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Otolaryngology (ENT)94 papers

Allergic fungal sinusitis

Last edited: 2 h ago

Overview

Allergic fungal sinusitis (AFS) is a chronic, noninvasive form of fungal sinusitis characterized by an IgE-mediated hypersensitivity reaction to environmental fungi. It predominantly affects immunocompetent individuals, often presenting with chronic rhinosinusitis, nasal polyps, and elevated total serum IgE levels. AFS accounts for 5 to 10% of chronic rhinosinusitis cases and can lead to significant morbidity if not accurately diagnosed and treated. Accurate diagnosis is crucial for effective management, as inappropriate treatment can result in persistent symptoms and complications. Understanding AFS is essential for clinicians to differentiate it from other forms of sinusitis and to tailor appropriate therapeutic interventions 1 4 33 50.

Pathophysiology

AFS arises from a complex interplay between host immune response and fungal antigens. The sinus mucosa, sensitized by environmental fungi, mounts a robust Th2-type immune response characterized by elevated IgE production and eosinophilic inflammation. Eosinophils, laden with granule proteins, release mediators that contribute to mucosal edema, mucus overproduction, and tissue remodeling, leading to sinus obstruction and mucocele formation. Intact and degenerating eosinophils, along with Charcot-Leyden crystals and scant fungal hyphae, are hallmarks found in the characteristic "peanut butter" allergic mucin. Over time, this chronic inflammation can result in bony erosion and, in rare cases, intracranial extension, highlighting the potential severity of the condition 1 4 12 33 50.

Epidemiology

The incidence of AFS varies but is estimated to affect 5-10% of patients with chronic rhinosinusitis. It predominantly impacts young to middle-aged adults, with a slight male predominance observed in some studies. Geographic and environmental factors play a significant role, with higher prevalence noted in certain regions due to fungal exposure patterns. For instance, studies from the southern United States have reported higher incidences compared to northern regions. Additionally, atopy and a history of asthma are recognized risk factors, with asthma prevalence ranging from 20% to 40% in AFS patients. Trends suggest an increasing recognition of AFS, possibly due to improved diagnostic techniques and heightened awareness among clinicians 3 33 50 67.

Clinical Presentation

Patients with AFS typically present with chronic nasal congestion, facial pain or pressure, and nasal polyposis. Common symptoms include unilateral or bilateral nasal obstruction, purulent nasal discharge, and reduced sense of smell. Additional features may include headache, fever (less common), and, in severe cases, orbital or intracranial complications such as visual disturbances or neurological deficits. Red-flag symptoms like sudden visual loss or neurological changes warrant urgent evaluation for potential complications like mucoceles or intracranial extension 1 3 9 27 33.

Diagnosis

The diagnosis of AFS involves a combination of clinical evaluation, imaging, and histopathological examination. Key diagnostic criteria include:

Clinical Features: Chronic rhinosinusitis, nasal polyps, and elevated total serum IgE levels.

Imaging:

- CT Scan: Sinus opacification, mucocele formation, and bony erosion patterns. - MRI: Characteristic signal intensity changes in sinus mucosa, distinguishing from other forms of sinusitis.

Histopathology: Presence of eosinophilic mucin with intact and degenerating eosinophils, Charcot-Leyden crystals, and minimal fungal hyphae.

Immunological Tests: Elevated total IgE, specific IgE to fungal antigens (though not always definitive).

Culture and Molecular Identification: Identification of fungal species, such as Schizophyllum commune, Trichoderma longibrachiatum, and others, can support the diagnosis 1 3 12 33 44 50.

Differential Diagnosis:

Chronic Rhinosinusitis without Nasal Polyps: Absence of polyps and less prominent eosinophilic inflammation.

Invasive Fungal Sinusitis: Presence of tissue invasion, more severe clinical presentation, and often immunocompromised status.

Allergic Bronchopulmonary Aspergillosis: Pulmonary involvement and more pronounced systemic symptoms 1 3 12 33 44 50.

Management

Initial Treatment

Surgical Intervention: Functional endoscopic sinus surgery (FESS) to remove allergic mucin and polyps.

- Specifics: Removal of obstructive polyps and mucin, ensuring thorough debridement of affected sinuses.

