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Wise et al.

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To differentiate AR [allergic rhinitis] from atrophic rhinitis, it should be noted that AR is an immunological response to a benign substance, the allergen, that manifests primarily as nasal inflammation. AR is IgE-dependent 235 and characterized by sneezing, clear rhinorrhea, watery eyes, and nasal and ocular pruritus. 1 This condition has a clear distinction from ENS and atrophic rhinitis in its clinical presentation and pathophysiology. III.C.13. Autoimmune, granulomatous, and vasculitic rhinitis— Both the upper and lower airways can be affected by systemic disorders including vasculitic, granulomatous, and autoimmune diseases. Commonly, affected patients may present with nonspecific sinonasal symptoms (nasal obstruction, rhinorrhea, facial pain, and loss of smell) mimicking AR. Allergy testing will, however, be negative or not clinically relevant. Clinicians should consider broadening the differential to consider systemic etiologies if either crusting or recurrent epistaxis is seen. 236 Oral steroids are the mainstay of treatment for the entities discussed in this section, although the recent introduction of monoclonal antibodies targeting specific biomarkers represents an important hallmark for future therapy. Granulomatosis with polyangiitis.: Previously referred to as Wegener’s disease, granulomatosis with polyangiitis (GPA) is an idiopathic disease characterized by necrotizing and granulomatous inflammation of the upper and lower airways (85%), glomerulonephritis (75%) and systemic vasculitis. 237-239 Limited forms of GPA involving only the head and neck may also be seen. GPA predominantly affects small to medium sized arteries and vein walls. 240 GPA affects both men and women in a similar proportion, being frequently diagnosed in the fourth to sixth decades of life. 240 In the US, estimated prevalence is 13 to 30 cases permillion people per 5-year period. Nasal symptoms include obstruction, rhinorrhea, recurrent epistaxis, crusting, and pain over the nasal dorsum. 237,241 Nasal mucosa disruption may lead to anosmia while tissue necrosis with secondary infection may lead to cacosmia. 236 Nasal endoscopy can reveal an erythematous, friable mucosa with crusting and granulation that is seen in the septum and inferior turbinate. 240 Patients with severe forms can present with nonvascular necrosis causing perforation or bony destruction of the nasal septum and/or other nasal structures. 242 Diagnosis is based on clinical symptoms, physical findings, radiological examinations, laboratory tests (positive c-ANCA [anti-nuclear cytoplasmic antibody] in 60–90%), and biopsy of affected tissue for pathological examination. 237,238,240 Profiling the nasal transcriptome in GPA reveals unique gene expression signatures related to innate immunity, inflammatory cell chemotaxis, extracellular matrix composition, and epithelial barrier integrity that may eventually be used clinically. 243,244 Treatment includes prednisone, cyclophosphamide, or methotrexate. 237,238,245 Rituximab, anti-CD20 monoclonal antibody, may be an effective therapy in refractory or relapsing c-ANCA vasculitis, 246 although additional study is needed. Eosinophilic granulomatosis with polyangiitis.: Previously known as Churg-Strauss Syndrome, eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small-sized vessel vasculitis with a prevalence of 1.3 cases per 100,000, 247 typically diagnosed in patients age 30 to 50 years. 236 Rhinitis (75% of patients) is one of the initial manifestations of EGPA, 248 in addition to CRS with nasal polyps (CRSwNP), and partial/total smell loss. 249 Diagnosis should be suspected in patients with asthma, with increased peripheral blood eosinophil

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Int Forum Allergy Rhinol . Author manuscript; available in PMC 2020 June 10.