成人选择性免疫球蛋白A缺乏症的临床及实验室指标分析

Analysis of clinical and laboratory characteristics of adult patients with selective immunoglobulin A deficiency

目的探讨成人选择性免疫球蛋白A缺乏(selective immunoglobulin A deficiency,SIgAD)患者临床表现及相关实验室指标的特点,以增进对中国西部地区SIgAD的认识。方法选取2015年1月至2019年12月于空军军医大学第一附属医院确诊的SIgAD成人患者45例。回顾性分析其临床表现、自身抗体、免疫球蛋白(immunoglobulin,Ig)G与IgM、补体(complement,C)3与C4、白细胞(white blood cell,WBC)绝对计数、淋巴细胞亚群绝对计数等指标的变化情况。结果在45例SIgAD患者中,20例(20/45,44%)出现了反复感染,其中13例(13/45,29%)为呼吸道感染。34例(34/45,76%)SIgAD患者合并自身免疫性疾病(autoimmune disease,AID),其中20例(20/45,44%)为系统性红斑狼疮(systemic lupus erythematosus,SLE)。34例(34/45,76%)患者IgG水平升高,34例(34/45,76%)患者IgM水平正常,22例(22/45,49%)患者补体C3降低、24例(24/45,53%)患者补体C4降低。合并AID与非合并AID的SIgAD患者比较,IgG、IgM、C3、C4在差异均无统计学意义[(20.94±7.70)g/L比(19.84±7.63)g/L,(1.25±0.97)g/L比(1.15±0.77)g/L,(0.77±0.31)g/L比(0.84±0.18)g/L,(0.15±0.08)比(0.19±0.08)g/L,t/z值分别为-0.449、-0.295、0.670、1.155,P值均>0.05]。多数患者[77%(34/44)]WBC正常,15例(15/44,34%)患者淋巴细胞计数降低。合并AID的SIgAD患者(26/31,84%)出现抗核抗体阳性。在45例SIgAD患者中男性12例,女性33例,男女比为1∶2.75,男女患者IgG、IgM,补体C3、C4、ANA之间差异均无统计学意义(t/χ^(2)值分别为-1.102、-1.240、-1.023、0.991、1.822,P值均>0.05)。结论成人SIgAD患者常伴有AID,以SLE最为多见,患者常出现反复的感染。合并AID的SIgAD患者多数患者出现ANA阳性,免疫球蛋白IgG的升高,补体C3、C4降低。

Objective To study the clinical manifestations and laboratory test results of adult patients with selective immunoglobulin A deficiency(SIgAD),and enhance the understanding of SIgAD in western China.Methods A total of 45 confirmed adult SIgAD patients with immunoglobulin detection in the First affiliated Hospital of Air Force Military Medical University from January 2015 to December 2019 were selected.The clinical manifestations of patients and related laboratory test results of autoantibodies,immunoglobulin G(IgG)and immunoglobulin M(IgM),complement(c)3 and C4,white blood cell count(WBC)and lymphocyte subsets count were retrospectively analyzed.Results In the 45 SIgAD patients,20 patients(20/45,44%)were found repeated infections,and 13(13/45,29%)of which patients had respiratory infections.Total of 34 patients(34/45,76%)with SIgAD had autoimmune disease(AID),20(20/45,44%)of which were systemic lupus erythematosus(SLE).There were increased IgG(76%,34/45)and normal IgM(76%,34/45)in most of the patient,and nearly half of them with decreased C3(49%,22/45)and C4(53%,24/45).The levels of IgG,IgM,C3 and C4 had no significant differences between patients with or without AID[(20.94±7.70)g/L vs(19.84±7.63)g/L,(1.25±0.97)g/L vs(1.15±0.77)g/L,(0.77±0.31)g/L vs(0.84±0.18)g/L,(0.15±0.08)g/L vs(0.19±0.08)g/L;t/z values were-0.449,-0.295,0.670,1.155 respectively,all P values>0.05].As for WBC results,it showed that 34 patients(34/44,77%)had normal WBC,while 15 patients(15/44,34%)had a reduced lymphocyte count.ANA in 26 patients SIgAD with(26/31,84%)combined with AID showed positive results.There were 12 males and 33 females in 45 patients with sIgAD.The ratio of male to female was 1∶2.75.The levels of IgG,IgM,C3,C4 and ANA had no significant differences between males and females(t/χ^(2) values were-1.102,-1.240-1.023,0.991,1.822 respectively,all P values>0.05).Conclusion The main clinical complications of SIgAD are recurrent infections and AID,with SLE being the most common.ANA positivity,increased IgG and decreased complement were found in most SIgAD patients with AID.