Postoperative Care:

- Nasal Steroids: Intranasal corticosteroids to reduce inflammation (e.g., fluticasone, mometasone). - Dose: Typically 50-100 mcg twice daily. - Duration: Several months post-surgery to maintain control. - Oral Corticosteroids: Short-term use to manage acute exacerbations. - Dose: Prednisone 40-60 mg daily for 5-7 days. - Monitoring: Regular follow-up for side effects like hyperglycemia, hypertension.

Second-Line Treatment

Antifungal Therapy: Postoperative adjunctive therapy to prevent recurrence.

- Itraconazole: Oral antifungal prophylaxis. - Dose: 200 mg daily for 3-6 months post-surgery. - Monitoring: Liver function tests, complete blood count. - Fluconazole: Alternative option for patients intolerant to itraconazole. - Dose: 400 mg daily. - Duration: Similar to itraconazole.

Refractory Cases

Immunotherapy: Consideration in cases with persistent symptoms despite conventional therapy.

- Specifics: Subcutaneous or sublingual immunotherapy targeting specific fungal allergens. - Monitoring: Regular assessment of symptom control and adverse reactions.

Specialist Referral: For complex cases involving intracranial complications or refractory disease.

- Consultations: Neurosurgery, infectious disease specialist, or immunologist.

Contraindications:

Severe immunosuppression.

Known severe hypersensitivity reactions to antifungal agents.

Complications

Bony Erosion: Progressive erosion of sinus walls, potentially leading to orbital or intracranial complications.

Orbital Involvement: Proptosis, diplopia, and vision loss due to orbital inflammation or mucocele expansion.

Intracranial Extension: Rare but serious complications including brain abscesses, meningitis, or cranial nerve palsies.

Recurrent Disease: Persistent symptoms despite treatment, often necessitating repeated surgical interventions.

- Management Triggers: Inadequate surgical clearance, non-compliance with postoperative care, or underlying immune dysregulation 1 27 33 50 64.

Prognosis & Follow-up

The prognosis for AFS is generally good with appropriate management, but recurrence rates can be high, ranging from 20% to 40%. Prognostic indicators include the extent of bony erosion, presence of intracranial complications, and patient compliance with postoperative care. Recommended follow-up intervals typically include:

Initial Follow-up: 1-2 weeks post-surgery to assess healing and control symptoms.

Subsequent Follow-ups: Every 3-6 months for the first year, then annually to monitor for recurrence and manage chronic symptoms.

Monitoring: Regular imaging (CT/MRI), nasal endoscopy, and serological markers (IgE levels) to guide treatment adjustments 1 33 50.

Special Populations

Pediatric Patients: AFS can occur in children, often presenting with similar symptoms but may also include developmental delays if complications arise. Early diagnosis and intervention are crucial.

- Management: Conservative surgical approaches initially, with close monitoring for complications.

Immunocompromised Individuals: Although rare, these patients may present with atypical features and require heightened vigilance for invasive fungal elements.

- Management: Collaboration with infectious disease specialists for tailored antifungal therapy.

Ethnic and Geographic Variations: Certain ethnic groups and geographic regions may have higher exposure to specific fungal species, influencing prevalence and clinical presentation. Tailored environmental and immunological assessments may be necessary 9 33 50 67.

Key Recommendations

Surgical Intervention: Perform FESS for definitive removal of allergic mucin and polyps in diagnosed cases [Evidence: Strong] 1 33.

Postoperative Nasal Steroids: Use intranasal corticosteroids for at least 3-6 months post-surgery to control inflammation [Evidence: Strong] 1 33.

Short-Term Oral Corticosteroids: Consider short-term oral corticosteroids for acute exacerbations to manage inflammation [Evidence: Moderate] 1 33.

Postoperative Antifungal Prophylaxis: Administer itraconazole 200 mg daily for 3-6 months post-surgery to prevent recurrence [Evidence: Moderate] 16 50.

Regular Follow-Up: Schedule follow-up visits every 3-6 months for the first year, then annually, including imaging and serological markers [Evidence: Moderate] 1 33.

Consider Immunotherapy: Evaluate immunotherapy for patients with persistent symptoms despite conventional treatment [Evidence: Weak] 1 33.

Monitor for Complications: Closely monitor for signs of bony erosion, orbital involvement, and intracranial extension, especially in complex cases [Evidence: Expert opinion] 27 64.

Specialist Referral: Refer refractory or complicated cases to specialists such as neurosurgeons or immunologists [Evidence: Expert opinion] 1 33.

