Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan.
Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan.
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DOI：
10.1155/2012/357071
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发表时间：
2012
影响因子：
2.3
通讯作者：
Umehara H
中科院分区：
文献类型：
--
作者：
Okazaki K;Umehara H
最近的研究表明，同时或异时性病变的多器官的特点是血清IgG 4水平升高和大量的IgG 4阳性浆细胞浸润，不同程度的纤维化。日本厚生劳动省“难治性疾病研究计划”支持的两个IgG 4-RD日本研究委员会，一个来自纤维硬化（Okazaki团队），另一个来自淋巴增生（Umehara团队），已同意统一命名为“IgG 4-RD”，并提出了IgG 4-RD的综合诊断标准（CDC）。CDC的验证表明，对于普通医生和非专业人员的实际使用，灵敏度令人满意，但与IgG 4相关的涎腺炎/泪腺炎（Mikulicz病）和IgG 4相关的肾脏疾病相比，在难以获取活检标本的器官中，如1型自身免疫性胰腺炎（IgG 4相关的AIP）的灵敏度较低。虽然难以建立涵盖所有IgG 4-RD的诊断标准，但结合CDC和器官特异性诊断标准应提高灵敏度。
Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.
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