Selective IgA deficiency in children and adults with systemic lupus erythematosus
Selective IgA deficiency in children and adults with systemic lupus erythematosus
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DOI：
10.1177/0961203307077543
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发表时间：
2007-01-01
期刊：
影响因子：
2.6
通讯作者：
Selby, C. L.
中科院分区：
文献类型：
--
作者：
Cassidy, J. T.;Kitson, R. K.;Selby, C. L.
本研究的目的是确定患有系统性红斑狼疮 (SLE) 的儿童和成人选择性 IgA 缺乏症 (SIgAD) 的频率和临床特征，并评估 SLE 的表现和病程的潜在差异。 IgA 缺乏症定义为血清 I-A 浓度 = 20 年，对一年内接受调查的 152 名成年人进行了血清 IgA 水平测定。比较了 I-A 缺陷患者和 I-A 正常患者的疾病特征。确定了 12 名 SIgAD 患者： 1) Juveilile(J)-SLE：四名青少年发病的儿童（！l 8 岁），其他四名是成年时发病； 2) 成人(A)-SLE：四名成人发病的患者。除了复发性感染可能增加以及观察到只有两名非裔美国人之外，在临床表现或病程上没有发现显着差异。 5 名患者接受了输血，但未出现任何反应：其中 3 名患者血清中存在抗 IgA 抗体。一名儿科患者 IgA 水平较低（
The objective of this study was to determine the frequency and clinical characteristics of selective IgA deficiency (SIgAD) in children and adults with systemic lupus erythematosus (SLE), and evaluate potential differences in presentation and course of the SLE. IgA deficiency was defined as a serum I-A concentration = 20 years and 152 adults surveyed during a one-year period were assayed for serum IgA levels. Disease characteristics were compared among the deficient patients and the I-A-normal patients. Twelve patients with SIgAD were identified: 1) Juveilile(J)-SLE: four children with juvenile onset (! l 8 years) and four others encountered as adults; and 2) Adult(A)-SLE: four patients with adult onset. No significant differences were found in clinical presentation or course except for a possible increase in recurrent infections and the observation that there were only two African-Americans. Five patients had received blood transfusions with no reactions: three of these patients had serum anti-IgA antibodies. One pediatric patient developed low levels of IgA (