Díez Rubén, García M Jesús, Vivas Santiago, Arias Laura, Rascarachi Gabriela, Pozo Elvira del, Vaquero Luis M, Miguel Aleida, Sierra Mónica, Calleja Sara, Ruiz De Morales José M
Sección de Aparato Digestivo, Hospital Universitario de León, León, España.
Gastroenterol Hepatol. 2010 May;33(5):347-51. doi: 10.1016/j.gastrohep.2009.12.012. Epub 2010 Mar 1.
Primary immunodeficiencies can lead to gastrointestinal manifestations that are still not well defined.
To analyze gastrointestinal manifestations associated with primary immunodeficiencies.
We performed a retrospective study that included patients diagnosed with primary antibody deficiencies in a third-level hospital. The patients were divided into two groups: isolated IgA deficiency and common variable immunodeficiency syndrome (CVIS). The timing of presentation and type of gastrointestinal symptoms were analyzed.
There were 57 patients: 20 with CVIS (35%) and 37 with isolated IgA deficiency (65%). Diagnosis was made in the pediatric age in 17 patients, of whom 13 had isolated IgA deficiency. In 84% of the patients, diagnosis of immunodeficiency was made before the development of gastrointestinal manifestations. Digestive symptoms were found in 74% of the patients, the most frequent being diarrhea. In 46% of the patients, digestive disease was confirmed, mainly through endoscopy. Celiac-like lesions, chronic atrophic gastritis, ulcerative colitis-like disease and Crohn's disease were more common in CVIS. In isolated IgA deficiency, Helicobacter pylori-positive chronic gastritis predominated. Mean age was significantly higher (36 vs. 24 years, p=0.02) and IgA titer significantly lower (17 vs. 34UI/ml; p=0.008) in patients with associated gastrointestinal disease.
Gastrointestinal symptoms are frequent and lead to endoscopic diagnosis in half of patients with primary immunodeficiencies. Ulcerative colitis, and celiac- and Crohn's-like disease are atypical entities that occur in CVIS.
原发性免疫缺陷可导致胃肠道表现，但目前仍未完全明确。
分析与原发性免疫缺陷相关的胃肠道表现。
我们进行了一项回顾性研究，纳入了一家三级医院中被诊断为原发性抗体缺陷的患者。患者分为两组：孤立性IgA缺乏症和常见变异型免疫缺陷综合征（CVIS）。分析了症状出现的时间和胃肠道症状的类型。
共有57例患者，其中20例患有CVIS（35%），37例患有孤立性IgA缺乏症（65%）。17例患者在儿童期确诊，其中13例患有孤立性IgA缺乏症。84%的患者在出现胃肠道表现之前就已确诊免疫缺陷。74%的患者出现消化系统症状，最常见的是腹泻。46%的患者经内镜检查确诊为消化系统疾病。乳糜泻样病变、慢性萎缩性胃炎、溃疡性结肠炎样疾病和克罗恩病在CVIS中更为常见。在孤立性IgA缺乏症中，幽门螺杆菌阳性的慢性胃炎最为常见。伴有胃肠道疾病的患者平均年龄显著更高（36岁对24岁，p=0.02），IgA滴度显著更低（17对34UI/ml；p=0.008）。
胃肠道症状在原发性免疫缺陷患者中很常见，半数患者经内镜检查确诊。溃疡性结肠炎以及乳糜泻样和克罗恩病样疾病是CVIS中出现的非典型病症。
