中国大动脉炎性肾动脉炎(TARA)诊治多学科专家共识

大动脉炎性肾动脉炎诊治多学科共识中国专家组

复旦学报(医学版) ›› 2019, Vol. 46 ›› Issue (06) : 711-725.

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复旦学报(医学版) ›› 2019, Vol. 46 ›› Issue (06) : 711-725. DOI: 10.3969/j.issn.1672-8467.2019.06.001
专家论坛

中国大动脉炎性肾动脉炎(TARA)诊治多学科专家共识

  • 大动脉炎性肾动脉炎诊治多学科共识中国专家组
作者信息 +

Chinese multidisciplinary recommendations on the diagnosis and treatment of Takayasu's arteritis-induced renal arteritis (TARA)

  • Chinese Multidisciplinary Expert Task Force on TARA
Author information +
文章历史 +

摘要

大动脉炎(Takayasu's arteritis,TA)是中国、日本等东亚国家及地区青年女性好发的大血管炎症性疾病。TA累及肾动脉可引起大动脉炎性肾动脉炎(Takayasu's arteritis-induced renal arteritis,TARA),导致大动脉炎性肾动脉狭窄(Takayasu's arteritis-induced renal artery stenosis,TARAS),是青年人群发生恶性高血压、肾功能不全的首要原因。目前国内外均无TARA的临床诊治规范及指南,因此我们联合国内风湿免疫科、血管外科、心血管内科、泌尿系统内外科和放射诊断科等专家共同制定了中国大动脉炎性肾动脉炎诊治多学科专家共识,旨在规范疾病诊疗、促进多学科协作,为全科医师及不同学科医师提供临床实践指导。本共识的主要观点为:(1)TA是40岁以下高血压人群中的首要病因。(2)TARA高危人群为40岁以下高血压、腹部杂音、不明原因的肾萎缩患者。(3)TARA肾动脉中重度狭窄激活肾素-血管紧张素-醛固酮系统(renin-angiotensin-aldosterone system,RAAS),继而导致恶性高血压、心脑血管疾病、缺血性肾病等严重并发症,是TA不良预后以及早期死亡原因之一。(4)影像学检查是诊断与评价TARA的主要手段,包括血管多普勒超声、磁共振血管造影(magnetic resonance angiography,MRA)、计算机断层血管造影(computed tomography angiography,CTA)等,数字减影血管造影(digital subtraction angiography,DSA)仍是诊断金标准。(5)对TARA应当全面评估疾病活动度、肾脏功能学以及其他重要靶器官,对临床病情严重性予以分层。(6)TARA治疗应以风湿免疫科为主导,对于中重度严重患者由多学科合作诊疗制定个体化治疗方案。(7)TARA内科治疗诱导病情缓解和维持持续缓解,主要以糖皮质激素与化学合成缓解病情抗风湿病药(conventional synthetic disease-modifying anti-rheumatic drugs,cDMARDs)、生物合成缓解病情抗风湿病药(biological disease-modifying anti-rheumatic drugs,bDMARDs)联合治疗。(8)TARA外科治疗强调术前抗炎治疗并获得病情充分缓解、术后继续序贯内科治疗与评估,外科手术可获得较好的长期生存。(9)TARA合并多处血管病变,高血压可按照"脑-心-肾"依次评估并制定降压目标和降压决策。(10)对于TARA患者的妊娠风险与时机,需要多学科团队(multidisciplinary team,MDT)全面评估病情活动度,充分权衡脏器功能水平。

Abstract

Takayasu's arteritis (TA) is a major large-vessel vasculitis involved in young women of East Asia countries and regions including China and Japan.Takayasu's arteritis-induced renal arteritis (TARA) involving renal arteries will result in Takayasu's arteritis-induced renal artery stenosis (TARAS),which has been considered the primary cause of malignant hypertension and renal insufficiency in young population.To date,there has been no standard or guideline for the diagnosis and treatment of TARA both in China and abroad.Therefore,a multidisciplinary expert recommendations on the diagnosis and treatment of TARA has been established by a task force including Chinese experts from rheumatology,vascular surgery,cardiology,nephrology,urology surgery and radiology,etc.,aims to standardize the diagnosis and treatment,to promote multi-disciplinary collaboration,and to serve general practitioners and doctors from different disciplines at different levels.The keypoints of the recommendations include:(1) TA is the leading cause of hypertension in people under 40 years old.(2) High-risk population in patients with TARA are characterized with hypertension,abdominal murmurs and unknown renal atrophy under 40 years old.(3) Moderate and severe stenosis of TARA activates renin-angiotensin-aldosterone system (RAAS),which leads to severe complications such as malignant hypertension,cardiovascular and cerebrovascular diseases and ischemic nephropathy,and is one of the causes of adverse prognosis and early mortality of TA.(4) Imaging examination is the main method of diagnosis and evaluation of TARA,including vascular Doppler ultrasound,magnetic resonance angiography (MRA),computed tomography angiography (CTA),etc.Digital subtraction angiography (DSA) is still the gold standard.(5) TARA should be comprehensively assessed disease activity and functions of kidney and other important target organs,so as to stratify the disease severity in clinical conditions.(6) TARA combined treatment should be dominated by rheumatologists,and individualized treatment strategy should be formulated for moderate to severe patients by multidisciplinary team (MDT).(7) Medical treatment in TARA induces remission mainly using the combined strategy of glucocorticoids and conventional synthetic disease-modifying anti-rheumatic drugs (cDMARDs) or biological disease-modifying anti-rheumatic drugs (bDMARDs).(8) Surgical treatment in TARA emphasizes pre-operative anti-inflammatory treatment and full remission of the disease as well as continuing sequential medical treatment and evaluation after operation,so that it can achieve better long-term survival.(9) If patients with TARA combined with multiple vascular lesions,hypertension can be assessed according to the order of "brain-heart-kidney" in order to make the goal and the strategy of lowering blood pressure treatment.(10) Risk and timing at pregnancy in TARA require MDT to comprehensively assess disease activity and fully weigh organ function.

关键词

大动脉炎(TA) / 肾动脉炎(RA) / 肾动脉狭窄 / 共识

Key words

Takayasu's arteritis (TA) / renal arteritis (RA) / renal artery stenosis / recommendation

引用本文

导出引用
大动脉炎性肾动脉炎诊治多学科共识中国专家组. 中国大动脉炎性肾动脉炎(TARA)诊治多学科专家共识[J]. 复旦学报(医学版), 2019, 46(06): 711-725 https://doi.org/10.3969/j.issn.1672-8467.2019.06.001
Chinese Multidisciplinary Expert Task Force on TARA. Chinese multidisciplinary recommendations on the diagnosis and treatment of Takayasu's arteritis-induced renal arteritis (TARA)[J]. Fudan University Journal of Medical Sciences, 2019, 46(06): 711-725 https://doi.org/10.3969/j.issn.1672-8467.2019.06.001
中图分类号: R543.5    R593.2   

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国家自然科学基金(81771730,81601398);上海市科委研究项目(17140902000)
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