Division of Allergy-Immunology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
Am J Rhinol Allergy. 2013 Jan;27(1):34-8. doi: 10.2500/ajra.2013.27.3831.
A subset of patients with chronic rhinosinusitis (CRS) has refractory disease. The risk factors for refractory CRS include atopy, a disrupted mucociliary transport system, medical conditions affecting the sinonasal tract mucosa, and immunodeficiency.
We review four primary immunodeficiencies reported in individuals with CRS: common variable immune deficiency (CVID), selective IgA deficiency, IgG subclass deficiency, and specific antibody deficiency. We also review treatment options for individuals with both CRS and a concomitant immune defect.
There is a high prevalence of CRS in individuals with CVID and selective IgA deficiency. While many reports describe IgG subclass deficiency in individuals with CRS, the clinical relevance of this is unclear. Specific antibody deficiency may play a more significant role in the pathogenesis of refractory CRS.
Screening for a primary immunodeficiency should be part of the diagnostic workup of refractory CRS, as its identification may allow for more effective long-term therapeutic options.
一部分慢性鼻-鼻窦炎（CRS）患者疾病顽固难愈。导致难治性 CRS 的危险因素包括特应性、黏膜纤毛传输系统障碍、影响鼻窦黏膜的医学状况以及免疫缺陷。
我们综述了 CRS 患者中报告的四种原发性免疫缺陷：普通可变免疫缺陷（CVID）、选择性 IgA 缺乏、IgG 亚类缺乏和特异性抗体缺乏。我们还综述了同时存在 CRS 和免疫缺陷的患者的治疗选择。
在 CVID 和选择性 IgA 缺乏患者中，CRS 的患病率很高。虽然许多报告描述了 CRS 患者 IgG 亚类缺乏，但这一情况的临床相关性尚不清楚。特异性抗体缺乏可能在难治性 CRS 的发病机制中发挥更重要的作用。
对于难治性 CRS，应将原发性免疫缺陷筛查作为诊断评估的一部分，因为其明确可能有助于更有效的长期治疗选择。
