背景:免疫球蛋白G4(IgG4)相关疾病(IgG4-RD)是一种全身性免疫介导的纤维炎症性疾病,可导致多个器官的组织破坏。IgG4-RD常被误诊为恶性,传染性,或其他炎症性疾病。
方法:我们描述了一名56岁女性出现黄疸和体重减轻。影像学表现为胰腺常见肝管壁增厚和结节状病变,高度怀疑恶性肿瘤。然而,她是活检的禁忌;因此,IgG4-RD的诊断是基于她的高血清IgG4水平,多器官受累,和类固醇反应。类固醇治疗的效果是显著的,尽管疾病在维持治疗期间复发。类固醇的剂量重新增加,患者正在接受密切随访。
结论:IgG4-RD的诊断由于其表现多样而具有挑战性。因此,仔细的系统评估对于提高IgG4-RD诊断的准确性是必要的,密切随访对于监测疾病发展以及相应调整治疗策略非常重要。
BACKGROUND: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory disease that results in the tissue destruction of multiple organs. IgG4-RD is often underdiagnosed or misdiagnosed as malignant, infectious, or other inflammatory disorder.
METHODS: We describe a 56-year-old woman presented with jaundice and weight loss. Radiological imaging showed common hepatic duct wall thickening and nodular lesions in the pancreas, which was highly suspicious of malignancy. However, she was contraindicated for biopsy; hence, the diagnosis of IgG4-RD was made based on her high serum IgG4 level, multiorgan involvement, and steroid response. The effect of steroid therapy was significant, although the disease relapsed during the maintenance treatment. The dosage of steroid was re-increased, and the patient was under close follow-up.
CONCLUSIONS: The diagnosis of IgG4-RD is challenging due to its diverse manifestations. Therefore, a careful systematic assessment is necessary to improve the accuracy of IgG4-RD diagnosis, and a close follow-up is important to monitor the disease development as well as adjust the treatment strategy accordingly.