IgG4相关性疾病

1、IgG4-reIated diseaseCONTENTSConcept and history1 Features 2Diagnosisi3Treament4Conclution and discussion5Concepta systemic diseaseAextensive IgG4一一positiveBT lymphocyte infiltrationCvarious organsD autoimmune disease EHistory:1995 Yoshida autoimmune pancreatitis(AIP)A2001 AIP relationship with IgG4

2、B2003 Kamisawa IgG4 Positive multiple organ lymphocyte proliferation syndromeC2010 Autoimmun Rev New diseaseDFeatures1.tumor-like swelling of involved organs2.lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells3. a variable degree of fibrosis that has a characteristic “storiform” pat

3、tern4.serum concentrations of IgG4 are found5.The majority of patients respond to glucocorticoids, particularly in early stages of disease.1.自身免疫性垂体炎自身免疫性垂体炎2.眼眶炎性假瘤眼眶炎性假瘤3.MUKULICZS 病病4.慢性硬化性颌下腺炎慢性硬化性颌下腺炎5.桥本氏甲状腺炎桥本氏甲状腺炎6.慢性纤维性甲状腺炎慢性纤维性甲状腺炎7.间质性肺炎间质性肺炎8.自身免疫性胰腺炎自身免疫性胰腺炎9.硬化性胆管炎硬化性胆管炎10.间质性肾小管肾炎间质性肾

4、小管肾炎11.腹膜后纤维化腹膜后纤维化12.淋巴浆细胞性淋巴浆细胞性主动脉炎主动脉炎13.炎性主动脉瘤炎性主动脉瘤14.嗜酸细胞纤维化嗜酸细胞纤维化15.炎性假瘤炎性假瘤16.前列腺炎前列腺炎17.皮肤假性淋巴瘤皮肤假性淋巴瘤18.巨淋巴结病性窦组织细胞增生症巨淋巴结病性窦组织细胞增生症High risk for having IgG4-RD:Pancreatitis of unknown originSclerosing cholangitisBilateral salivary and/or lacrimal gland enlargementRetroperitoneal fibrosisOrbital pseudotumor or proptosisDIAGNOSIS:Treament No standard treatment. Some IgG4 RD was sensitive to glucocorticoid. Needs Attention: 1.Other disease. 2.Contraindication.conclusion: 1.IgG4 RD is a systemic disease,extensive IgG4 a