Nambam Bimota, Winter William E, Schatz Desmond A
aDepartment of Pediatrics, Division of Pediatric Endocrinology bDepartments of Pathology, Immunology and Laboratory Medicine, Pediatrics, and Molecular Genetics and Microbiology, University of Florida, Gainesville, Florida, USA.
Curr Opin Pediatr. 2014 Aug;26(4):493-9. doi: 10.1097/MOP.0000000000000107.
This review discusses the IgG4-related disease spectrum (IgG4-RD), the autoimmune polyglandular syndromes (APS), the association of IgG4 with APS, and possible pathobiology.
IgG4-RD is a multiorgan autoimmune disorder characterized by fibrous inflammation, IgG4-positive plasma cell infiltration in affected tissues, and elevated serum concentrations of IgG4. IgG4-RD can affect any organ and has a heterogeneous presentation. Consensus criteria for diagnosis in specific organs have been established. The recognition and diagnosis of IgG4-RD are crucial because the disease responds favorably to immunosuppression (e.g., glucocorticoids, rituximab). The precise mechanisms leading to disease are unknown, but IgG4 antibodies may undergo a half antibody exchange, which renders them incapable of activating the complement pathway.
Despite significant advances in disease recognition and treatment strategies, the disorder remains poorly understood. The precise role of IgG4, whether it is protective or pathogenic, is still being debated.
本综述讨论IgG4相关疾病谱（IgG4-RD）、自身免疫性多腺体综合征（APS）、IgG4与APS的关联以及可能的病理生物学机制。
IgG4-RD是一种多器官自身免疫性疾病，其特征为纤维性炎症、受累组织中IgG4阳性浆细胞浸润以及血清IgG4浓度升高。IgG4-RD可累及任何器官，表现多样。已制定了特定器官的诊断共识标准。IgG4-RD的识别和诊断至关重要，因为该疾病对免疫抑制（如糖皮质激素、利妥昔单抗）反应良好。导致该疾病的确切机制尚不清楚，但IgG4抗体可能会发生半抗体交换，使其无法激活补体途径。
尽管在疾病识别和治疗策略方面取得了重大进展，但对该疾病仍知之甚少。IgG4的确切作用，无论是保护性还是致病性，仍在争论中。
