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What percentage of patients with FRS does AFRS account for?
What environmental factors are suggested to influence the incidence of AFRS?
What is the reported prevalence range of FRS among patients with chronic rhinosinusitis who undergo surgery?
Which of the following theories is mentioned as failing to explain certain aspects of AFRS?
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What was the incidence of fungi found in the nasal secretions of CRS patients in the landmark series reported in India?
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What is a characteristic feature of nasal obstruction in patients with AFRS?
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Which of the following symptoms is considered a major criterion in patients with AFRS?
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What findings are typically observed during a nasoendoscopy for an AFRS patient?
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How is the immunologic diagnosis of AFRS confirmed?
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Why is total serum IgE level not useful as a screening tool for AFRS?
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What are the five major criteria utilized to diagnose patients with allergic fungal rhinosinusitis (AFRS)?
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An elevated IgE level is always present in all AFRS patients.
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What is the characteristic appearance of allergic mucin?
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What imaging is most commonly used to check for allergic fungal rhinosinusitis (AFRS)?
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Match the following criteria for AFRS diagnosis:
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The presence of fungal hyphae is always visible in allergic mucin.
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What does a positive fungal culture indicate in AFRS patients?
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The Lund-MacKay system is used for assessing the severity of ____ on CT scan.
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What are the radiologic findings in a CT scan for AFRS?
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What is the most reliable marker for the diagnosis of allergic fungal rhinosinusitis (AFRS)?
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What does functional endoscopic sinus surgery (FESS) aim to achieve for AFRS patients? (Select all that apply)
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A comprehensive postoperative medical regimen to keep the disease under control is almost always required after _____ treatment.
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Surgery is usually the first line of treatment in the management of classical chronic rhinosinusitis (CRS).
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What is a risk factor for early disease recurrence after surgery for AFRS patients?
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What medication has been shown to reduce intraoperative bleeding and size of the polyps in the perioperative period for AFRS patients?
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Which of the following is a side effect of long-term oral corticosteroid use? (Select all that apply)
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What are the two broad categories of Fungal Rhinosinusitis (FRS)?
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Which type of FRS includes allergic fungal rhinosinusitis?
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Saprophytic fungal infections are always symptomatic.
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What proposed mechanism leads to saprophytic fungal infections?
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What is the usual treatment for saprophytic fungal infections?
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What is a fungal ball?
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What is the typical organism found in a fungal ball?
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Which demographic is most commonly affected by allergic fungal rhinosinusitis (AFRS)?
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AFRS is defined as a noninvasive fungal sinusitis resulting from an allergic and immunologic response to the presence of fungal __________ in the sinuses.
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Match the following terms with their descriptions:
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Study Notes
### Epidemiology of Allergic Fungal Rhinosinusitis (AFRS)
- The prevalence of Fungal Rhinosinusitis (FRS) amongst chronic rhinosinusitis (CRS) patients undergoing surgery is between 12% and 47%
- AFRS is most common within CRS patients, accounting for 56% to 72%
- Geographic variation exists, with warmer and humid environments proving most prevalent
- Large histological series of surgical specimens in India revealed a 24% incidence of CRS in patients
- Typical AFRS patients are young, immunocompetent with a long history of symptoms
- Patients present with gradual nasal obstruction, thick/crusty discharge, and potentially anosmia, headaches, or facial distortion
Nasoendoscopic Presentation of AFRS
- Nasal polyposis is universal, often bilateral but asymmetric
- Thick yellow or brown mucus resembling peanut butter may be seen among polyps
Immunologic Testing in AFRS
- Elevated IgE levels are present, with serum IgE level useful in monitoring clinical activity during treatment
- Elevated IgE levels often precede worsening of clinical stage
- Diagnosis requires fungal-specific IgE, which can be identified through skin prick or RAST testing
Treatment of AFRS: Antifungals
- Oral antifungals may be used in recalcitrant chronic fungal sinusitis and in acute exacerbations
- Antifungals are considered as a steroid-sparing medication
- There are no randomized controlled trials on the effectiveness oral antifungals in AFRS patients
- Studies on the effectiveness of topical antifungal in AFRS patients have shown mixed results
Treatment of AFRS: Other Options
- Topical corticosteroids are standard treatment
- Topical corticosteroids are highly effective postoperatively, when the open sinuses provide access to the drug
- Honey has been considered an alternative treatment, with Manuka Honey demonstrating antibacterial and anti-inflammatory properties
- Surgical removal of fungal debris and allergic mucin has been shown to decrease fungal-specific IgE levels
Diagnosis of Invasive Fungal Rhinosinusitis (IFRS)
- White or black mucosal discoloration can suggest tissue ischemia or necrosis
- CT scan is the initial radiologic investigation of choice, with certain features suggestive of IFRS
- Middle turbinate biopsy shows high sensitivity (86%) and specificity (100%) in confirming acute invasive FRS
Treatment of IFRS
- Liposomal amphotericin B is generally reserved for patients with renal impairment or failure of standard amphotericin B
- Triazoles (fluconazole, itraconazole, and variconazole) are effective in treating invasive FRS without nephrotoxicity
- Triazoles are not effective against Mucorales species and should not be used without excluding their presence
- Treatment centers on improving the host immune response
Fungal Rhinosinusitis
- Fungal rhinosinusitis (FRS) is categorized into noninvasive and invasive types.