Evaluate Total IgE Levels: Include total IgE levels in diagnostic workup to support the diagnosis of AFS [Evidence: Moderate] 1 33.

Histopathological Confirmation: Ensure histopathological examination confirms eosinophilic mucin and minimal fungal hyphae for definitive diagnosis [Evidence: Strong] 1 33 44.

References

1 Alsalem S, Almontashri A, Alsalem M, Altamimi F, Alyami N, Hajjaf S et al.. Value of MRI signal intensity in evaluation of allergic fungal rhinosinusitis compared with CT Hounsfield units: Retrospective study. Medicine 2024. link 2 Won EJ, Shin JH, Lim SC, Shin MG, Suh SP, Ryang DW. Molecular identification of Schizophyllum commune as a cause of allergic fungal sinusitis. Annals of laboratory medicine 2012. link 3 Telmesani LM. Prevalence of allergic fungal sinusitis among patients with nasal polyps. Annals of Saudi medicine 2009. link 4 Tang P, Mohan S, Sigler L, Witterick I, Summerbell R, Campbell I et al.. Allergic fungal sinusitis associated with Trichoderma longibrachiatum. Journal of clinical microbiology 2003. link 5 Ghorbel M, Kolsi F, Makni S, Cherif I, Hammemi B, Boudawara MZ. Exploring complexity: A compelling case of chronic allergic fungal sinusitis caused by Alterna Alternata with intracranial expansion and orbital involvement in an immunocompetent male - An illuminating case study and in-depth literature review. Diagnostic microbiology and infectious disease 2026. link 6 Xu T, Guo XT, Zhou YC, Zhou Q, Wang YF. Consideration of the Clinical Diagnosis of Allergic Fungal Sinusitis: A Single-Center Retrospective Study. Ear, nose, & throat journal 2025. link 7 Shrivastava J, Shah K, Shah N. Chrysosporium: A rare cause of allergic fungal rhinosinusitis. Indian journal of pathology & microbiology 2023. link 8 O'Donnell EP, Kane AE. Permanent Unilateral Vision Loss From Allergic Fungal Sinusitis. Pediatric emergency care 2022. link 9 Mancuso RF. Pediatric Allergic Fungal Sinusitis: A Clinical Review. Pediatric annals 2021. link 10 Donner K, Kou YF, Tomkies A, Rakheja D, Mitchell RB, Shah G. Gonorrhea-positive sinus cultures in a 15-year-old with allergic fungal sinusitis. JAAPA : official journal of the American Academy of Physician Assistants 2020. link 11 Alajmi S, Koratum RM, Khan Z, Ahmad S, Jeragh A, Ibrahim H et al.. Allergic Fungal Sinusitis Caused by Exserohilum rostratum and Literature Review. Mycopathologia 2019. link 12 Rai G, Das S, Ansari MA, Singh PK, Gupta N, Sharma S et al.. Phenotypic and functional profile of Th17 and Treg cells in allergic fungal sinusitis. International immunopharmacology 2018. link 13 Loftus PA, Wise SK. Allergic Fungal Rhinosinusitis: The Latest in Diagnosis and Management. Advances in oto-rhino-laryngology 2016. link 14 Menendez JY, Woodworth BA, Johnston JM. Gynecomastia and Hyperprolactinemia Secondary to Advanced Allergic Fungal Rhinosinusitis in a Pediatric Patient. Turkish neurosurgery 2016. link 15 Mostafa Bel-D, Taha MS, Abdel Hamid T, Omran A, Lotfi N. Evaluation of vitamin D levels in allergic fungal sinusitis, chronic rhinosinusitis, and chronic rhinosinusitis with polyposis. International forum of allergy & rhinology 2016. link 16 Patro SK, Verma RK, Panda NK, Chakrabarti A, Singh P. Efficacy of preoperative itraconazole in allergic fungal rhinosinusitis. American journal of rhinology & allergy 2015. link 17 White LC, Jang DW, Yelvertan JC, Kountakis SE. Bony erosion patterns in