系统性红斑狼疮合并选择性 IgA 缺乏症4例并文献复习

摘要

目的：探讨系统性红斑狼疮（systemic lupus erythematosus，SLE）合并选择性 IgA 缺乏症（selective immunoglobulin A deficiency，SIgAD）的临床特点。方法报告广州医科大学附属第二医院2006年1月至2013年3月4例 SLE 合并 SIgAD 病例资料。结果4例患者均为女性，年龄20～48岁，3例有反复上呼吸道感染史。4例均有关节炎、肾炎，3例有颧部红斑，脑梗塞、光过敏、胸腔积液、白细胞计数减少各1例。抗核抗体和抗双链 DNA 抗体阳性各4例，抗 Sm 抗体和抗心磷脂抗体阳性各2例。血清 IgA 均＜0．07 g/L。4例患者经治疗后3例好转，1例死于感染性休克，其中1例 IgA 水平升至0．07～0．18 g/L。结论 SLE 可能合并 SIgAD，患者经治疗病情缓解后其 IgA 水平可能变化。%Objective To study the clinical features of patients with systemic lupus erythematosus complicating with selective immunoglobulin A deficiency (SIgAD).Methods We described four patients with SLE complicating with SIgAD treated in the Second Affiliated Hospital of Guangzhou Medical University from Jan 2006 to Mar 2013 and review the literature.Results Four patients were female,with the age ranged from 20 to 48 years old.Three patients suffered from recurrent upper respiratory tract infection.Arthritis and lupus nephritis existed in 4 patients,malar rash in 3 patients,cerebral infarction,photosensivity,pleuritis and leukopenia in 1 patient respectively.Positive ANA and anti-dsDNA antibody were found in 4 patients, positive anti-Sm antibody and anti-cardiolipin antibodies in 2 patients.Serological immunoglobulins (IgG, lgM,IgA)were measured and IgA was lower than 0.07 g/L in 4 patients.After therapy,three patients improved and one patient died of septic shock,IgA increased in one patient (0.07-0.18 g/L).Conclusion Patients with SLE may complicate with SIgAD.The level of IgA may change after their disease get remission.