IgG4-Related Disease
IgG4-Related Disease
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DOI：
10.1007/978-3-319-99139-9_4
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发表时间：
2018-10
期刊：
影响因子：
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通讯作者：
Tom Foster;Carmen Bodlak
中科院分区：
文献类型：
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作者：
Tom Foster;Carmen Bodlak
IgG4-related disease (IgG4-RD) is a fibro-inflammatory disease that is characterized by elevation of the serum IgG4 level and organ swelling and can involve essentially any organ simultaneously or metachronously. Since IgG4-RD mimics several malignant or inflammatory diseases, it is critical to differentiate IgG4-RD from malignant tumors and similar inflammatory diseases of the affected organ in an accurate and timely manner to avoid misdiagnosis and apply appropriate therapy. Characteristic histopathological findings of IgG4-RD are abundant infiltration of lymphocytes and IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. The diagnosis of IgG4-RD is a significant clinical challenge. Since there is no simple diagnostic test for IgG4-RD, it is diagnosed by a combination of clinical, imaging, serological, and pathological findings; other organ involvement; and steroid responsiveness. IgG4-RD responds well to steroids, but it sometimes relapses. Rituximab has recently been reported to be useful for its treatment. Although the short-term outcome is usually good, the long-term prognosis is unknown.
