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与常见可变免疫缺陷(CVID)和慢性肉芽肿病(CGD)相关的胃肠道疾病

Gastrointestinal Disorders Associated with Common Variable Immune Deficiency (CVID) and Chronic Granulomatous Disease (CGD).

作者信息

Uzzan Mathieu, Ko Huaibin M, Mehandru Saurabh, Cunningham-Rundles Charlotte

机构信息

Division of Gastroenterology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.

The Immunology Institute, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.

出版信息

Curr Gastroenterol Rep. 2016 Apr;18(4):17. doi: 10.1007/s11894-016-0491-3.

Abstract

Common variable immune deficiency (CVID) and chronic granulomatous disease (CGD) are two of the well-characterized primary immune deficiencies with distinct pathologic defects. While CVID is predominantly a disorder of the adaptive immune system, in CGD, innate immunity is impaired. In both syndromes, the clinical manifestations include an increased susceptibility to infections and a number of non-infectious, inflammatory conditions including systemic autoimmunity, as well as organ-specific pathology. Among the organ-associated disorders, gastrointestinal (GI) manifestations are one of the most intractable. As such, non-infectious inflammatory disorders of the GI tract are clinically challenging as they have protean manifestations, often resembling inflammatory bowel disease (IBD) or celiac disease, are notoriously difficult to treat, and hence are associated with significant morbidity and mortality. Therefore, assessing the pathogenesis and defining appropriate therapeutic approaches for GI disease in patients with CVID and CGD is imperative.

摘要

普通可变免疫缺陷(CVID)和慢性肉芽肿病(CGD)是两种特征明确的原发性免疫缺陷,具有不同的病理缺陷。虽然CVID主要是适应性免疫系统的疾病,但在CGD中,先天免疫受损。在这两种综合征中,临床表现都包括对感染的易感性增加以及一些非感染性炎症性疾病,包括全身性自身免疫以及器官特异性病变。在与器官相关的疾病中,胃肠道(GI)表现是最难处理的问题之一。因此,胃肠道的非感染性炎症性疾病在临床上具有挑战性,因为它们具有多样的表现,常常类似于炎症性肠病(IBD)或乳糜泻, notoriously difficult to treat,因此与显著的发病率和死亡率相关。因此,评估CVID和CGD患者胃肠道疾病的发病机制并确定适当的治疗方法势在必行。

原文中“notoriously difficult to treat”直接保留英文未翻译完整,可能是原文有误,若按正确英文翻译后完整译文为:普通可变免疫缺陷(CVID)和慢性肉芽肿病(CGD)是两种特征明确的原发性免疫缺陷,具有不同的病理缺陷。虽然CVID主要是适应性免疫系统的疾病,但在CGD中,先天免疫受损。在这两种综合征中,临床表现都包括对感染的易感性增加以及一些非感染性炎症性疾病,包括全身性自身免疫以及器官特异性病变。在与器官相关的疾病中,胃肠道(GI)表现是最难处理的问题之一。因此,胃肠道的非感染性炎症性疾病在临床上具有挑战性,因为它们具有多样的表现,常常类似于炎症性肠病(IBD)或乳糜泻,众所周知难以治疗,因此与显著的发病率和死亡率相关。因此,评估CVID和CGD患者胃肠道疾病的发病机制并确定适当的治疗方法势在必行。

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