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系统性红斑狼疮患者的免疫球蛋白缺乏症。

Immunoglobulin deficiency in patients with systemic lupus erythematosus.

作者信息

Cronin M E, Balow J E, Tsokos G C

机构信息

Arthritis and Rheumatism Branch, National Institute of Arthritis, Bethesda, Maryland 20892.

出版信息

Clin Exp Rheumatol. 1989 Jul-Aug;7(4):359-64.

PMID:2531646
Abstract

Hypergammaglobulinemia is a common laboratory finding in patients with active systemic lupus erythematosus; in contrast, immunoglobulin deficiency, except for immunoglobulin A, is unusual. We report 18 patients who developed low immunoglobulin G levels 4 months to 22 years (median = 4 years) following the diagnosis of systemic lupus erythematosus. This phenomenon was transient in 10 patients (median duration 10.5 months). Eight patients had received cytotoxic drugs prior to the development of hypogammaglobulinemia, while all had received prednisone. The nadir levels of serum IgG were 132-550 mg/dl (median = 363 mg/dl). The presence and degree of immunoglobulin G deficiency did not correlate, in general, with the type or dose of medication. None of the patients had renal failure. Only 4 patients developed recurrent infections. Urinary loss of protein was not a cause of this disorder. Study of the in vitro cellular immune responses of peripheral blood lymphocytes in 5 patients showed that excessive 'suppressor' T cell activity and decreased numbers of B cells may be responsible for the development of immunoglobulin deficiency. Serum immunoglobulin levels should not be employed as an indication of disease activity in systemic lupus erythematosus, as all 18 patients continued to have significant clinical disease. Deficiencies of immunoglobulins are often transient and may not require treatment.

摘要

高球蛋白血症是活动性系统性红斑狼疮患者常见的实验室检查结果;相比之下,除免疫球蛋白A外的免疫球蛋白缺乏并不常见。我们报告了18例系统性红斑狼疮诊断后4个月至22年(中位数 = 4年)出现低免疫球蛋白G水平的患者。这种现象在10例患者中是短暂的(中位数持续时间10.5个月)。8例患者在出现低丙种球蛋白血症之前接受过细胞毒性药物治疗,而所有患者均接受过泼尼松治疗。血清IgG的最低点水平为132 - 550mg/dl(中位数 = 363mg/dl)。一般而言,免疫球蛋白G缺乏的存在和程度与药物类型或剂量无关。所有患者均无肾衰竭。只有4例患者发生反复感染。蛋白尿并非该疾病的病因。对5例患者外周血淋巴细胞的体外细胞免疫反应研究表明,过度的“抑制性”T细胞活性和B细胞数量减少可能是免疫球蛋白缺乏发生的原因。血清免疫球蛋白水平不应作为系统性红斑狼疮疾病活动的指标,因为所有18例患者仍有明显的临床疾病。免疫球蛋白缺乏通常是短暂的,可能不需要治疗。

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