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AFRS

Subscribe‪@theentingdoc‬ AFRS is coupled with the clinical entity of fungus ball (mycetoma) as a form of noninvasive fungal sinus disease, separate from and unrelated to invasive fungal sinus pathology. AFRS is a truly unique pathologic entity, defined largely by the presence of allergic fungal mucin, which is a thick, tenacious, eosinophilic secretion with characteristic histologic findings. This mucin is grossly and microscopically similar to that found in the lungs of patients with allergic bronchopulmonary aspergillosis (ABPA), and this pulmonary correlate helped guide the early understanding of the pathogenesis of AFRS.2 Since its initial characterization in the 1970s, AFRS has been the subject of much debate and controversy regarding its pathogenesis, diagnosis, classification, and optimal management. The Ochsner Journal Ochsner Clinic Foundation Allergic Fungal Rhinosinusitis: A Review Daniel Glass, MD and Ronald G. Amedee, MD Additional article information Abstract Background Allergic fungal rhinosinusitis (AFRS) is a relatively new and incompletely understood clinical entity with characteristic clinical, radiographic, and histopathologic findings. AFRS is often misdiagnosed. Recognition and understanding of this unique disease will lead to efficient diagnosis and treatment of this curable process. Methods The following is a review, conducted via a PubMed English language search, of the current diagnosis, pathogenesis, and treatment of AFRS. Results AFRS is an immune-modulated disease entity. The Bent and Kuhn diagnostic criteria are the standard for diagnosis of this disease that occurs because of an incompletely understood allergic mechanism. Multimodality treatment relies heavily on surgical therapy along with corticosteroid use and immunotherapy. Conclusions AFRS is a unique disease process that differs from other forms of sinusitis and as such requires that physicians understand its diagnosis and management to provide care for patients with this condition. Keywords: Allergic fungal sinusitis, allergic mucin, Aspergillus, Bent and Kuhn, Bipolaris, dematiaceous fungi, rhinosinusitis, sinusitis, type I and III hypersensitivity INTRODUCTION Allergic fungal rhinosinusitis (AFRS) was first reported as a distinct clinical entity in 1976.1 AFRS is coupled with the clinical entity of fungus ball (mycetoma) as a form of noninvasive fungal sinus disease, separate from and unrelated to invasive fungal sinus pathology. AFRS is a truly unique pathologic entity, defined largely by the presence of allergic fungal mucin, which is a thick, tenacious, eosinophilic secretion with characteristic histologic findings. This mucin is grossly and microscopically similar to that found in the lungs of patients with allergic bronchopulmonary aspergillosis (ABPA), and this pulmonary correlate helped guide the early understanding of the pathogenesis of AFRS.2 Since its initial characterization in the 1970s, AFRS has been the subject of much debate and controversy regarding its pathogenesis, diagnosis, classification, and optimal management. DIAGNOSIS Diagnosis begins with a thorough clinical history. Commonly, the patient will present with a history of sinus disease strongly recalcitrant to traditional medical and even surgical therapy aimed largely at bacterial rhinosinusitis.3 Several courses of antibiotics and topical nasal preparations may have been tried with little success. Unique features of AFRS that can alert the clinician to a possible diagnosis include a young (mean age is 22 years), immunocompetent patient with unilateral or asymmetric involvement of the paranasal sinuses, a history of atopy, nasal casts, and polyposis, and a lack of significant pain.4 Nasal casts are green to black rubbery formed elements made of allergic mucin. The presentation may be dramatic, with a significant number of patients presenting with proptosis, telecanthus, or gross facial dysmorphia.5 AFRS occurs throughout the United States, with increased prevalence in the Mississippi basin and southwestern states.6 The diagnostic dilemma is differentiating AFRS from other fungal entities involving the paranasal sinuses, including saprophytic fungal growth, mycetoma, eosinophilic mucin rhinosinusitis, and invasive fungal sinusitis. In 1994, Bent and Kuhn published their diagnostic criteria centered on the histologic, radiographic, and immunologic characteristics of the disease.7 Others have proposed several sets of criteria that have served to further the discussion of and investigation into this unique disease; however, the Bent and Kuhn criteria (Table 1) are largely regarded as the standard for diagnosis today. Patients must meet all the major criteria for diagnosis, while the minor criteria serve to support the diagnosis and describe individual patients but are not used to make a diagnosis.