patients with allergic fungal sinusitis. American journal of rhinology & allergy 2015. link 18 Devars du Mayne M, Gratacap M, Malinvaud D, Grenouillet F, Bonfils P. An uncommon cause of allergic fungal sinusitis: Rhizopus oryzae. Ear, nose, & throat journal 2015. link 19 Marglani O. Update in the management of allergic fungal sinusitis. Saudi medical journal 2014. link 20 Cavanna C, Seminari E, Pusateri A, Mangione F, Lallitto F, Esposto MC et al.. Allergic fungal rhinosinusitis due to Curvularia lunata. The new microbiologica 2014. link 21 Braun JJ, Dupret A, Veillon F, Riehm S. Pseudotumoral allergic fungal sinusitis with skull base involvement. B-ENT 2014. link 22 Agarwal K, Kathuria S, Sundar G, Singh P, Khanna G, Chowdhary A. A case of allergic fungal rhinosinusitis due to Ceratocystis adiposa. Diagnostic microbiology and infectious disease 2014. link 23 Kim C, Kacker A, Chee RI, Lelli GJ. Allergic fungal sinusitis causing nasolacrimal duct obstruction. Orbit (Amsterdam, Netherlands) 2013. link 24 Bozeman S, deShazo R, Stringer S, Wright L. Complications of allergic fungal sinusitis. The American journal of medicine 2011. link 25 Ogawa H, Fujimura M, Takeuchi Y, Makimura K. A case of sinobronchial allergic mycosis; possibility of basidiomycetous fungi as a causative antigen. Internal medicine (Tokyo, Japan) 2011. link 26 Zakirullah, Nawaz G, Sattar SF. Presentation and diagnosis of allergic fungal sinusitis. 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Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2006. link61237-9) 41 Jen A, Kacker A, Huang C, Anand V. Fluconazole nasal spray in the treatment of allergic fungal sinusitis: a pilot study. Ear, nose, & throat journal 2004. link 42 Heffner DK. Allergic fungal sinusitis is a histopathologic diagnosis; paranasal mucocele is not. Annals of diagnostic pathology 2004. link 43 Luong A, Marple BF. Allergic fungal rhinosinusitis. Current allergy and asthma reports 2004. link 44 Taj-Aldeen SJ, Hilal AA, Schell WA. Allergic fungal rhinosinusitis: a report of 8 cases. American journal of otolaryngology 2004. link 45 Watanabe K, Misu T, Ohde S, Edamatsu H. Characteristics of eosinophils migrating around fungal hyphae in nasal discharge. The Annals of otology, rhinology, and laryngology 2004. link 46 Thakar A, Sarkar C, Dhiwakar M, Bahadur S, Dahiya S. Allergic fungal sinusitis: expanding the clinicopathologic spectrum. 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The Journal of laryngology and otology 2001. link 57 Serrano E, Percodani J, Uro-Coste E, Yardeni E, Abbal M, Linas MD et al.. Value of investigation in the diagnosis of allergic fungal rhinosinusitis: results of a prospective study. The Journal of laryngology and otology 2001. link 58 Andes D, Proctor R, Bush RK, Pasic TR. Report of successful prolonged antifungal therapy for refractory allergic fungal sinusitis. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America 2000. link 59 Marple BF. Allergic fungal rhinosinusitis: surgical management. Otolaryngologic clinics of North America 2000. link80015-8) 60 Mabry RL, Marple BF, Mabry CS. Mold testing by RAST and skin test methods in patients with allergic fungal sinusitis. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery 1999. link70180-4) 61 Schnadig VJ, Rassekh CH, Gourley WK. Allergic fungal sinusitis. 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Original source

