A 51-year-old male presented to the emergency department with pain and facial swelling in the right cheek, which had been ongoing for 2 to 3 months. In the emergency department, a computerized tomography (CT) scan showed inflammation in the right maxillary sinus with mucosal thickening. A complete blood count (CBC) with differential and comprehensive metabolic panel (CMP) were both within normal limits. He was started on a 10 days course of oral antibiotics and fluticasone nasal spray.
Nine days later the patient presented to his primary care physician with concern for worsening right facial swelling, despite being on an antibiotic, and represented to the emergency department. The patient was started on vancomycin, piperacillin/tazobactam, and dexamethasone. A magnetic resonance image (MRI) of the brain was obtained and revealed a mass within the right infratemporal and pterygopalatine fossae extending into the inferior right orbital fissure along V2 and the vidian nerve, causing concern for potential malignancy (Figures 1 and 2).
He was transferred to a tertiary care center for evaluation and management. On physical examination showed diminished sensation at the right maxillary division of the trigeminal nerve (V2) but no double vision. His right face was swollen but not fluctant and he had trismus. The patient was taken to the operating room for biopsy. The sinonasal mucosal surface all appeared normal. The posterior wall of the maxillary sinus was removed to allow access to the mass, the internal maxillary artery appeared necrotic. Histological assessment demonstrated multiple arteries with partial to complete luminal obliteration associated with loosely formed non-necrotizing granulomas, consistent with granulomatous vasculitis (Figure 3). Serum studies were positive for antinuclear antibodies (ANAs) but were negative for anti-neutrophil cytoplasmic antibodies (ANCA).
The patient was evaluated by an infectious disease specialist and was negative for human immunodeficiency virus (HIV), hepatitis B and C, Mycobacterium tuberculosis, and other diverse opportunistic organisms. The patient was referred to a rheumatologist and nephrologist. Urine sediment showed 1 to 2 dysmorphic red blood cells (RBCs) per high-power field and 3 to 5 monomorphic RBCs per high-power field, concerning for renal involving vasculitis. The patient was diagnosed with ANCA-negative granulomatosis polyangiitis and was started on immunosuppressive treatment with prednisone and mycophenolate. At his 1-month follow-up with a nephrologist and otolaryngologist, the patient reported improved symptoms with decreased facial swelling, the ability to open his mouth, and improved sensation on the right side of his face. An informed written consent was obtained from the patient for publication of this case report.