Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge

Abstract

Granulomatosis with polyangiitis (GPA) is an idiopathic vasculitis of medium and small arteries, characterized by necrotizing granulomatous inflammation. GPA typically affects upper and lower respiratory tract with coexisting glomerulonephritis. This disease is generally characterized by antineutrophil cytoplasm antibodies (ANCA), nevertheless, there are rare cases with negative ANCA. GPA affects people at any age, with predominance of the sixth and seventh decade of life. In 80%–95% of the patients the first symptoms of GPA are otorhinolaryngological manifestations of head and neck including nose/sinuses, ears, eyes, larynx/trachea, oral cavity, and salivary glands. Diagnosis of GPA is based on Criteria of the American College of Rheumatology. In clinical practice diagnosis, the presence of distinctive ANCA antibodies and biopsy of affected organ are crucial. GPA must be differentiated from neoplastic, infectious or inflammatory ulcerative lesions of the head and neck. The standard treatment procedure is divided into two essential phases, induction and maintenance. The induction phase is based on combination of systemic corticosteroid and immunosuppressant therapy, whereas the maintenance phase comprises corticosteroids and azathioprine/methotrexate supplementation. Surgical treatment ought to be considered for patients who are not responding to pharmacotherapy.

Unique case of granulomatous arteritis in a grey mouse lemur (Microcebus murinus) – first case description

“…GPA usually involves small-to medium-sized vessels of the upper and lower respiratory tract (Wojciechowska et al, 2016). Pauci-immune glomerulonephritis is a common feature (Jennette et al, 2013;Takeuchi et al, 2016), but this lesion was not seen in the present case.…”

“…The main histologic criteria are the presence of giant cells, which was a very prominent feature of the present case, and fibrinoid necrosis of vessel walls, which was less obvious. GPA is generally characterized by antineutrophil cytoplasm antibodies (ANCA) (Wojciechowska et al, 2016). Unfortunately, it was not possible to measure ANCA Lie, 1978, andJennette et al, 2013).…”

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

“…Overall, otologic manifestations are reported to constitute 36.4% of all cases while it was 13% in our GPA patients [21]. Otological manifestations namely otitis media and hearing loss can be the initial presentations of GPA [22][23][24]. It is reported that otitis media occurs in 25% of GPA patients and hearing loss in 6% while in our patients, otitis media was observed in 4.3% and hearing loss in 8.7% patients [22].…”

Laryngeal Primary Amyloidosis: Differential Diagnosis of Localized Granulomatosis with Polyangiitis. Report of Two Cases

“…It is important to emphasize that this diagnosis should be considered in cases of recurrent upper respiratory tract infections, airway infections, otitis, mucosal ulcers and laryngitis. Proper diagnosis and early treatment are essential to prevent irreversible damage in cases of GPA [8,9].…”