Clin Exp Otorhinolaryngol.  2016 Mar;9(1):8-13. 10.21053/ceo.2016.9.1.8.

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

• Wojciechowska J
• Krajewski
• Krajewski
• KrÄ™cicki

• KMID: 2166274
• DOI: http://doi.org/10.21053/ceo.2016.9.1.8

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.

Keyword

Granulomatosis with Polyangiitis; Wegener Granulomatosis; Otolaryngology

MeSH Terms

Adrenal Cortex Hormones
Antibodies
Antibodies, Antineutrophil Cytoplasmic
Arteries
Biopsy
Cytoplasm
Diagnosis
Drug Therapy
Ear
Glomerulonephritis
Head
Humans
Inflammation
Mouth
Neck
Otolaryngology
Respiratory System
Rheumatology
Salivary Glands
Ulcer
Vasculitis
Wegener Granulomatosis
Adrenal Cortex Hormones
Antibodies
Antibodies, Antineutrophil Cytoplasmic

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