Proceedings of the Japan Academy, Series B. Physical and Biological Sciences | |
Current perspectives on autoimmune pancreatitis and IgG4-related disease | |
Kazuichi OKAZAKI1  | |
[1] Department of Gastroenterology and Hepatology, Kansai Medical University | |
关键词: IgG4-related disease; IgG4; autoimmune pancreatitis; | |
DOI : 10.2183/pjab.94.027 | |
学科分类:物理(综合) | |
来源: Japan Academy | |
【 摘 要 】
IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder recognized as a novel clinical entity with either synchronous or metachronous multi-organ involvement. Patients with IgG4-RD show diffuse or focal organ enlargement and mass-forming or nodular/thickened lesions with abundant infiltration of IgG4-positive plasmacytes and fibrosis, and such patients respond well to steroid treatment. It should be differentiated from mimics by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line drug is corticosteroids, or rituximab in high-risk patients for steroid intolerance. Although relapse rates are high, standardized protocols for relapsed cases have not been approved yet. Based on genetic factors, disease-specific or -related antigens, abnormal innate and adaptive immunity may be involved, although the precise pathogenic mechanism and long-term outcome still remain unclear.
【 授权许可】
Unknown
【 预 览 】
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RO201910253525301ZK.pdf | 5307KB | download |