Oral manifestations of granulomatosis with polyangiitis - Clinical and radiological assessment

Abstract

Background/purpose: Granulomatosis with polyangiitis (GPA) is a type of primary systemic vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). GPA mainly affects medium and small vessels and may manifest in different organs, most commonly upper respiratory tract. Oral lesions occur in 6-13% of GPA patients and might be the first symptom observed by the patient. This study presents the evaluation of orofacial manifestations of patients with GPA.

Materials and methods: Prospective study was conducted between June 2014 and November 2017 in Department of Allergy and Immunology at University Hospital in Krakow. Patients diagnosed with GPA, after medical assessment, were examined including precise dental inspection, bacterial and fungal flora of oral cavity evaluation and Cone Beam Computed Tomography (CBCT) imaging.

Results: Nine patients were enrolled in the study. Characteristic for GPA strawberry gingivitis was observed in one patient. Bone destruction and inflammatory lesions in paranasal sinuses was confirmed by CBCT in 55.6% of patients. Fungal infection was revealed in 66.7% of patients.

Conclusion: These findings oblige dentists to consult patient with laryngologist or internal medicine physician to establish further diagnostic approach, because early diagnosis of GPA is crucial for implementing appropriate treatment and preventing chronic organ damage.

Keywords: ANCA; Gingivitis; Granulomatosis with polyangiitis; Oral mucosa; Vasculitis.

Fig. 1

A. Healing stage of strawberry gingivitis in granulomatosis with polyangiitis (GPA) patient. B. Histopathological pictures of healing stage of strawberry gingivitis. Small vessels of mucosal stroma with features of wall hyalinisation, lumen obliteration and minor inflammatory infiltrate.

Fig. 2

Coated tongue with Candida albicans infection in granulomatosis with polyangiitis (GPA) patient.

Fig. 3

Cone beam computed tomography (CBCT) image. Sinus lesions in the course of granulomatosis with polyangiitis (GPA). Maxillary sinuses destruction with the remodeling and thickening of their walls. The lumen of the maxillary sinuses is significantly reduced on both sides, partially filled with bone masses and soft tissue masses. Partial bone loss of the nasal cavity lateral walls on both sides. On the left side, the soft tissue masses pass from the maxillary sinus to the lumen of the nasal cavity to form a longitudinal structure. No nasal turbinates on both sides.

Fig. 4

Cone beam computed tomography (CBCT) image. The sinuses lesions that correspond to sinusitis in the course of granulomatosis with polyangiitis (GPA). Bilateral destruction and heterogeneous remodeling of bone structure with bone and soft tissue masses filling the sinuses.