选择性IgA缺乏症并发自身免疫现象的临床特征

Clinical characteristics of selective IgA deficiency complicated with autoimmune phenomena

目的探讨选择性IgA缺乏症(selective IgA deficiency,SIgAD)并发自身免疫现象(autoimmune phenomena,AP)的临床特征与疾病转归。方法回顾性分析1999年10月至2019年10月于中国医学科学院北京协和医院住院的SIgAD患者33例,其中并发AP患者(SIgAD-AP)14例(42.4%),总结其临床症状、免疫学检查及疾病转归的特点,与未并发AP的SIgAD患者(SIgAD-nAP)进行比较。结果SIgAD患者中4例发生肿瘤,有8例抗核抗体阳性。SIgAD-AP组男∶女比例3∶4,7例患者确诊AP时间早于SIgAD,诊断SIgAD和AP的中位年龄分别为22.5(8.3,34.5)岁和16.5(8.3,29.5)岁。SIgAD-AP中AP表现多样,常见AP为系统性红斑狼疮(4例)、幼年型特发性关节炎(2例)、白癜风(2例)。SIgAD-AP组和SIgAD-nAP组并发感染无明显差异。SIgAD-AP组中1例患者并发肺癌死亡,SIgAD-nAP组中2例并发肿瘤患者死亡,余长期稳定。结论SIgAD并发AP和/或自身抗体者并不少见且AP表现多样,对SIgAD患者进行病情评估时需注意筛查AP。

Objective To investigate the clinical characteristics and outcomes of selective IgA deficiency(SIgAD)complicated with autoimmune phenomena(AP).Methods A total of 33 patients with SIgAD admitted to Peking Union Medical College Hospital(PUMCH)from October 1999 to October 2019 were retrospectively analyzed.Among them,14 patients(42.4%)were diagnosed of SIgAD-AP.The clinical characteristics,immunological tests,and outcomes of SIgAD-AP patients were summarized and compared to those without AP(SIgAD-nAP).Results Eight patients(24.2%)of this SIgAD cohort presented positive anti-nuclear antibodies.In SIgAD-AP group,the gender ratio of F∶M was 4∶3,and the median age of SIgAD diagnosis and AP diagnosis were 22.5(8.3,34.5)years old and 16.5(8.3,29.5)years old,respectively.The most common AP were systemic lupus erythematosus(4 cases),juvenile idiopathic arthritis(2 cases),and vitiligo(2 cases).No significant differences in concurrent infections were found between SIgAD-AP group and SIgAD-nAP group.One patient of SIgAD-AP group died of lung cancer,and the rest were stable during follow-up.Conclusions AP and/or autoantibodies were not rarely found in SigAD.Clinicians should be alert of AP when assessing SIgAD.