A Case of Clinically Diagnosed ANCA Negative Granulomatosis With Polyangiitis

Abstract

Significance Statement: : Granulomatosis with polyangitis (GPA) is a systemic necrotizing vasculitis comprising of inflammation of small and medium-sized vessels.1 It typically presents with involvement of the upper and lower airways as well as the kidneys. If left untreated, end-organ damage may occur. Hematological investigations typically demonstrate the presence of antinuclear cytoplasmic antibodies (ANCA).2 Here, we discuss an unusual presentation of ANCA negative GPA, presenting initially with nasal symptoms.