IgG4相关性疾病60例临床分析

Clinical analysis of IgG4-related disease:A study of 60 cases

目的通过对IgG4相关性疾病(IgG4-RD)的病例进行临床分析提高对本病的诊治水平。方法回顾性分析60例IgG4-RD病例的一般特征、临床表现、实验室及病理检查等。结果男女比例2.75∶1,年龄(58.72±14.11)岁。起病至诊断时间(21.77±44.22)个月。曾被诊断为肿瘤15例(25.0%)。仅4例在风湿科诊断本病。最常见受累器官有胆道31例(51.7%)、淋巴结31例(51.7%)、胰腺25例(41.7%);其次常见肺、胸膜、肝脏、肾脏、唾液腺、鼻或鼻窦、眼部、胃肠、输尿管、腹膜后纤维化。每个患者受累器官3个以上者占61.7%,2个以上占91.7%。血IgG4(9.31±8.39)g/L,不升高者11.7%。肾损害者C3水平显著低于无肾损害者。IgG4-RD反应指数12.5(9,15)。41例行病理检查,淋巴浆细胞浸润35例(85.4%),嗜酸细胞浸润7例(17.1%),纤维化21例(51.2%),确诊及可能性大者IgG4+细胞超过10个/HP,IgG4+/IgG+细胞比例超过40%。大部分患者用糖皮质激素后好转。结论 IgG4相关性疾病是一种累及多器官的免疫介导的慢性炎症伴纤维化疾病,易误诊为肿瘤,病理是诊断的重要手段,本病早期正确诊断有赖于提高各科医生对本病的认识。

Objective Toanalyze clinical characteristics of IgG4-related diseases(IgG4-RD) and improve the diagnosis and treatment.Methods The data of 60 IgG4-RD cases were analyzed retrospectively. Results The proportion of males and females was 2.75 : 1,and the age was 58.72±14.11.Time from onset to diagnosiswas21.77 ± 44.22 months. 15 cases(25.0%) were oncediagnosed as tumors. Only 4 cases acquired diagnosis of IgG4-RD in rheumatology department.The most common affected organs were biliary tract( 3 lcases, 51.7%), lymph nodes(31 cases, 51.7%), and pancreas(25 cases, 41.7%), following by lung, pleura, liver, kidney, salivary gland,nasal or sinus, eye, gastrointestinal, ureter, and retroperitoneal fibrosis. More than 3 organs affected were found in 37 patients(61.7%) and more than 2 organs were in 55 patients(91.7%). Average serum IgG4 was 9.31 g/L,while 7 patients( 11.7%) with no increase of serum IgG4. C3 level of patients with kidney involvement was significantly lower than those without kidney damage. IgG4-RD responder index was 12.5( 9, 15).In 41 patients with histopathological examination, lymphoplasmacytic infiltration was seen in 35 cases(85.4%), eosinophil infiltration in 7 cases( 17.1%), and fibrosis in 21 cases(51.2%). in 29 patients there were more than 10 IgG4+ cells/HP,and ratio of IgG4+/IgG+ cells exceeded 40%. Most of cases who had used glucocorticoids improved and their IgG4 decreased. Conclusions IgG4-RDisan immune-mediated chronic disease with inflammation and fibrosis involving multiple organs.It is easily misdiagnosed as a tumor. Pathology is important fordiagnosis. Early diagnosis depends on improving doctors-awareness of disease. The more number of affected organs, the shorter time before diagnosis.