干燥综合征合并ANCA相关性血管炎肾损害及文献荟萃  

Clinicopathological analysis of Sjogren＇s syndrome complicated with ANCA associated vasculitis with renal involvement

作　　者：吴海婷[1] 叶葳[1] 文煜冰[1] 蔡建芳[1] 李航[1] 陈丽萌[1] 李明喜[1] 李雪梅[1] 李学旺[1] Wu Haiting;Ye Wei;Wen Yubing;Cai Jianfang;Li Hang;Chen Limeng;Li Mingxi;Li Xuemei;Li Xuewang.(Division of Nephrology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China)

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肾内科,北京100730

出　　处：《中华肾脏病杂志》2018年第3期161-166,共6页Chinese Journal of Nephrology

摘　　要：目的探讨干燥综合征（ss）合并伴肾脏累及的抗中性粒细胞胞质抗体（ANCA）相关性血管炎患者的临床及肾脏病理表现。方法检索1990年1月至2017年6月间经北京协和医院诊断及文献报道的SS合并ANCA相关性血管炎肾损害患者，收集及分析其基本信息、受累脏器、免疫学指标、肾脏受累的临床和（或）病理表现等。结果本院4例，文献检索所得14例，共18例。ss均在ANCA相关性血管炎之前或同期诊断。83．3％（15／18）存在腺体及肾脏以外脏器受累，均存在髓过氧化物酶（MPO）．ANCA阳性，其中2例合并PR3-ANCA阳性，83．3％（15／18）抗核抗体（ANA）阳性，55．6％（10／18）类风湿因子阳性，77．8％（14／18）抗SSA抗体阳性，38．9％（7／18）抗SSB抗体阳性。肾脏方面以肾功能不全（中位肌酐174μmol／L）、镜下血尿、中等量蛋白尿（中位24h尿蛋白1．70g）为表现，肾脏病理符合寡免疫复合物的肾炎、伴不同程度新月体和（或）纤维素坏死及间质病变。结论sS合并ANCA相关性血管炎肾损害是一种少见的临床情况，这部分患者几乎均为MPO-ANCA阳性，有较高的ANA阳性率及腺外器官受累。对SS患者，若出现肾小球受累表现和（或）SS不能解释的。肾功能异常，应检测ANCA并积极行肾活检以明确是否合并ANCA相关性肾炎。Objective To investigate the clinical and pathological features of patients with a combination of Sjogren＇s syndrome （SS） and antineutrophil cytoplasmic antibody （ANCA） associated vasculitis with renal involvement. Methods By searching the Peking Union Medical College Hospital medical database and literature between January 1990 and June 2017, patients had a combination of SS and ANCA associated vasculitis with renal involvement were included. Data of clinical information, autoimmune antibodies, renal manifestations and renal pathology were retrieved and analyzed. Results Eighteen patients were enrolled： 4 from our hospital and 14 from literature. SS was diagnosed no later than ANCA associated vasculitis in all the patients, among which 83.3%（15/18） of patients had extra-glandular and extra-renal organs involved. All the patients were tested positive for myeloperoxidase （MPO）-ANCA, and only two were protein 3 （PR3）-ANCA positive concurrently. The positivity rates of antinuelear antibody （ANA）, rheumatoid factor （RF）, anti-SSA antibody, and anti-SSB antibody were 83.3%（15/18）, 55.6%（10/18）, 77.8%（14/18）, and 38.9%（7/18）, respectively. The renal manifestations were characterized by renal insufficiency with a median serum ereatinine of 174 μmol/L, hematuria, moderate proteinuria with a median 24 hour urine protein of 1.70 g, and necrotizing vasculitis with oligo- immune complex and varying degrees of interstitial damage in pathology. Conclusions A combination of Sjogren＇s syndrome and ANCA associated vasculitis with renal involvement is rare in clinical setting, and almost all of the patients are MPO-ANCA positive, with high probability of ANA positivity and extra- glandular involvement. Physicians should beware of ANCA associated glomerulonephritis in SS patients with inexplicable renal dysfunction and renal biopsy should be carried out in time.

关 键 词：干燥综合征 ANCA相关性血管炎 ANCA相关性肾小球肾炎 

分 类 号：R593.2[医药卫生—内科学] R692[医药卫生—临床医学]