Beyond the Norm: Diagnosing ANCA-Negative Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) - A Pathologist’s perspective

Abstract Granulomatosis with polyangiitis (GPA) is a systemic small- and medium-vessel vasculitis which is frequently linked to antineutrophil cytoplasmic antibodies (ANCAs). Patients with GPA may exhibit a wide range of signs and symptoms, including nonspecific constitutional symptoms, which may cause a delay in diagnosis. GPA, formerly known as Wegener’s granulomatosis, is an uncommon condition characterized by necrotizing granulomatosis of the upper and lower respiratory tracts and glomerulonephritis. We present a unique case of a 35-year-old male patient who presented with complaints of swelling in the submandibular region for 2 months along with hoarseness of voice for the last 1 year. No other systemic involvement was seen. Biopsy was done from the same site which showed features of GPA, but ANCA was negative in this patient. The literature for ANCA-negative GPA shows a limited number of cases since it is a difficult entity to diagnose. A careful histopathological diagnosis in correlation with the clinical picture and investigations is a must for a correct diagnosis.