[1]
Value of MRI signal intensity in evaluation of allergic fungal rhinosinusitis compared with CT Hounsfield units: Retrospective study. — Alsalem S, Almontashri A, Alsalem M, Altamimi F, Alyami N, Hajjaf S et al. Medicine (2024)
[2]
Molecular identification of Schizophyllum commune as a cause of allergic fungal sinusitis. — Won EJ, Shin JH, Lim SC, Shin MG, Suh SP, Ryang DW Annals of laboratory medicine (2012)
[3]
Prevalence of allergic fungal sinusitis among patients with nasal polyps. — Telmesani LM Annals of Saudi medicine (2009)
[4]
Allergic fungal sinusitis associated with Trichoderma longibrachiatum. — Tang P, Mohan S, Sigler L, Witterick I, Summerbell R, Campbell I et al. Journal of clinical microbiology (2003)
[5]
Exploring complexity: A compelling case of chronic allergic fungal sinusitis caused by Alterna Alternata with intracranial expansion and orbital involvement in an immunocompetent male - An illuminating case study and in-depth literature review. — Ghorbel M, Kolsi F, Makni S, Cherif I, Hammemi B, Boudawara MZ Diagnostic microbiology and infectious disease (2026)
[6]
Consideration of the Clinical Diagnosis of Allergic Fungal Sinusitis: A Single-Center Retrospective Study. — Xu T, Guo XT, Zhou YC, Zhou Q, Wang YF Ear, nose, & throat journal (2025)
[7]
Chrysosporium: A rare cause of allergic fungal rhinosinusitis. — Shrivastava J, Shah K, Shah N Indian journal of pathology & microbiology (2023)
[8]
Permanent Unilateral Vision Loss From Allergic Fungal Sinusitis. — O'Donnell EP, Kane AE Pediatric emergency care (2022)
[9]
Pediatric Allergic Fungal Sinusitis: A Clinical Review. — Mancuso RF Pediatric annals (2021)
[10]
Gonorrhea-positive sinus cultures in a 15-year-old with allergic fungal sinusitis. — Donner K, Kou YF, Tomkies A, Rakheja D, Mitchell RB, Shah G JAAPA : official journal of the American Academy of Physician Assistants (2020)
[11]
Allergic Fungal Sinusitis Caused by Exserohilum rostratum and Literature Review. — Alajmi S, Koratum RM, Khan Z, Ahmad S, Jeragh A, Ibrahim H et al. Mycopathologia (2019)
[12]
Phenotypic and functional profile of Th17 and Treg cells in allergic fungal sinusitis. — Rai G, Das S, Ansari MA, Singh PK, Gupta N, Sharma S et al. International immunopharmacology (2018)
[13]
Allergic Fungal Rhinosinusitis: The Latest in Diagnosis and Management. — Loftus PA, Wise SK Advances in oto-rhino-laryngology (2016)
[14]
Gynecomastia and Hyperprolactinemia Secondary to Advanced Allergic Fungal Rhinosinusitis in a Pediatric Patient. — Menendez JY, Woodworth BA, Johnston JM Turkish neurosurgery (2016)
[15]
Evaluation of vitamin D levels in allergic fungal sinusitis, chronic rhinosinusitis, and chronic rhinosinusitis with polyposis. — Mostafa Bel-D, Taha MS, Abdel Hamid T, Omran A, Lotfi N International forum of allergy & rhinology (2016)
[16]
Efficacy of preoperative itraconazole in allergic fungal rhinosinusitis. — Patro SK, Verma RK, Panda NK, Chakrabarti A, Singh P American journal of rhinology & allergy (2015)
[17]
Bony erosion patterns in patients with allergic fungal sinusitis. — White LC, Jang DW, Yelvertan JC, Kountakis SE American journal of rhinology & allergy (2015)
[18]
An uncommon cause of allergic fungal sinusitis: Rhizopus oryzae. — Devars du Mayne M, Gratacap M, Malinvaud D, Grenouillet F, Bonfils P Ear, nose, & throat journal (2015)
[19]
Update in the management of allergic fungal sinusitis. — Marglani O Saudi medical journal (2014)
[20]
Allergic fungal rhinosinusitis due to Curvularia lunata. — Cavanna C, Seminari E, Pusateri A, Mangione F, Lallitto F, Esposto MC et al. The new microbiologica (2014)
[21]
Pseudotumoral allergic fungal sinusitis with skull base involvement. — Braun JJ, Dupret A, Veillon F, Riehm S B-ENT (2014)
[22]
A case of allergic fungal rhinosinusitis due to Ceratocystis adiposa. — Agarwal K, Kathuria S, Sundar G, Singh P, Khanna G, Chowdhary A Diagnostic microbiology and infectious disease (2014)
[23]
Allergic fungal sinusitis causing nasolacrimal duct obstruction. — Kim C, Kacker A, Chee RI, Lelli GJ Orbit (Amsterdam, Netherlands) (2013)
[24]
Complications of allergic fungal sinusitis. — Bozeman S, deShazo R, Stringer S, Wright L The American journal of medicine (2011)
[25]
A case of sinobronchial allergic mycosis; possibility of basidiomycetous fungi as a causative antigen. — Ogawa H, Fujimura M, Takeuchi Y, Makimura K Internal medicine (Tokyo, Japan) (2011)
[26]
Presentation and diagnosis of allergic fungal sinusitis. — Zakirullah, Nawaz G, Sattar SF Journal of Ayub Medical College, Abbottabad : JAMC (2010)
[27]
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