Gingival hyperplasia as first sign of recurrence of granulomatosis with polyangiitis (Wegener’s granulomatosis): case report and review of the literature

BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener’s granulomatosis, is a rare systemic disease of unknown etiology which can affect all areas of the body, including the oral cavity. The typical oral manifestations occur as nonspecific erosive/ulcerative lesions of the oral cavity or appear with hyperplastic gingivitis, a so called “strawberry gingivitis”. CASE PRESENTATION: We report here about an extremely rare case with hyperplastic gingivitis as the first sign of recurrence of GPA in the absence of oral manifestations in the primary disease. A 72 year-old female was referred to our Department of Cranio-Maxillofacial Surgery with hyperplastic gingivitis. The patient was diagnosed with GPA already eight years before. We referred the patient to our Clinic of Internal Medicine where she was successfully treated with rituximab. At the follow-up visit, the patient showed complete remission of the hyperplastic gingiva. CONCLUSION: The often overlooked oral manifestation may be interpreted as the first evidence of resurgent GPA in general and therefore could be pathognostic for the disease. This case affirms the need of health professionals to be acquainted with orofacial manifestations of rare diseases such as GPA. As a consequence, dentists will be able to assist in diagnosing GPA more easily leading to a better prognosis for patients suffering from this disease.