Background: 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.
Methods: 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.
Results: 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.
Conclusion: 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.