Post Graduate Clinical Education Center, Japanese Red Cross Akita Hospital, Akita, Japan.
Department of Respiratory Medicine, Japanese Red Cross Akita Hospital, Akita, Japan.
Medicine (Baltimore). 2023 Jul 28;102(30):e34492. doi: 10.1097/MD.0000000000034492.
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.
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.
Vasculitis can present with lesions and pathological findings similar to those of IgG4-RD.
血管炎通常不会引起腺体组织损伤。在此，我们报告一例血管炎病例，其分布类似于 IgG4 相关疾病（IgG4-RD），包括颌下腺和泪腺以及主动脉旁炎。
一名 70 岁男性因呼吸急促就诊。实验室检查发现 IgG4 水平为 191mg/dL，抗中性粒细胞胞质抗体检测阴性，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颌下腺和泪腺增大。颈部至骨盆计算机断层扫描显示双肺下叶双侧浸润性阴影、双肺肿块影和腹主动脉至髂总动脉的主动脉旁炎。因此，患者被诊断为 IgG4 相关呼吸疾病和主动脉旁炎/动脉炎。给予泼尼松龙 35mg 剂量（0.6mg/kg 每日）。在观察治疗效果的同时逐渐减少剂量。影像学检查结果表明有所改善，C 反应蛋白和 IgG4 水平下降，表明治疗过程成功。然而，在重新检查病理发现后，诊断从 IgG4-RD 更改为血管炎。治疗开始一年后，患者症状稳定。
血管炎可表现出与 IgG4-RD 相似的病变和病理发现。
