Head and Neck Manifestations of Eosinophilic Granulomatosis with Polyangiitis

Objective To conduct the first and only systematic review of the existing literature on head and neck manifestations of eosinophilic granulomatosis with polyangiitis to guide clinical decision making for the otolaryngologist. Data Sources PubMed, Cochrane Library, Scopus, and LILACS. Review Methods A systematic review of the aforementioned sources was conducted per the PRISMA guidelines. Results From an initial 574 studies, 28 trials and reports were included, accounting for a total of 1175 patients with eosinophilic granulomatosis with polyangiitis. Among clinical and cohort studies, 48.0% to 96.0% of all included patients presented with head and neck manifestations. In a distinct group of patients detailed in case reports describing patients presenting with head and neck manifestations, patients on average fulfilled 4.6 diagnostic criteria per the American College of Rheumatology. Furthermore, 95.8% of reported cases were responsive to steroids, and 60% required additional therapy. Conclusion Otolaryngologists are in a unique position for the early diagnosis and prevention of late complications of eosinophilic granulomatosis with polyangiitis. The American College of Rheumatology criteria should be relied on in the diagnostic workup. Close surveillance of these patients in a multidisciplinary fashion and with baseline complete blood counts, chest radiographs, and autoimmune laboratory tests is often necessary. Such patients with head and neck manifestations of the disease are nearly always responsive to steroids and often require additional immunosuppressive therapy or surgical intervention in cases of cranial neuropathies, temporal bone involvement, and refractory symptoms.