Pham, Michael M.

ABSTRACT Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis that commonly presents with multiorgan involvement of the upper airways, lungs, and kidneys. We report a 55 year-old-man who presented with painful oral ulcers, diffuse joint pain, epistaxis, and intermittent maroon bowel movements. Infectious evaluation was unremarkable. Rheumatologic evaluation demonstrated elevated c-anti-neutrophil cytoplasmic antibody and proteinase 3 antibodies. Colonoscopy with terminal ileum biopsies demonstrated angiocentric inflammatory findings supportive of a diagnosis of GPA. This case was unusual in its presentation with predominately oral manifestations. A diagnosis of GPA should remain on the differential for patients with gastrointestinal bleeding, and these systemic symptoms require a high level of suspicion to lead to timely diagnosis.