硬皮病肾危象合并肺动脉高压13例临床分析  

A clinical analysis of 13 patients with scleroderma renal crisis and pulmonary arterial hypertension

作　　者：李娟[1,2] 赵梦珠 徐东 周佳鑫[1] 李梦涛[1] 曾小峰[1] 张奉春[1] 侯勇[1] Li Juan;Zhao Mengzhu;Xu Dong;Zhou Jiaxin;Li Mengtao;Zeng Xiaofeng;Zhang Fengchun;Hou Yong(Department of Rheumatology and Immunology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Key Laboratory of Rheumatology and Clinical Immunology,Ministry of Education,National Clinical Research Center for Dermatologic and Immunologic Diseases(NCRC-DID),Beijing 100730,China;Department of Rheumatology and Immunology,Luohe Central Hospital,Henan Province)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院(风湿免疫病学教育部重点实验室国家皮肤与免疫疾病临床医学研究中心)风湿免疫科,北京100730 [2]河南省漯河市中心医院风湿免疫科

出　　处：《中华风湿病学杂志》2022年第4期238-242,共5页Chinese Journal of Rheumatology

摘　　要：目的分析总结硬皮病肾危象合并肺动脉高压(SRC-PAH)患者的特点。方法回顾分析2012年1月至2020年10月北京协和医院住院的472例SSc患者中SRC-PAH患者的特点。结果 13例SRC-PAH患者中, 1例皮肤受累为局限型、12例为弥漫型。5例患者肾危象发生在PAH之前, 4例患者PAH发生在肾危象之前, 其余4例二者同时被发现。其中11例患者存在雷诺现象, 7例出现消化道出血, 6例出现肺水肿, 3例有毛细血管扩张。12例ANA阳性, 4例抗Scl-70抗体阳性。11例患者N-末端脑钠肽前体(NT-proBNP)>1 400 ng/L。2例患者合并血栓性微血管病(TMA)。13例患者中, 3例患者住院期间死亡;2例失访;2例在随诊5年内死亡;6例患者存活, 其中4例规律透析患者中的1例已脱离透析。结论硬皮病患者中, SRC的发生可以早于、晚于或同时与SSc-PAH发生;患者可能有更高的NT-proBNP及消化道出血的发生率。磷酸二酯酶5抑制剂及内皮素受体拮抗剂的应用可能有益。Objective To describe a series of systemic sclerosis(SSc)patients with the combination of scleroderma renal crisis(SRC)and pulmonary arterial hypertension(PAH).Methods The medical records of 472 SSc patients in Peking Union Medical College Hospital between January 2012 and October 2020 were reviewed and a retrospective analysis of the characteristics of patients with SRC and PAH among SSc patients was conducted.Results Thirteen patients suffered from SRC and PAH in the SSc patients,1 case was limited cutaneous SSc,and 12 cases were diffuse cutaneous SSc.Five patients had renal crisis before pulmonary arterial hypertension,4 patients had pulmonary arterial hypertension before the occurrence of renal crisis,and the remaining 4 patients were found at the same time.Among them,11 patients had Raynaud's phenomenon,7 had gastrointestinal bleeding,6 had pulmonary edema and 3 had telangiectasias.Twelve cases were positive for anti-nuclear antibodies and 4 cases were positive for anti-Scl-70 antibodies.N-terminal pro-brain natriuretic peptide(NT-proBNP)>1400 ng/L in 11 patients.Two patients had thrombotic microangiopathy(TMA).Among the 13 patients,3 patients died during hospitalization,2 patients were lost to follow-up,and 2 patients died within 5 years of follow-up.Six patients survived,and 1 of the 4 patients with regular dialysis were discharged from dialysis.Conclusion In patients with scleroderma,SRC can occur earlier,later than,or at the same time with SSc-PAH.Patients may have a higher incidence of gastrointestinal bleeding and higher level of NT-proBNP.PDE5i or ERAs may be beneficial.

关 键 词：硬皮病 系统性 高血压 肺性 硬皮病肾危象 

分 类 号：R593.25[医药卫生—内科学] R544.1[医药卫生—临床医学]