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Current Rheumatology Reviews

Editor-in-Chief

ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Case Report

Atypical Debut of Granulomatosis with Polyangiitis as Acute Tonsilitis and Strawberry Gum: A Case Report

Author(s): Cristiana Sieiro Santos*, Clara Moriano Morales, Carolina Álvarez Castro and Elvira Diez Alvarez

Volume 19, Issue 1, 2023

Published on: 17 June, 2022

Page: [102 - 107] Pages: 6

DOI: 10.2174/1573397118666220413120904

Price: $65

Open Access Journals Promotions 2
Abstract

Abstract: Background: Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis characterized by necrosis, granulomatous inflammation, and vasculitis. It is characterized by the triad of the upper and lower respiratory system, lung, and kidney disease. Although it is usually a multisystemic disease, limited forms have also been described, and otolaryngological involvement is the first manifestation in up to 80-95% of the cases.

Case Presentation: In this report, we describe the case of an ANCA negative patient with a limited form of GPA that presented a necrotic lesion confined to the right tonsil compatible with granulomatosis with polyangiitis, which later presented positive ANCA antibodies. Oral lesions may be the initial manifestation of GPA, and systemic involvement can be presented within weeks or months. Although the oral manifestations have been well described, the initial presentation with oral lesions is very rare, and presentation with oropharyngeal manifestation is even rarer. This disease is generally characterized by anti-neutrophil cytoplasmic antibodies (ANCA); however, there are rare cases with negative ANCA.

Conclusion: The diagnosis was established based on the clinical presentation and the histopathological findings of the characteristic inflammatory pattern.

Keywords: Tonsillitis, granulomatosis, strawberry gum, vasculitis, GPA, fibrinoid necrosis, polyangiitis.

Graphical Abstract
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