Oral granulomatosis with polyangiitis a systematic review

Abstract

Objective: Granulomatosis with polyangiitis is an unusual multisystemic inflammatory disease, with vasculitis of small- and medium-sized vessels, with a predilection for upper lower airways and kidneys. The etiology remains unknown although it may originate from different stimuli, in genetically susceptible patients.

Materials and methods: A detailed database search was performed. The variables were demographics, localization, histopathological findings, antineutrophil cytoplasmic autoantibody, cytoplasmic (c-ANCA) tests, treatment, and follow-up.

Results: Fifty-two cases were identified; the mean age was 49.6 years, with a range from 6 to 87 years. It was most frequently seen in females (57.7%). The most common race was white (59.6%). The most frequent location was in the maxillary gingiva (28.8%), followed by both the upper and lower gingiva (19.2%). The most common clinical presentation was "strawberry gingivitis" (61.5%). The main symptom was pain, in 50%. Regarding the c-ANCA test, it was positive in 71.2% of cases. The most common therapy was prednisone and cyclophosphamide, utilized in 51.9%. The average follow-up was 23.6 months, and 88.5% of patients were still alive at follow-up.

Conclusion: The diagnosis initially was difficult to establish, an early diagnosis and treatment are mandatory. If untreated the disease can be associated with morbidity and mortality. For the oral clinician, this disease needs to be addressed in the differential diagnosis of oral lesions.

Keywords: antineutrophil cytoplasmic; gingivitis; granulomatosis with polyangiitis.

Figure 1

Flowchart

Figure 2

Legend clinical strawberry gingivitis and the molecular process. Genetic environmental factors and/or infectious (Staphylococcus aureus) increase the risk of granulomatosis with polyangiitis (GPA) to initiate or relapse. Overproduction of autoantibodies directed mainly against proteinase 3 (PR3‐ANCA). PR3 is expressed at the neutrophil surface, whereas myeloperoxidase is not. Increasing production of tumor necrosis factor (TNF‐α). Inducing expression of major histocompatibility complex, Class II, DP beta 1 (HLA‐DPB1) considerably more prevalent in patients with GPA. Also polymorphisms in the genes encoding 1‐antitrypsin (SERPINA1) and PR3 (PRTN3). This results in early neutrophil apoptosis which, combined with decreased clearance of apoptotic neutrophils by macrophages, induces the release of toxic mediators that convert the vessel inflammation to necrotizing vasculitis. Monocytes may also be activated by circulating ANCA, allowing granuloma formation. c‐ANCA, cytoplasmic antineutrophil cytoplasmic antibody; IgG, immunoglobulin G; IL‐1, interleukin‐1; PCNA, proliferating cell nuclear antigen.