Division of Allergy and Immunology, Department of Pediatrics, Children's Hospital of Philadelphia, Pennsylvania.
Institute for Immunology, University of Pennsylvania, Philadelphia.
J Pediatric Infect Dis Soc. 2018 May 9;7(suppl_1):S18-S24. doi: 10.1093/jpids/piy014.
Chronic granulomatous disease (CGD), a primary immunodeficiency characterized by a deficient neutrophil oxidative burst and the inadequate killing of microbes, is well known to cause a significantly increased risk of invasive infection. However, infectious complications are not the sole manifestations of CGD; substantial additional morbidity is driven by noninfectious complications also. These complications can include, for example, a wide range of inflammatory diseases that affect the gastrointestinal tract, lung, skin, and genitourinary tract and overt autoimmune disease. These diseases can occur at any age and are especially problematic in adolescents and adults with CGD. Many of these noninfectious complications present a highly challenging therapeutic conundrum, wherein immunosuppression must be balanced against an already markedly increased risk of invasive fungal and bacterial infections. In this review, the myriad noninfectious complications of CGD are discussed, as are important gaps in our understanding of these processes, which warrant further investigation.
慢性肉芽肿病（CGD）是一种原发性免疫缺陷病，其特征为中性粒细胞氧化爆发缺陷和微生物杀伤不足，众所周知，它会显著增加侵袭性感染的风险。然而，感染并发症并不是 CGD 的唯一表现；非感染性并发症也会导致大量的发病率增加。这些并发症包括，例如，影响胃肠道、肺、皮肤和泌尿生殖道的广泛炎症性疾病，以及明显的自身免疫性疾病。这些疾病可以发生在任何年龄，在 CGD 的青少年和成年人中尤其成问题。许多这些非感染性并发症提出了一个极具挑战性的治疗难题，其中必须平衡免疫抑制与已经显著增加的侵袭性真菌感染和细菌感染的风险。在这篇综述中，讨论了 CGD 的多种非感染性并发症，以及我们对这些过程的理解存在的重要差距，这需要进一步研究。
