Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China.
Clin Rheumatol. 2023 Feb;42(2):463-470. doi: 10.1007/s10067-022-06397-4. Epub 2022 Oct 3.
Spontaneous renal hemorrhage (SRH) in ANCA-associated vasculitis (AAV) is rare but fatal. We aimed to characterize clinical manifestations and managements of AAV patients with SRH.
Hospitalized AAV patients were screened from January 2000 to April 2021, at Peking Union Medical College Hospital (PUMCH). Also, a systematic review was based on retrieving all the relevant literature from PubMed, MedlinePlus, and Web of Science until April 2021. Clinical features, management, and prognosis of the patients were collected and concluded.
In PUMCH, four out of 1640 AAV patients with SRH were included in our study; three had granulomatosis with polyangiitis (GPA) and one had microscopic polyangiitis (MPA). The ratio of men to women was 3 to 1, and the average age of onset was 55 years. The Birmingham Vasculitis Activity Score (BVAS) ranged from 21 to 23. Combining with documented reports, 13 patients were diagnosed as AAV complicated with SRH (including four from PUMCH), 7 with GPA, and 6 with MPA. Mean BVAS was 25.2 ± 6.6. The symptoms of SRH presented as severe back or abdominal pain. Patients with SRH to age- and gender-matched patients without SRH were compared, and we found that in the SRH group, the duration of disease was shorter, and BVAS, renal function, and inflammatory markers (WBC and ESR) were significantly greater, whereas Hb, Alb, and renal function greatly reduced.
This is the first summary of clinical features and treatments of SRH in AAV. Patients with AAV in early stage and with high disease activity appeared to be more likely to develop SRH. Key Points • This is the first summary of clinical features and treatments of SRH in AAV. • SRH more likely occurs in AAV patients in the early stage (≤ 3 months) and with high disease activity. • Clinicians should be aware of the possibility of SRH when AAV patients complain of back or abdominal pain.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）合并自发性肾出血（SRH）较为罕见，但后果严重。本研究旨在明确 AAV 合并 SRH 的临床表现和治疗方法。
本研究纳入 2000 年 1 月至 2021 年 4 月在北京协和医院（PUMCH）住院的 AAV 患者，同时进行系统性文献回顾，检索 PubMed、MedlinePlus 和 Web of Science 中所有相关文献，检索时间截至 2021 年 4 月。收集患者的临床特征、治疗和预后并进行总结。
PUMCH 的 1640 例 AAV 患者中，有 4 例发生 SRH，其中 3 例为肉芽肿性多血管炎（GPA），1 例为显微镜下多血管炎（MPA）。男女比例为 3:1，发病年龄平均为 55 岁。Birmingham 血管炎活动评分（BVAS）为 21-23 分。结合已发表的文献，共 13 例患者被诊断为 AAV 合并 SRH（包括 PUMCH 的 4 例），其中 7 例为 GPA，6 例为 MPA。平均 BVAS 为 25.2±6.6。SRH 的症状表现为严重的背痛或腹痛。SRH 患者与无 SRH 的年龄和性别匹配的患者相比，我们发现 SRH 组疾病持续时间更短，BVAS、肾功能和炎症标志物（白细胞和红细胞沉降率）显著升高，而血红蛋白、白蛋白和肾功能显著降低。
这是第一份关于 AAV 合并 SRH 的临床特征和治疗的总结。疾病早期且活动度高的 AAV 患者似乎更有可能发生 SRH。关键点 • 这是第一份关于 AAV 合并 SRH 的临床特征和治疗的总结。 • SRH 更可能发生在疾病早期（≤3 个月）且活动度高的 AAV 患者中。 • 当 AAV 患者出现背痛或腹痛时，临床医生应意识到发生 SRH 的可能性。
