Ebe Hiroshi, Tsuboi Hiroto, Hagiya Chihiro, Takahashi Hiroyuki, Yokosawa Masahiro, Hagiwara Shinya, Hirota Tomoya, Kurashima Yuko, Takai Chinatsu, Miki Haruka, Asashima Hiromitsu, Umeda Naoto, Kondo Yuya, Ogishima Hiroshi, Suzuki Takeshi, Chino Yusuke, Matsumoto Isao, Sumida Takayuki
Department of Internal Medicine, Faculty of Medicine, University of Tsukuba , Tsukuba , Japan.
Mod Rheumatol. 2015 Jan;25(1):105-9. doi: 10.3109/14397595.2014.903596. Epub 2014 Apr 23.
To define the clinical features of IgG4-related disease (IgG4-RD) complicated with perivascular lesions.
The clinical features of seven patients with IgG4-RD and perivascular lesions diagnosed at the University of Tsukuba Hospital between October 2008 and October 2013, were analyzed, including clinical background, results of imaging studies, satisfaction of the 2011 comprehensive diagnostic criteria (CDC) for IgG4-RD, laboratory data, distribution of perivascular lesions, involvement of other organs, and response to steroid therapy.
We studied six men and one woman with a mean age of 66.9 ± 6.7 years (± SD). Six of seven patients were diagnosed as definite IgG4-RD, while the seventh was considered possible IgG4-RD, based on the CDC for IgG4-RD. Serum IgG4 levels at diagnosis were higher than 135 mg/dl in all seven patients (mean, 933 ± 527). Serum C-reactive protein (CRP) levels were elevated in two only (mean, 1.42 ± 3.56 mg/dl). The perivascular lesions were located in the pulmonary artery (n = 1), thoracic aorta (n = 2), abdominal aorta (n = 6), coronary (n = 1), celiac (n = 1), superior mesenteric (n = 1), renal (n = 2), inferior mesenteric (n = 5), and iliac (n = 3) arteries. In addition to perivascular lesions, six patients showed involvement of other organs. All seven patients were treated with prednisolone (0.6 mg/kg/day), which rapidly improved the perivascular and other organ lesions in six patients (the other one patient have not yet been evaluated due to the short follow-up).
Perivascular lesions show wide distribution in patients with IgG4-RD. Serum CRP levels are not necessarily elevated in these patients. Steroid therapy is effective in IgG4-RD and results in resolution of lesions.
明确IgG4相关性疾病（IgG4-RD）合并血管周围病变的临床特征。
分析2008年10月至2013年10月在筑波大学医院诊断的7例IgG4-RD合并血管周围病变患者的临床特征，包括临床背景、影像学检查结果、2011年IgG4-RD综合诊断标准（CDC）的符合情况、实验室数据、血管周围病变分布、其他器官受累情况以及对类固醇治疗的反应。
我们研究了6名男性和1名女性，平均年龄为66.9±6.7岁（±标准差）。根据IgG4-RD的CDC，7例患者中有6例被诊断为确诊的IgG4-RD，而第7例被认为可能是IgG4-RD。所有7例患者诊断时血清IgG4水平均高于135mg/dl（平均，933±527）。仅2例患者血清C反应蛋白（CRP）水平升高（平均，1.42±3.56mg/dl）。血管周围病变位于肺动脉（n = 1）、胸主动脉（n = 2）、腹主动脉（n = 6）、冠状动脉（n = 1）、腹腔动脉（n = 1）、肠系膜上动脉（n = 1）、肾动脉（n = 2）、肠系膜下动脉（n = 5）和髂动脉（n = 3）。除血管周围病变外，6例患者还表现出其他器官受累。所有7例患者均接受泼尼松龙治疗（0.6mg/kg/天），6例患者的血管周围及其他器官病变迅速改善（另1例患者因随访时间短尚未评估）。
血管周围病变在IgG4-RD患者中分布广泛。这些患者的血清CRP水平不一定升高。类固醇治疗对IgG4-RD有效，可使病变消退。
