Progress in diagnosis and treatment of IgG4-related disease
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摘要: IgG4 相关性疾病是一种与IgG4 淋巴细胞密切相关的慢性、系统性疾病，该类疾病以血清IgG4 水平升高以及IgG4 阳性细胞浸润多种器官和组织为特征，病理学改变呈现为席纹状纤维化，累及的器官或组织由于慢性炎症及纤维化进程可导致弥漫性肿大，常见受累器官包括泪腺、胰腺和腹膜后间隙等。该类疾病对皮质激素治疗反应良好。文中我们回顾了IgG4 相关性疾病前阶段的诊断标准，并补充不常见受累器官的国际诊断标准，更新各个诊断标准的国际共识，认为免疫抑制剂、生物制剂将成为治疗的新进展。Abstract: IgG4-related disease is a kind of chronic and systemic disease which is characterized by increased serum level of IgG4 and abundant IgG4-bearing plasma cell infiltration, storiform fibrosis pathological features in the involved organs.Affected organs or tissues may show a diffuse swelling as a result of chronic inflammatory process, accompanied with progressive fibrosis. The most commonly involved organs or tissues include the salivary glands, pancreas, and retroperitoneal space and so on. A good response to glucocorticoid treatment. In this article, we reviewed before stage diagnostic standards about IgG4-related disease, add international diagnostic standards of uncommon affected organs，update diagnostic standards of international consensus，think the immune inhibitors and biological agents will become the new progress of treatment.
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Key words:
- IgG4-related disease /
- diagnosis /
- treatment /
- progress
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表 1 2008 年日本干燥综合征学会 IgG4 相关性 MD 的诊断标准
(1) 泪腺、腮腺及颌下腺中腺体对称性肿大（≥2 对腺体，≥3 个月）；血清 IgG4 浓度的增高（≥135 mg/dl）； 并且 (2) 血清中 IgG4≥135 mg/dl； 或者 (3) 组织病理学检测显示：组织学显示大量淋巴细胞和浆细胞浸润与纤维化（IgG4+浆细胞/IgG+浆细胞>50%）。 
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表 2 2011 年美国学者的诊断标准
(1) 肾小管间质内可见大量的IgG+浆细胞浸润，IgG4+浆细胞数量>10 个/HPF； (2) 肾小管基底膜增厚，在电镜、免疫组化或免疫荧光下可见免疫复合物的沉积； (3) 影像学可见双肾皮质区可见小灶性、楔形或弥漫性低密度影，甚则累及整个肾脏； (4) 血清中IgG4或IgG水平增高； (5) 其他脏器受累，如 AIP、SC、唾液腺炎、大动脉炎、RPF 或炎性假瘤。
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表 3 2011 年日本学者也提出建议诊断标准
(1) 肾功能异常伴有 IgG 升高,或补体降低,或 IgE 水平升高; (2) 影像学中,增强 CT 可见低密度灶,弥漫性肿大､单发性肾肿瘤､肾盂表明的增厚; (3) 血清中 IgG4≥135 mg/dl; (4) 肾脏组织学显示 ①IgG4+浆细胞/IgG+浆细胞比≥40%或 IgG4+浆细胞>10 个/HP ②淋巴细胞和浆细胞浸润区有席纹装纤维化改变; (5) 肾外组织学检查显示浆细胞密集区 IgG4 +细胞>10 个/HPF,IgG4+/IgG+浆细胞>40%｡
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