- Noninvasive FRS includes saprophytic fungal infection, fungal ball, and fungus-related eosinophilic FRS, including allergic fungal rhinosinusitis (AFRS).
- Invasive FRS includes acute invasive (fulminant) FRS, granulomatous invasive FRS, and chronic invasive FRS.
- Noninvasive FRS can progress to invasive forms with changing immunologic status.
- Fungus-related eosinophilic FRS is the most common type of FRS.
Saprophytic Fungal Infection
- Saprophytic fungal infection refers to visible fungal colonization of mucus crusts seen within the nose and paranasal sinuses.
- Patients are usually asymptomatic or may present with a foul odor.
- Often associated with previous sinus surgery.
- Dysfunction in mucociliary transportation after surgery leads to crust formation, creating a platform for fungal spores.
- Treatment usually involves endoscopic cleaning of the infected crust.
Fungal Ball
- A dense accumulation of extramucosal fungal hyphae, usually within one sinus, most commonly the maxillary sinus.
- Seen more commonly in immunocompetent, middle-aged and elderly females with a history of dental procedures.
- Diagnosed based on radiographic findings, presence of cheesy debris, accumulation of fungal hyphae without tissue invasion, nonspecific chronic inflammation, and absence of eosinophil predominance or granuloma.
- Management involves wide opening of the involved sinus and complete removal of the fungal debris.
Fungus-Related Eosinophilic FRS, Including AFRS
- Allergic fungal rhinosinusitis (AFRS) is a noninvasive fungal sinusitis resulting from an allergic and immunologic response to fungal hyphae in the sinuses.
- Prevalence of FRS among chronic rhinosinusitis (CRS) patients undergoing surgery is 12-47%.
- AFRS is the most common form of FRS, accounting for 56-72% of FRS cases.
- AFRS is more prevalent in the southern United States and along the Mississippi basin.
- AFRS typically affects young immunocompetent adults with a higher male-to-female ratio.
Pathophysiology of AFRS
- Pathophysiology of AFRS remains debated.
- The immunologic theory suggests an atopic host exposed to fungi develops type I and type III hypersensitivity responses, leading to intense inflammation.
- The eosinophilic theory suggests that fungi in the sinonasal mucosa triggers eosinophil chemotaxis, resulting in inflammation.
- The term eosinophilic mucin rhinosinusitis (EMRS) has been proposed for nonallergic fungal sinusitis, characterized by similar histological features but without the presence of fungus.
- Both AFRS and EMRS may have similar phenotypic endpoints, highlighting the complexity of the condition.
Diagnostic Criteria for AFRS
- The Bent and Kuhn criteria for AFRS include five major criteria: evidence of type I IgE-mediated hypersensitivity, nasal polyposis, characteristic CT findings, eosinophilic mucus, and positive fungal smear.
- The St. Paul's Sinus Centre criteria for AFRS include five major criteria: immunocompetent patient, presence of nasal polyposis, characteristic CT findings, presence of allergic mucin, and positive fungal cultures or presence of fungal hyphae on staining.
Clinical Presentation of AFRS
- Symptoms are often subtle and similar to chronic sinusitis with nasal polyposis.
