Yamamoto Hideya, Sugiyama Eiji, Serikawa Masahiro, Tatsugami Fuminari, Yokozaki Michiya, Kitagawa Toshiro, Senoo Atsushi, Nakamoto Yumiko Kubo, Awai Kazuo, Chayama Kazuaki, Kihara Yasuki
Department of Cardiovascular Medicine, Hiroshima University Graduate School of Biomedical and Health Sciences, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan.
Heart Vessels. 2017 Oct;32(10):1176-1185. doi: 10.1007/s00380-017-0987-2. Epub 2017 May 4.
Vascular and/or perivascular involvements of sclerotic inflammation (perivasculitis) are a complication of immunoglobulin G4-related disease (IgG4-RD). We sought to examine clinical manifestations of perivasculitis by computed tomography (CT) in patients with elevated serum IgG4 levels, and then to evaluate some potential predictors of perivasculitis in definite IgG4-RD patients. From a database of patients with serum IgG4 measurements, we selected 81 patients with elevated serum IgG4 levels (≥135 mg/dl). Perivasculitis was defined radiologically as thickened contrast-enhanced rind surrounding the aorta and its major artery on CT imaging. We found 15 patients with perivasculitis; 10 patients in the definite (n = 37), four in the possible (n = 18), and one in the excluded (n = 26) IgG4-RD groups. Clinical predictors of perivasculitis were investigated in 34 untreated patients with definite IgG4-RD. Patients with perivasculitis (n = 10) had significantly higher age at diagnosis (74.2 ± 8.8 vs 63.5 ± 9.9 years, P = 0.006), higher levels of serum IgG4 (754 vs 292 mg/dl, P = 0.007) and C-reactive protein (CRP, 0.52 mg/dl vs 0.10 mg/dl, P = 0.001) than patients without perivasculitis (n = 24). The sensitivity and specificity of serum CRP ≥0.25 mg/dl for identifying perivasculitis in the definite IgG4-RD group were 100 and 71%, respectively (area under the receiver operating characteristic curve 0.863). Our results indicate that IgG4-related perivasculitis was associated with elevated levels of serum CRP and older age, and that CRP may be a useful marker for detecting perivascular involvement in IgG4-RD.
硬化性炎症的血管和/或血管周围受累（血管周炎）是免疫球蛋白G4相关疾病（IgG4-RD）的一种并发症。我们试图通过计算机断层扫描（CT）检查血清IgG4水平升高患者的血管周炎临床表现，然后评估确诊IgG4-RD患者血管周炎的一些潜在预测因素。从血清IgG4检测患者数据库中，我们选择了81例血清IgG4水平升高（≥135mg/dl）的患者。血管周炎在影像学上定义为CT成像时围绕主动脉及其主要动脉的强化增厚包壳。我们发现15例血管周炎患者；确诊IgG4-RD组（n = 37）中有10例，可能IgG4-RD组（n = 18）中有4例，排除IgG4-RD组（n = 26）中有1例。在34例未经治疗的确诊IgG4-RD患者中研究了血管周炎的临床预测因素。血管周炎患者（n = 10）诊断时的年龄显著高于无血管周炎患者（n = 24）（74.2±8.8岁 vs 63.±9.9岁，P = 0.006），血清IgG4水平（754 vs 292mg/dl，P = 0.007）和C反应蛋白（CRP，0.52mg/dl vs 0.10mg/dl，P = 0.001）更高。血清CRP≥0.25mg/dl在确诊IgG4-RD组中识别血管周炎的敏感性和特异性分别为100%和71%（受试者工作特征曲线下面积为0.863）。我们的结果表明，IgG4相关血管周炎与血清CRP水平升高和年龄较大有关，并且CRP可能是检测IgG4-RD血管周围受累的有用标志物。
