Clinical features of patients with IgG4-related disease complicated with perivascular lesions
Clinical features of patients with IgG4-related disease complicated with perivascular lesions
复制标题
DOI：
10.3109/14397595.2014.903596
复制
发表时间：
2015-01-01
影响因子：
2.2
通讯作者：
Sumida, Takayuki
中科院分区：
文献类型：
--
作者：
Ebe, Hiroshi;Tsuboi, Hiroto;Sumida, Takayuki
目标。目的探讨IgG4相关性疾病(IgG4-RD)合并血管周围病变的临床特点。对2008年10月至2013年10月在筑波大学医院就诊的7例IgG4-RD及血管周围病变患者的临床资料进行分析，包括临床背景、影像检查结果、对2011年综合诊断标准(CDC)的满意度、实验室资料、血管周围病变的分布、其他器官受累及激素治疗反应。我们研究了6名男性和1名女性，他们的平均年龄为66.9+/-6.7岁(+/-SD)。7例中6例确诊为IgG4-RD，7例根据疾病预防控制中心诊断为可能为IgG4-RD。所有7名患者确诊时血清IgG4水平均高于135 mg/dl(平均933+/-527)。仅2例患者血清C反应蛋白(CRP)水平升高(平均为1.42+/-3.56 mg/dl)。病变位于肺动脉1例，胸主动脉2例，腹主动脉6例，冠状动脉1例，腹主动脉1例，肠系膜上动脉1例，肾动脉2例，肠系膜下动脉5例，髂动脉3例。除血管周围损害外，6名患者还表现出其他器官受累。7例患者均给予强的松龙(0.6 mg/kg/d)治疗，其中6例患者血管周围及其他脏器病变迅速改善(另1例患者因随访时间较短尚未评估)。IgG4-RD患者血管周围病变广泛分布。这些患者的血清CRP水平并不一定升高。激素治疗对IgG4-RD是有效的，并导致皮损的消退。
Objective. To define the clinical features of IgG4-related disease (IgG4-RD) complicated with perivascular lesions.Methods. 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.Results. 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).Conclusion. 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.