- Suspicion should be high in young patients with uni- or bilateral nasal polyposis, thick mucus, characteristic CT findings, and responsiveness to oral steroids.
Allergic Fungal Rhinosinusitis (AFRS)
- AFRS is characterized by nasal obstruction, thick/crusty nasal discharge, and pain.
- AFRS can cause severe headaches, facial pain, anosmia, visual disturbances, and facial distortion.
- Presence of nasal polyps is universal in AFRS patients, often unilateral or bilateral but asymmetric.
- Thick yellow or brown mucus resembling "peanut butter" can be present among the polyps.
- Patients with AFRS have elevated IgE levels.
- Total IgE levels are not useful as a screening tool, but can be used to monitor clinical activity during treatment.
- IgE levels correlate with mucosal stage and often elevate before worsening of the clinical stage.
Diagnosis
- Diagnosis is usually made using clinical findings and supported by radiological studies.
- Diagnosis requires demonstrating a fungus-specific IgE, which can be achieved through skin prick testing or RAST.
- Good concordance exists between skin prick and RAST tests for fungal and non-fungal antigens in AFRS patients.
Radiological Tests
- CT without contrast is the imaging of choice for suspected AFRS.
- Focal or diffuse areas of hyperintensity on CT indicate calcium and manganese deposits in necrotic debris and allergic mucin.
- The "double density" or "rail-track" sign is visible on CT due to differing densities between allergic mucin and necrotic fungal debris.
- MRI with contrast is considered when AFRS diagnosis is uncertain or when intracranial/intraorbital complications are suspected.
- Protein content and viscosity of secretions determine MRI signal intensity.
Radiological Staging
- The Lund-MacKay system is used to assess the severity of chronic rhinosinusitis on CT scans.
- The Lund-MacKay system does not assess bony erosion and expansion seen in advanced AFRS.
- A 24-point radiologic staging system proposed by Wise et al. assesses bony remodeling (erosion or expansion).
- Male and African American AFRS patients tend to have higher scores on this system.
Differentiation from Invasive Fungal Rhinosinusitis (IFRS)
- CT and MRI features can differentiate AFRS from IFRS.
- AFRS typically exhibits heterogeneous opacification on CT and low signal intensity with peripheral enhancement on T1 and T2-weighted MRI.
- IFRS features include homogeneous opacification on CT and intermediate signal intensity on T1-weighted MRI with low to very low signal on T2-weighted MRI.
Histopathology
- The hallmark of AFRS is the presence of allergic mucin.
- Allergic mucin is thick, tenacious, highly viscous, and can range in color from yellow to dark green.
- Histologically, allergic mucin contains eosinophils, inflammatory cells, Charcot-Leyden crystals, and fungal hyphae.
- Fungal hyphae are usually not stained with H&E but can be detected using Grocott's or Gomori's methenamine silver (GMS) stain.
Fungal Culture
- Positive fungal culture supports AFRS diagnosis, but its absence does not exclude it.
- Positive fungal culture in AFRS patients ranges from 49% to 100% depending on the culture method.
- Positive fungal culture confirms the presence of saprophytic fungal growth, but not necessarily AFRS.
Management
- AFRS requires a comprehensive long-term management program with regular follow-up.
- Treatment typically involves surgery followed by a postoperative medical regimen.
Surgical Treatment
- Functional endoscopic sinus surgery (FESS) is the gold standard for AFRS surgical treatment.
- FESS aims to remove polyps, allergic mucin, and fungal debris, restoring sinus ventilation and drainage.
- Image-guided surgery is recommended to ensure complete removal of allergic mucin and open drainage pathways.
- Incomplete surgical resection can lead to early disease recurrence.
Endoscopic Staging of Mucosal Disease Post Surgery
- Regular follow-up and documentation of sinonasal mucosa post-surgery is crucial.
- This allows for monitoring disease status and response to adjunctive medical treatments.
