Objective The purpose of this review is to update the oral manifestations of Granulomatosis with polyangiitis. Study Design A systematic review was conducted through a comprehensive electronic search up to and including February 2021, using the search strategy determined in five electronic databases: National Library of Medicine, Washington, D.C. (MEDLINE-PubMed); Embase®; ISI Web of Science; Cochrane; and LIVIVO. Results Ninety-nine published articles were included in this review and reported a total of 126 cases of oral manifestation of GPA. The average age of the patients was 43.66 years and the most common systemic alterations were hypertension, diabetes, and renal failure. The most reported systemic signs were weight loss, fever, pulmonary alterations, and musculoskeletal involvement. Most cases had localized GPA and most had oral involvement in only one location, most prevalently gingiva, followed by oropharynx and hard palate. Predominant lesions were ulcer, followed by strawberry gingivitis, presenting painful symptoms in 73% of cases. Most prevalent biopsy site was gums, followed by palate. Conclusion Oral manifestations of GPA are predominantly symptomatic ulcer and strawberry gingivitis affecting mainly gingiva and oropharynx. Acknowledgements This study was financed by CNPq-Brazil (MCTIC Universal no 408884/2018-5), FAPEMIG and CAPES (Finance Code 001). The purpose of this review is to update the oral manifestations of Granulomatosis with polyangiitis. A systematic review was conducted through a comprehensive electronic search up to and including February 2021, using the search strategy determined in five electronic databases: National Library of Medicine, Washington, D.C. (MEDLINE-PubMed); Embase®; ISI Web of Science; Cochrane; and LIVIVO. Ninety-nine published articles were included in this review and reported a total of 126 cases of oral manifestation of GPA. The average age of the patients was 43.66 years and the most common systemic alterations were hypertension, diabetes, and renal failure. The most reported systemic signs were weight loss, fever, pulmonary alterations, and musculoskeletal involvement. Most cases had localized GPA and most had oral involvement in only one location, most prevalently gingiva, followed by oropharynx and hard palate. Predominant lesions were ulcer, followed by strawberry gingivitis, presenting painful symptoms in 73% of cases. Most prevalent biopsy site was gums, followed by palate. Oral manifestations of GPA are predominantly symptomatic ulcer and strawberry gingivitis affecting mainly gingiva and oropharynx.
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