title: Gingival granulomatosis with polyangiitis (Wegener's granulomatosis) as a primary manifestation of the disease.
abstract: Granulomatosis with polyangiitis (GPA) is a potentially lethal disease characterized by systemic necrotizing vasculitis, which affects small- and medium-sized blood vessels and is often associated with serum cytoplasmic antineutrophil cytoplasmic antibody. The upper and lower respiratory tract and kidney are the most involved sites, but oral lesions can be identified in 6-13% of the cases, whereas in only 2% of the cases, oral manifestations represent the first signal of the disease usually as gingival swellings or unspecific ulcerations. Without treatment, the mainstay of which is the combination of immunosuppressants and systemic corticosteroids, GPA may run a fatal course. In this report we describe an original case of GPA affecting a 75-year-old female patient referred to our service due to a gingival swelling with 3-month duration. Although the patient was correctly diagnosed and promptly treated, she died 3 months after the initial diagnosis.
authors.affiliation: Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, Brazil.
authors.forename: F P
authors.initials: FP
authors.lastname: Fonseca
authors.affiliation: Oral Medicine Department, Sírio-Libanês Hospital, São Paulo, Brazil.
authors.forename: B M
authors.initials: BM
authors.lastname: Benites
authors.affiliation: Department of Rheumatology, School of Medical Sciences, University of Campinas, Campinas, Brazil.
authors.forename: Alv
authors.initials: A
authors.lastname: Ferrari
authors.affiliation: Department of Rheumatology, School of Medical Sciences, University of Campinas, Campinas, Brazil.
authors.forename: Z
authors.initials: Z
authors.lastname: Sachetto
authors.affiliation: Periodontist at Private Practice, São Paulo, Brazil.
authors.forename: G V
authors.initials: GV
authors.lastname: de Campos
authors.affiliation: Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, Brazil.
authors.forename: O P
authors.initials: OP
authors.lastname: de Almeida
authors.affiliation: Oral Medicine Department, Sírio-Libanês Hospital, São Paulo, Brazil.
authors.forename: E R
authors.initials: ER
authors.lastname: Fregnani
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use*; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D005882:Gingival Diseases / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents
keywords: ANCA; Wegener's granulomatosis; granulomatosis with polyangiitis; necrotizing vasculitis; oral cavity