IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease
IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease
复制标题
IgG4 相关主动脉炎/主动脉周围炎和动脉周围炎：一系列独特的 IgG4 相关疾病
DOI：
10.1186/s13075-020-02197-w
复制
发表时间：
2020-05-04
影响因子：
4.9
通讯作者：
Zhang, Fengchun
中科院分区：
文献类型：
--
作者：
Peng, Linyi;Zhang, Panpan;Zhang, Fengchun
背景免疫球蛋白（IG）-G4相关疾病（IgG 4-RD）可能涉及大血管。本研究旨在明确IgG 4-RD合并腹膜炎/腹膜炎和动脉周围炎（PAO/PA）的临床特点，评价其治疗效果。IgG 4相关PAO/PA的分布分为4种类型：1型，胸主动脉; 2a型，腹主动脉; 2b型，腹主动脉和髂动脉; 2c型，髂动脉; 3型，胸主动脉和腹主动脉; 4型，其他动脉。患者的人口统计学数据，临床特征，实验室参数，和治疗efficacy进行了analysed.ResultsOf 587 IgG 4-RD患者，89（15.2%）有PAO/PA。平均年龄为58.3 ± 11.1岁，男性居多（85.4%）。受累血管如下：腹主动脉（83.1%）、髂动脉（70.8%）、胸主动脉（13.5%）和其他血管（13.5%）。IgG 4相关PAO/PA的最常见分布类型为2b型，有74例（83.1%）患者，其次为2a型、2c型、3型和1型。55例（61.8%）PAO/PA患者发生肾积水，43例（48.3%）发生肾功能不全，31例（34.8%）PAO/PA患者因严重输尿管梗阻而接受D-J支架引流。结论IgG 4-RD合并PAO/PA与非PAO/PA患者在人口学特征、器官受累分布、炎性标志物、血清IgG 4和IgE水平等方面存在差异。最常见的受影响的血管是腹主动脉，大多数患者对治疗反应良好。
BackgroundLarge vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA).MethodsThis study prospectively enrolled 587 patients with IgG4-RD with a follow-up time of more than 6 months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; type 2b, abdominal aorta and iliac artery; type 2c, iliac artery; type 3, thoracic and abdominal aorta; and type 4, other arteries. Patient’s demographic data, clinical characteristics, laboratory parameters, and treatment efficacy were analyzed.ResultsOf 587 IgG4-RD patients, 89 (15.2%) had PAO/PA. The average age was 58.3 ± 11.1 years, with male predominance (85.4%). Vessels affected were as follows: abdominal aorta (83.1%), iliac artery (70.8%), thoracic aorta (13.5%), and other vessels (13.5%). The most prevalent distribution type of IgG4-related PAO/PA was type 2b, with 74 (83.1%) patients, followed by type 2a, type 2c, type 3, and type 1. Fifty-five (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurring in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with a glucocorticoid and immunosuppressants, 82% patients achieved remission with shrinking of the perivascular mass by more than 30%.ConclusionsIgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organ involvement distribution, inflammatory markers, and serum IgG4 and IgE. The most common affected vessel was the abdominal aorta, and most patients responded well with treatment.