Analyzing Fatal Cases of Chinese Patients with Primary Antineutrophil Cytoplasmic Antibodies-Associated Renal Vasculitis: A 10-Year Retrospective Study
Analyzing Fatal Cases of Chinese Patients with Primary Antineutrophil Cytoplasmic Antibodies-Associated Renal Vasculitis: A 10-Year Retrospective Study
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中国原发性抗中性粒细胞胞浆抗体相关性肾血管炎患者死亡病例分析：一项 10 年回顾性研究
DOI：
10.1159/000165117
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发表时间：
2008-01-01
影响因子：
2.8
通讯作者：
Chen, Nan
中科院分区：
文献类型：
--
作者：
Chen, Yong-Xi;Yu, Hai-Jin;Chen, Nan
背景/目标：原发性抗神经细胞胞浆抗体（ANCA）相关性系统性血管炎（AASV）预后差，肾脏受累是其最常见的表现。很少有研究发表关注预后不良的AASV患者。研究方法：1997 ~ 2006年，上海瑞金医院共收治ANCA相关性肾血管炎101例，其中显微镜下多血管炎（MPA）70例，韦格纳肉芽肿病（WG）14例，变应性肉芽肿综合征（CSS）3例，肾局限性血管炎（RLV）14例，死亡26例。对患者资料进行回顾性分析。结果：WG、MPA和RLV分别占死亡病例的23.1%（6/26）、65.4%（17/26）和11.5%（3/26）。CSS患者中未观察到死亡。仅感染一项就造成13人死亡。感染合并肺部活动性血管炎占3例。活动性血管炎的器官特异性受累单独导致8例死亡。其他人死于急性心肌梗死或胃癌。与存活患者相比，死亡患者肾功能不全程度更重，年龄更大（P < 0.01）。在首次诱导缓解治疗后存活的患者和未存活的患者之间，诊断时的临床表现和死亡原因无显著差异。感染仍然是主要的死亡原因。结论：感染是ANCA相关性肾血管炎患者死亡的主要原因，在预后不良的患者中，治疗反应可能与疾病严重程度无关。合理应用免疫抑制剂可改善预后。
Background/Aims: Primary antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitis (AASV) used to have poor prognosis, and renal involvement is its most common manifestation. Few studies have been published focusing on AASV patients with poor prognosis. Methods: From 1997 to 2006, 101 patients with ANCA-associated renal vasculitis (70 microscopic polyangiitis, MPA; 14 Wegener’s granulomatosis, WG; 3 Churg-Strauss syndrome, CSS; 14 renal limited vasculitis, RLV) were diagnosed in Shanghai Ruijin Hospital and 26 deaths were recorded among them. Patients’ data were retrospectively analyzed. Results: Patients with WG, MPA and RLV made up for 23.1% (6/26), 65.4% (17/26) and 11.5% (3/26) of all deaths. No deaths were observed among CSS patients. Infection alone accounted for 13 deaths. Infection together with pulmonary involvement of active vasculitis accounted for 3. Organ-specific involvement of active vasculitis alone caused 8 deaths. Others died of acute myocardial infarction or gastric carcinoma. Compared with patients who survived, nonsurvivors had more severe renal insufficiency and older age (p < 0.01). There was no significant difference regarding clinical presentation at diagnosis and cause of death between patients who survived first remission-induction treatment and those who did not. Infection remained the major cause of death. Conclusion: Infection is the major cause of death in patients with ANCA-associated renal vasculitis, and treatment response might not correlate to severity of disease in patients with poor prognosis. Rational use of immunosuppressants could improve the prognosis.