者超声心动图估测的肺动脉收缩压为(66±21)mmHg(1 mmHg=0.133 kPa)。其中6例患者进行了右心导管检查,测得的肺动脉平均压为(35±9)mmHg,肺血管阻力为(4.00±2.10)Wood U。7例患者确诊后均接受了针对POEMS综合征的药物治疗,5例活动耐量改善、世界卫生组织功能分级稳定或好转,1例病情控制欠佳开始血液透析治疗,1例死亡。4例患者复查了超声心动图,2例肺动脉压力完全逆转,1例部分逆转,1例未逆转。结论以肺高血压首诊的POEMS综合征患者多合并多系统临床表现,血流动力学特征主要表现为毛细血管前肺高血压,单纯进行肺高血压靶向药物治疗效果欠佳,而对POEMS综合征原发疾病治疗多可逆转肺高血压。Objective To analyze the clinical characteristics,diagnosis and treatments of patients with POEMS syndrome initially diagnosed as pulmonary hypertension(PH).Methods Clinical data of 7 patients who were initially diagnosed as PH and finally diagnosed as POEMS syndrome in Shanghai Pulmonary Hospital from May 2013 to November 2021 were retrospectively reviewed.Clinical manifestations,laboratory tests,echocardiography,hemodynamic findings,treatment and prognosis of patients were analyzed.Results Seven patients,including 4 males and 3 female,aged(55±9)(44-62)years were presented with elevated pulmonary artery pressure by echocardiography at admission.Chest tightness and shortness of breath(7/7),fatigue(6/7)and lower limb edema(4/7)were the most common symptoms in the first-episode.Meanwhile,patients also presented symptoms associated with POEMS syndrome,including multiple peripheral neuropathy(7/7),multiserosal cavity effusion(6/7),organomegaly(5/7),skin changes(5/7),and endocrine lesions(4/7).Serum levels of vascular endothelial growth factor(VEGF)were significantly increased in all patients.The pulmonary arterial systolic blood pressure was(66±21)mmHg(1 mmHg=0.133 kPa)estimated by echocardiography.Six patients underwent right heart catheterization and significantly increased mean pulmonary artery pressure((35±9)mmHg)was confirmed;and their pulmonary vascular resistance was(4.00±2.10)Wood U.All patients received corresponding treatment for POEMS syndrome.The excise tolerance was improved in 5 patients after successful treatment with stable or reversed WHO functional class.One patient received hemodialysis treatment for uncontrolled POEMS.One patient died during follow-up.The echocardiography was followed up in 4 patients,and 2 of whom had a complete reversal of PH,1 had a partial reversal,and 1 had not yet reversed.Conclusions In patients with PH who have multisystem manifestations,such as multiple peripheral neuropathy,multiserosal cavity effusion,organomegaly and skin changes,POEMS syndrome should be considered

关 键 词：高血压 肺性 POEMS综合征 超声心动描记术 右心导管检查 

分 类 号：R544.1[医药卫生—心血管疾病]