Granulomatosis with Poliangeitis (Wegener's Granulomatosis): Orofacial Manifestations. Systematic Review and Case Report

Abstract

Purpose: To present an update the orofacial manifestations of granulomatosis with polyangiitis (GPA) and present a clinical case with the initial signs in the oral cavity.

Materials and methods: A bibliographic search was performed on Pubmed with the keywords 'Wegener's granulomatosis', 'etiology', 'oral manifestations', 'oral cavity', 'gingiva'. The inclusion criteria were papers published in English in the last 10 years that made reference to clinical cases with in which the oral cavity was affected. The quality of the results was assessed with 'The 2013 Care Checklist'.

Results: Nineteen clinical cases were analysed. The average quality was 7.68/13 (range 5-10/13). 73.7% of patients were women, the most frequent area for the lesions was the gingiva and the most prevalent lesion was gingival hyperplasia. 68.4% of the patients had this lesion as a first sign, 21.1% as a progression and 10.5% as a recurrence. 68.4% of the lesions resolved once medical treatment was established.

Conclusion: GPA is a multisystem disorder associated with considerable morbidity and mortality if not treated. Early diagnosis improves the prognosis. The first manifestation of the disease can be seen in the oral cavity. It is important that dentists recognise the oral manifestation in order to improve the prognosis. Key words: granulomatosis, polyangiiitis, Wegener's granulomatosis.

Fig 1

a) Ulcer with everted border in oral mucosa of the left maxilla; b) large ulcer in the left tonsillar pillar; c) mucosal fenestrations; d) initial panoramic radiograph.

Fig 2

Histological images of the skin biopsy. a) Image of the skin biopsy taken with a magnifying lens. b) 50X detail of the skin biopsy. c) 100X detail of the skin biopsy; granulation tissue can be seen deep in the ulcer. d) 200X detail of the skin biopsy, showing a vessel with vasculitis.

Fig 3

a) Thoracic radiograph: Discrete bilateral pleural effusion with subjacent parenchyma atelectasis. The remaining parenchyma has no significant alterations. b) Thoracic CT: Bilateral pleural effusion with areas of atelectasis in the subjacent parenchyma. Ganglia of unspecific size in the mediastinal territory with no signs of adenopathy. c) PET-CT of maxilla: hypermetabolism in left maxilla related with an increase of parts density and a fistulous tract in relation to an infectious-inflammatory process. Small submandibular laterocervical adenopathy (slightly contrast enhanced), related to a reactive-inflammatory process.

Fig 4

a) Check-up after dental treatment. Patient is stable. b) Healed mucosal fenestration.

Fig 5

a) Follow up of the oral lesions one year after diagnosis. Patient is stable and pending prosthetic dental rehabilitation. b) Healed ulcer in maxilla. c) Patient scheduled to have conservative treatment in teeth of the mandible. d) Panoramic radiograph (December 2018).