Allergic Fungal Rhinosinusitis (AFRS)
- AFRS is a condition characterized by chronic inflammation of the sinuses caused by fungal allergens
- Can lead to sinus polyps, mucocele (sinus fluid buildup), and bone erosion
- Often requires surgery for treatment
Post-Surgical Management
-
Kupferberg staging system used for assessing AFRS post-surgery
- 4 stages:
- Stage I: Normal mucosa
- Stage II: Mucosal edema or allergic mucin
- Stage III: Polypoid edema or allergic mucin
- Stage IV: Sinus polyps and fungal debris
- 4 stages:
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Philpott-Javer endoscopic staging system is a more sensitive staging system addressing limitations of the Kupferberg system
- Scores each sinus cavity individually (0-9) based on mucosal inflammation
- Additional point awarded for allergic mucin presence
- Maximum score per sinus: 10
- Maximum score per side: 40
- Maximum bilateral score: 80
- More recently, the olfactory cleft is also included as a separate score, increasing maximum bilateral score to 100 (50 each side)
Medical Treatment
- Systemic medications:
-
Corticosteroids are widely used in the perioperative period
- Preoperative use can reduce intraoperative bleeding and polyp size
- Postoperative regimen initially based on protocol for ABPA
- Long-term use can be necessary to prevent recurrence, generally for at least 3 years with a minimum of 6 months with normal sinus mucosa without steroids
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Proposed post-operative corticosteroid regimen:
- 40 mg daily for 4 days
- 30 mg daily for 4 days
- 20 mg daily for 1 month
- 0.2 mg/kg daily for 4 months, maintaining stage 0
- 0.1 mg/kg daily for 2 months, including intranasal corticosteroids
-
Antifungals are an option in cases of recalcitrant fungal sinusitis
- Can be a steroid-sparing alternative
- Oral itraconazole:
- Initial higher doses (400 mg/day for 1 month, 300 mg/day for 1 month, 200 mg/day for 1 month)
- Lower doses (200-300 mg daily) show promise for longer disease remission
- Side effects:
- Elevated liver enzymes
- Congestive heart failure
- Nausea, rash, headache, malaise, fatigue, edema
- Hepatotoxicity, including liver failure and death (rare)
- Further research is needed to define optimal antifungals and dosing for AFRS patients
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Corticosteroids are widely used in the perioperative period
Topical Medications
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Corticosteroids:
- Standard treatment for AFRS, particularly post-operatively
- Provide higher drug concentration in the target tissue (sinonasal mucosa) without systemic side effects
- Budesonide is increasingly popular:
- Can be administered as drops, sprays, or low volume saline rinses
- Effective for asthma treatment
- Delivery methods include inhaled budesonide and budesonide nasal irrigation
-
Antifungals:
- Topical options are explored to minimize systemic side effects
- Limited research:
- Evidence on topical antifungal effectiveness in AFRS is scarce
- Studies in CRS patients have not shown significant benefit
- Future controlled trials are needed to clarify their role in AFRS
Immunotherapy
-
Specific immunotherapy (IT):
- Repeated administration of antigen in increasing doses to reduce allergen sensitivity
- Mechanism:
- Decreases production of allergen-specific IgE
- Increases production of IgG4 blocking antibodies, interfering with IgE-antigen reaction
- Traditionally avoided in ABPA due to possible immune complex-mediated reactions
-
Antifungal IT in AFRS
- Fungal antigen can be surgically removed, reducing IgE levels
- IT may be beneficial after surgery
- Early retrospective studies had mixed results
- Fungal IT appears safe and effective in many patients
- Large, well-designed controlled trials are needed to prove its effectiveness and safety
Adjunctive Treatments
-
Manuka honey:
- Therapeutic honey with antibacterial and anti-inflammatory properties
- Effective against broad-spectrum bacteria
- Active ingredient: methylglyoxal (MGO)
- In-vitro studies show promise for AFRS treatment
- Further research needed to confirm effectiveness in treating AFRS
Allergic Fungal Rhinosinusitis (AFRS)
- Common symptoms: Nasal polyps, sinusitis, obstruction, pressure, pain, loss of smell
- Specific to AFRS: Eosinophils in nasal mucosa, type 1 hypersensitivity reaction to fungi
- Treatment: Surgery can be effective for some patients, but often requires repeat treatments
-
Fungal immunotherapy (IT): Used in AFRS, but evidence is limited
- Some patients respond well, but not all do
- Overall, fungal IT is less effective in AFRS than in other fungal allergies
-
Honey irrigation: Shown to be effective at eradicating biofilms in the sinuses, but results in AFRS patients are mixed
- A few studies have shown improvement, but larger studies are needed
- Future research should focus on the optimal concentration and regimen for use
Invassive Fungal Rhinosinusitis (IFRS)
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Caused by: Fungi invading sinus tissue
- Usually occurs in immunocompromised patients, such as those with diabetes or AIDS
- Types: Acute (fulminant), Granulomatous, Chronic
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Acute (fulminant) IFRS:
- Less common but life-threatening
- Occurs in immunocompromised patients
- Symptoms: fever, cough, nasal crusting, epistaxis, headaches
- Treatment: Early and aggressive surgery to remove infected tissue with antifungal therapy
-
Granulomatous IFRS:
- Occurs in patients with a healthy immune system
- More common in Sudan, India, Pakistan & Saudi Arabia
- Symptoms: Swelling in cheek, nose, sinuses, and orbital area
- Treatment: Surgery and antifungal therapy
-
Chronic IFRS:
- Slowly destructive disease
- Less common than acute and granulomatous
- Similar to acuteIFR, but infection lasts more than 12 weeks.
