Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.
Arthritis Res Ther. 2020 May 4;22(1):103. doi: 10.1186/s13075-020-02197-w.
Large 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).
This 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.
Of 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%.
IgG4-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.
大血管可能参与免疫球蛋白（Ig）-G4 相关疾病（IgG4-RD）。本研究旨在阐明大动脉炎/主动脉周围炎（PAO/PA）和 IgG4-RD 的临床特征，并评估其治疗效果。
本研究前瞻性纳入 587 例 IgG4-RD 患者，随访时间超过 6 个月。将 IgG4 相关 PAO/PA 的分布分为四型：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、腹主动脉；4 型，其他动脉。分析患者的人口统计学资料、临床特征、实验室参数和治疗效果。
587 例 IgG4-RD 患者中，89 例（15.2%）存在 PAO/PA。平均年龄为 58.3±11.1 岁，以男性为主（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%）。最常见的 IgG4 相关 PAO/PA 分布类型为 2b 型，共 74 例（83.1%），其次为 2a 型、2c 型、3 型和 1 型。55 例（61.8%）PAO/PA 患者存在肾积水，其中 43 例（48.3%）存在肾功能不全，31 例（34.8%）PAO/PA 患者因严重输尿管梗阻行 D-J 支架引流。经糖皮质激素和免疫抑制剂治疗后，82%患者的血管周围肿块缩小超过 30%，达到缓解。
PAO/PA 与非 PAO/PA 的 IgG4-RD 在人口统计学特征、器官受累分布、炎症标志物以及血清 IgG4 和 IgE 方面存在差异。最常见的受累血管为腹主动脉，多数患者经治疗后反应良好。
