A Case of Clinically Diagnosed ANCA Negative Granulomatosis With Polyangiitis

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.

Automated summary

GPA is a systemic necrotizing vasculitis that often involves the upper and lower airways and kidneys. ANCA negative GPA can present with nasal symptoms as the initial manifestation.