IgG4相关性甲状腺疾病9例临床特征分析

Clinical Characteristics of 9 Patients with IgG4-related Thyroid Disease

目的分析IgG4相关性甲状腺疾病(IgG4-RTD)患者的临床特征及诊疗经过。方法纳入2014年1月~2019年4月北京协和医院住院患者,依据穿刺活检或手术病理诊断为IgG4-RTD患者,回顾性分析患者人口统计学特征、甲状腺病变特点、实验室检查及治疗情况。结果9例IgG4-RTD患者中,男性2例(22.2%),女性7例(77.8%),平均诊断年龄46.56±10.30岁。3例(33.3%)患者为单发甲状腺受累,另外6例(66.7%)为多病灶受累。9例患者均存在不同程度的甲状腺弥漫性病变及肿大,其中2例(22.2%)患者因甲状腺肿大存在压迫症状。TGAb及TPOAb增高的患者分别为7例及6例,7例患者诊断时处于甲状腺功能减退状态;6例患者应用糖皮质激素治疗,起始剂量为30~50mg/d。治疗后血清IgG4水平下降程度为77.1%。4例患者糖皮质激素减量后出现IgG4水平反复波动性升高。结论IgG4-RTD多为IgG4相关性疾病的全身性受累的一部分。最常见的表现为甲状腺肿大,严重时可伴压迫症状,甲状腺自身抗体高效价增高以及甲状腺功能减退症。糖皮质激素治疗IgG4-RTD有效。

Objective To discuss the clinical characteristics and treatment of IgG4-related thyroid disease(IgG4-RTD).Methods IgG4-RTD patients with histopathology-provent were recruited from Peking Union Medical Hospital during January 2014 to April 2019.The demographic characteristics,thyroid involvements,laboratory examinations and treatment efficacy were retrospectively studied.Results A total of 9 patients were finally enrolled,including 7 females(78.8%)and 2 males(22.2%).The mean age of diagnosis was 46.56±10.30 years old.Six patients(66.7%)had multi-organ involvement and 3 patients(33.3%)only with single thyroid glands disease.All patients showed diffuse thyroid with ultrasound,2 patients(22.2%)of had compression symptoms due to thyroid enlargement.There were 6 cases(66.7%)and 7 cases(77.8%)with increased TPOAb and TGAb,respectively.Seven patients(77.8%)were in hypothyroidism at the time of diagnosis.There were 6 patients(66.7%)onset treated with glucocorticoids with 30-50mg/d.The serum IgG4 level had dropped by 77.1%.After medication reduction,4 patients(66.7%)of which developed relapse.Conclusion IgG4-RTD is usually part of the multi-organ involvement of IgG4-related disease.The most common clinical manifestation is thyroid enlargement,which causes compression symptoms in severe cases,high titer of thyroid autoantibodies and hypothyroidism.Glucocorticoids were effective for IgG4-RTD.