Head and Neck Manifestations of Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review.

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

A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

The clinical presentation of Systemic Lupus Erythematosus (SLE) is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA), polyarteritis nodosa (PAN), and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA), among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition