ANCA-Negative Granulomatous Polyangiitis Presenting With Isolated Oral and Esophageal Involvement

Granulomatosis with polyangiitis (GPA) is a necrotizing small vessel vasculitis, which is most classically associated with lung and renal involvement. In this article, we present a young man who presented with oral ulcers, odynophagia, and foreign body sensation in the esophagus. Upper endoscopy revealed many esophageal ulcers with biopsies highly suspicious for GPA and no infectious etiologies identified on extensive testing. To our knowledge, this is a unique diagnosis of ANCA-negative GPA in a patient presenting with isolated oral and esophageal involvement.