PDF(Pubmed)
Abstract：
BACKGROUND: Vasculitis does not usually induce glandular tissue lesions. Herein, we present a case of vasculitis with a distribution similar to that of immunoglobulin-G4-related disease (IgG4-RD), including that of the submandibular and lacrimal glands, and periaortitis.
METHODS: A 70-year-old man presented with shortness of breath. Laboratory findings revealed an IgG4 level of 191 mg/dL, negative antineutrophil cytoplasmic antibody test, and C-reactive protein level of 8.33 mg/dL. Magnetic resonance imaging of the head and computed tomography of the neck revealed bilaterally enlarged submandibular and lacrimal glands. Neck-to-pelvis computed tomography revealed bilateral infiltrative shadows in the lower lobes of both lungs, mass shadows in both lungs, and periaortitis of the abdominal aorta extending to the common iliac artery. Thus, the patient was diagnosed with IgG4-related respiratory disease and periaortitis/periarteritis. Prednisolone was administered at a dose of 35 mg (0.6 mg/kg daily). The dose was gradually tapered while observing the effects of the treatment. Imaging findings indicated an improvement and the C-reactive protein and IgG4 levels decreased, indicating a successful treatment course. However, after reexamination of the pathological findings, the diagnosis changed from IgG4-RD to vasculitis. One year after treatment initiation, the patient symptoms have stabilized.
CONCLUSIONS: Vasculitis can present with lesions and pathological findings similar to those of IgG4-RD.
METHODS: A 70-year-old man presented with shortness of breath. Laboratory findings revealed an IgG4 level of 191 mg/dL, negative antineutrophil cytoplasmic antibody test, and C-reactive protein level of 8.33 mg/dL. Magnetic resonance imaging of the head and computed tomography of the neck revealed bilaterally enlarged submandibular and lacrimal glands. Neck-to-pelvis computed tomography revealed bilateral infiltrative shadows in the lower lobes of both lungs, mass shadows in both lungs, and periaortitis of the abdominal aorta extending to the common iliac artery. Thus, the patient was diagnosed with IgG4-related respiratory disease and periaortitis/periarteritis. Prednisolone was administered at a dose of 35 mg (0.6 mg/kg daily). The dose was gradually tapered while observing the effects of the treatment. Imaging findings indicated an improvement and the C-reactive protein and IgG4 levels decreased, indicating a successful treatment course. However, after reexamination of the pathological findings, the diagnosis changed from IgG4-RD to vasculitis. One year after treatment initiation, the patient symptoms have stabilized.
CONCLUSIONS: Vasculitis can present with lesions and pathological findings similar to those of IgG4-RD.
摘要：
背景：血管炎通常不会引起腺体组织病变。在这里，我们介绍了一例血管炎，其分布与免疫球蛋白G4相关疾病（IgG4-RD）相似，包括颌下腺和泪腺，和主动脉炎。
方法：一名70岁男子出现呼吸急促。实验室发现IgG4水平为191mg/dL，抗中性粒细胞胞浆抗体试验阴性，C反应蛋白水平为8.33mg/dL。头部磁共振成像和颈部计算机断层扫描显示双侧下颌下腺和泪腺扩大。颈部至骨盆计算机断层扫描显示双肺下叶双侧浸润阴影，双肺的大量阴影，和延伸至髂总动脉的腹主动脉周围炎。因此，患者被诊断为IgG4相关呼吸系统疾病和主动脉炎/大动脉周炎.泼尼松龙以35mg(每日0.6mg/kg)的剂量施用。在观察治疗效果的同时逐渐减少剂量。影像学表现为改善，C反应蛋白和IgG4水平下降，表明一个成功的治疗过程。然而,在重新检查病理结果后，诊断由IgG4-RD转变为血管炎.治疗开始一年后，病人症状稳定了.
结论：血管炎可表现为与IgG4-RD相似的病变和病理结果。
方法：一名70岁男子出现呼吸急促。实验室发现IgG4水平为191mg/dL，抗中性粒细胞胞浆抗体试验阴性，C反应蛋白水平为8.33mg/dL。头部磁共振成像和颈部计算机断层扫描显示双侧下颌下腺和泪腺扩大。颈部至骨盆计算机断层扫描显示双肺下叶双侧浸润阴影，双肺的大量阴影，和延伸至髂总动脉的腹主动脉周围炎。因此，患者被诊断为IgG4相关呼吸系统疾病和主动脉炎/大动脉周炎.泼尼松龙以35mg(每日0.6mg/kg)的剂量施用。在观察治疗效果的同时逐渐减少剂量。影像学表现为改善，C反应蛋白和IgG4水平下降，表明一个成功的治疗过程。然而,在重新检查病理结果后，诊断由IgG4-RD转变为血管炎.治疗开始一年后，病人症状稳定了.
结论：血管炎可表现为与IgG4-RD相似的病变和病理结果。
