Español T, Catala M, Hernandez M, Caragol I, Bertran J M
Immunology Unit, C.S. Valle Hebrón, Barcelona, Spain.
Clin Immunol Immunopathol. 1996 Sep;80(3 Pt 1):333-5. doi: 10.1006/clin.1996.0132.
IgA deficiency (IgA-D) and common variable immunodeficiency (CVID) are two primary immunodeficiencies that share clinical features. Occasionally, both diseases have been diagnosed in the same family, which suggests the existence of some common pathogenic mechanism, but progression from IgA-D to CVID has rarely been documented. We report three cases of CVID diagnosed 1 to 12 years after IgA-D was detected. Two of these patients presented autoimmune diseases followed by a progressive decline in IgG levels. They are currently on intravenous immunoglobulin therapy with complete remission of their autoimmune and infectious symptoms.
IgA缺乏症（IgA-D）和常见变异型免疫缺陷病（CVID）是两种具有共同临床特征的原发性免疫缺陷病。偶尔，这两种疾病会在同一家族中被诊断出来，这表明存在一些共同的致病机制，但从IgA-D进展为CVID的情况鲜有记录。我们报告了3例在检测到IgA-D后1至12年被诊断为CVID的病例。其中2例患者出现自身免疫性疾病，随后IgG水平逐渐下降。他们目前正在接受静脉注射免疫球蛋白治疗，自身免疫和感染症状完全缓解。
