机构地区:[1]北京大学第三医院风湿免疫科,北京100191 [2]辽宁省健康产业集团本钢总医院风湿免疫科,辽宁本溪117000
出 处:《北京大学学报(医学版)》2023年第6期1028-1032,共5页Journal of Peking University:Health Sciences
摘 要:目的:了解临床表现复杂且容易被误诊和漏诊的IgG4相关性疾病(IgG4-related disease,IgG4-RD)患者的就诊情况及临床特征,以提高相关科室医师对IgG4-RD的认识。方法:选择2012年1月1日至2022年12月31日于北京大学第三医院住院且出院诊断为IgG4-RD患者的病例资料进行回顾性分析,总结患者就诊情况、临床表现、实验室检查、诊断及治疗情况。结果:共纳入出院诊断为IgG4-RD的病例116例,男女比例为2.52∶1,平均年龄(61.83±10.80)岁。首诊科室以消化科、普外科、眼科为主,诊断科室主要为消化科、风湿免疫科、呼吸科,21例(18.10%)患者经过3个或者3个以上科室诊断后才最终确诊。从出现临床症状到首次就诊中位时间为2(1,7)个月,诊断中位时间为1(1,12)个月。24例(20.69%)患者在最终诊断前进行了受累部位的手术切除。依据IgG4-RD的分类标准,确定诊断68例(58.62%),可能诊断8例(6.90%),可疑诊断40例(34.48%)。68例确定诊断的患者中,常见受累器官依次为颌下腺、胰腺、胆道、腮腺,血清IgG4水平的中位数为6.16(3.61,12.30)g/L,57例(83.82%)患者使用了糖皮质激素,14例(20.59%)患者使用了免疫抑制剂,使用免疫制剂者主要为风湿免疫科诊治患者(78.57%)。结论:IgG4-RD好发于老年男性,颌下腺、胰腺、胆道、腮腺易受累,首诊科室分布广泛,通过获取病理组织标本而确定诊断的比例较低,治疗上主要以激素为主,免疫抑制剂的使用率不高。Objective:To understand the medical treatment and clinical characteristics of patients with IgG4-related disease(IgG4-RD)with complex clinical manifestations and easy to be misdiagnosed and missed,and to improve the recognition of this disease among doctors from relevant medical departments.Methods:A retrospective analysis was conducted on the medical records of patients diagnosed with IgG4-RD who were hospitalized and discharged from Peking University Third Hospital from January 1,2012 to December 31,2022.The patient’s medical visit status,clinical manifestations,laboratory examinations,diagnosis,and treatment information were summarized.Results:A total of 116 patients diagnosed with IgG4-RD were included in this study,with a male to female ratio of 2.52∶1 and an average age of(61.83±10.80)years.The departments for initial visits were gastroenterology,general surgery,and ophthalmology.While the departments responsible for definitive diagnosis were gastroenterology,rheumatology and immunology,and respiratory medicine.Twenty-one patients(18.10%)required consultation and treatment from three or more departments before receiving a definitive diagnosis.The median time from symptom onset to the initial clinic visit was 2(1,7)months,and the median time from symptom onset to diagnosis was 1(1,12)month.Twenty-four patients(20.69%)underwent surgical resection of the affected sites before diagnosis.According to the classification criteria of IgG4-RD,sixty-eight(58.62%)cases were diagnosed definitively,eight(6.9%)cases were likely to be diagnosed,and 40(34.48%)cases were suspected to be diagnosed.In the 68 definitively diagnosed patients,the most commonly affected organs were submandibular gland,the pancreas,biliary tract,parotid in sequence.The median serum IgG4(IgG4,immunoglobulin G4)level was 6.16(3.61,12.30)g/L.Fifty-seven patients(83.82%)were treated with glucocorticoids,and 14 patients(20.59%)were treated with immunosuppressants.The use of immunosuppressants was mainly in the rheumatology and immunology departmen
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