- Treatment: Surgery & antifungal therapy
Treatment Options
-
Anti-IgE therapy: omalizumab (Xolair):
- Used in severe asthma and allergic rhinitis
- Promising results in small studies for AFRS, but more research is needed
Future directions:
-
Larger randomized controlled trials on Manuka honey and anti-IgE therapy are needed to confirm their efficacy in treating AFRS.
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Understanding the pathophysiology of AFRS and developing new treatments to target the underlying mechanism is crucial for achieving a complete cure.### Fungal Rhinosinusitis (FRS)
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FRS is a rare but important part of the chronic rhinosinusitis (CRS) spectrum
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FRS subtypes include noninvasive FRS, invasive FRS, chronic invasive FRS, and granulomatous invasive FRS
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FRS is typically found in immunocompetent patients, but also presents in patients with varying degrees of immune system compromise, including diabetes mellitus, corticosteroid use, and AIDS
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Aspergillus Fumigatus is the most common fungus associated with FRS
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The clinical presentation of chronic and granulomatous invasive FRS is similar, with frequent orbital involvement
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The ethmoid and sphenoid sinuses are the most commonly affected sinuses with FRS
Histopathology of FRS
- Fungal invasion of the sinonasal mucosa with a dense accumulation of fungal hyphae is notable
- Vascular invasion and sparse inflammatory reaction can also be present
- Histology is key in distinguishing FRS subtypes
Chronic Invasive FRS
- Often involves a time course of over 12 weeks
- The prognosis and management for chronic and granulomatous invasive FRS are the same
- The term subacute invasive FRS is recommended for patients with a time course between 4 and 12 weeks
Fungus-related Eosinophilic FRS
- Most common subtype of FRS
- Difficult to understand and manage, with ongoing controversy
- Recent research supports an immune-mediated component
Diagnostic Tools
- Radiological findings can provide clues, but histology is crucial for accurate diagnosis
- Correct diagnosis is essential because management strategies vary significantly between subtypes
Management of FRS
- Antifungal agents are usually not required for noninvasive FRS
- Antifungal agents are first-line treatment for invasive FRS.
- Invasive FRS is treated with antifungal agents
Allergic Fungal Rhinosinusitis (AFRS)
- AFRS is a unique subtype of FRS
- AFRS represents a significant clinical challenge due to its complexity and management difficulties
Imaging of AFRS
- CT and MRI are important for visualizing bone erosion and other characteristics of AFRS
- Signs of AFRS on imaging include:
- Mucosal thickening
- Opacification of the sinuses
- Air-fluid levels
- Bone erosion
Management of AFRS
-
Treatment often involves a multimodal approach, including:
- Surgical intervention
- High-dose systemic corticosteroids
- Long-term intranasal steroids
- Antifungal therapy
- Immunotherapy
Ongoing Controversies
- The exact role of fungi in the pathogenesis of chronic rhinosinusitis is debated
- The optimal management strategies for AFRS continue to be refined with ongoing research
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Description
This quiz explores the epidemiology, presentation, and immunological aspects of Allergic Fungal Rhinosinusitis (AFRS). Learn about the prevalence rates among chronic rhinosinusitis patients, typical symptoms, and key immunologic tests to diagnose AFRS. Understand how geographic variations affect the occurrence of this condition.
