Department of Diagnostic Imaging and Nuclear Medicine, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto, Japan.
Clin Radiol. 2012 Apr;67(4):297-305. doi: 10.1016/j.crad.2011.10.011. Epub 2011 Nov 26.
IgG4-related systemic disease (IgG4-RSD) is an emerging clinical entity about which much remains to be elucidated, in terms of its aetiology, pathogenesis, diagnosis, treatment and outcome. Autoimmune pancreatitis (AIP) and Mikulicz disease (MD) are the two major, well-studied constituents of IgG4-RSD. AIP and MD have common characteristics of forming tumour-mimicking lesions that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous immunoglobulin G4 (IgG4)-positive plasma cells, as well as various multi-organ manifestations of IgG4-RSD. 2-[(18)F]-fluoro-2-deoxy-d-glucose positron-emission tomography/ computed tomography (FDG PET/CT) enables the acquisition of whole-body images and provides functional information about disease activity; as such it has a valuable role in staging extent of disease, guiding biopsy, and monitoring response to treatment. However, FDG PET/CT is likely to be only one component of the management strategy, and clinical, laboratory, imaging and histological findings are crucial in the overall diagnosis of the condition. At present FDG PET/CT does not have a well-established role in the assessment of patients with IgG4-RSD and future prospective studies are required to define the cost-effectiveness and clinical impact in this patient group more accurately.
IgG4 相关系统性疾病（IgG4-RSD）是一种新兴的临床实体，其病因、发病机制、诊断、治疗和预后仍有许多未解之谜。自身免疫性胰腺炎（AIP）和米库利茨病（MD）是 IgG4-RSD 中两个主要的、研究充分的组成部分。AIP 和 MD 具有形成肿瘤样病变的共同特征，这些病变由淋巴浆细胞浸润和纤维化组成，伴有大量 IgG4 阳性浆细胞，以及 IgG4-RSD 的各种多器官表现。2-[(18)F]-氟-2-脱氧-d-葡萄糖正电子发射断层扫描/计算机断层扫描（FDG PET/CT）能够获取全身图像，并提供有关疾病活动的功能信息；因此，它在分期疾病程度、指导活检和监测治疗反应方面具有重要作用。然而，FDG PET/CT 可能只是管理策略的一个组成部分，临床、实验室、影像学和组织学发现对疾病的整体诊断至关重要。目前，FDG PET/CT 在评估 IgG4-RSD 患者方面尚未发挥明确作用，需要进一步的前瞻性研究来更准确地确定该患者群体的成本效益和临床影响。
