Granulomatosis with Polyangiitis Initially Presenting with Sinonasal Tumor: A Case Report

Ear, nose and throat involvement is the most common clinical manifestation of granulomatosis with polyangiitis (GPA) or Wegener's granulomatosis, and sinusitis is the most frequent presenting symptom, followed by fever, arthralgia, cough, rhinitis, hemoptysis, otitis, and ocular inflammation. Patients with GPA may present upper respiratory symptoms such as nasal obstruction and epistaxis initially. However, this would lead to a delayed diagnosis and treatment. A 46-year-old man presented with chronic facial pain, numbness, and epistaxis for 3 weeks. Nasal inspection showed an ulcerating tumor in the right osteomeatal complex region. Chest film showed a cavitary mass in the left upper lobe. GPA was confirmed by evidence of necrotizing granulomatous inflammation of nasal tissue and positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA). Flare-up of GPA occurred after pulse therapy with high-dose corticosteroid followed by low-dose steroid; anti-CD 20 target therapy with rituximab combined with pulse therapy was then prescribed as an alternative initial induction therapy, followed by low-dose steroid plus hydroxychloroquine. Mycophenolate mofetil as maintenance therapy controlled the progression of GPA and led to remission of the facial and nasal symptoms and the lung mass 3 months after diagnosis of GPA.