Mouthon L, Cohen P, Larroche C, André M H, Royer I, Casassus P, Guillevin L
Service de Médecine Interne, Hôpital Avicenne, Bobigny.
Ann Med Interne (Paris). 1999 Jun;150(4):275-82.
Common variable immunodeficiency (CVID) is a major antibody-deficiency syndrome, associated with increased risk of bacterial infection, as well as autoimmune and granulomatous disease. The clinical and immunological features are heterogeneous. This heterogeneity is expressed by the case reports of three selected patients. These observations will be discussed, with reference to a recent classification of CVID distinguishing four different clinical entities: i) CVID presenting with clinical and immunological features of X-linked agammaglobulinemia; ii) CVID presenting with clinical and immunological features of X-linked hyper-IgM syndrome; iii) CVID associated with systemic granulomatous disease; and iiii) CVID associated with autoimmune manifestations.
普通可变免疫缺陷（CVID）是一种主要的抗体缺陷综合征，与细菌感染风险增加以及自身免疫性和肉芽肿性疾病有关。其临床和免疫学特征具有异质性。这种异质性通过三名选定患者的病例报告得以体现。将结合最近对CVID的分类来讨论这些观察结果，该分类区分了四种不同的临床实体：i）具有X连锁无丙种球蛋白血症临床和免疫学特征的CVID；ii）具有X连锁高IgM综合征临床和免疫学特征的CVID；iii）与系统性肉芽肿性疾病相关的CVID；以及iiii）与自身免疫表现相关的CVID。
