Allergic fungal sinusitis: pathophysiology, diagnosis and management

Abstract

Allergic fungal sinusitis (AFS) is a noninvasive form of fungal rhinosinusitis with an incidence of between 6 and 9% of all rhinosinusitis requiring surgery. Regional variation in incidence has been reported, with the southern and southwestern US particularly endemic. Patients with AFS commonly present with chronic rhinosinusitis with nasal polyps, inhalant atopy, elevated total serum immunoglobulin E (IgE), and sinus-obstructing inspissates of a characteristic extramucosal 'peanut buttery' visco-elastic eosinophil-rich material called 'allergic mucin' that contains sparse numbers of fungal hyphae. Sinus CT is always abnormal, showing findings of chronic rhinosinusitis that often include central areas of increased contrast ('hyperattenuation') within abnormal paranasal sinuses that represent the presence of fungal-containing allergic mucin. AFS has been found to be analogous in several ways to allergic bronchopulmonary aspergillosis (ABPA). Both are chronic inflammatory respiratory tract disorders that are driven by hypersensitivity responses to the presence of small numbers of extramucosal fungi found growing within airway-impacting allergic mucin. AFS allergic mucin typically cultures positive for either dematiaceous fungi such as Bipolaris spicifera or Curvularia lunata, or Aspergillus species such as A. fumigatus, A. flavus or A. niger. As with ABPA, patients have type I immediate hypersensitivity to the etiologic mold in AFS. Further, both AFS and ABPA have been found to have association with specific class II major histocompatibility alleles. Proper diagnosis of AFS and differentiation from the other forms of both noninvasive and invasive fungal rhinosinusitis requires strict adherence to published diagnostic criteria. Medical treatment of AFS has been modeled to an extent after treatment approaches for ABPA that includes the use of postoperative oral corticosteroids and aggressive antiallergic inflammation therapy. The use of follow-up measurements of total serum IgE during treatment of both AFS and ABPA patients can help to monitor disease activity. Future AFS research will lead to further insights into pathogenesis, improved treatments, and ultimately decreases in surgical recurrence rates for this highly recurrent hypertrophic rhinosinusitis disorder.