RESUMO
Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis primarily affecting small blood vessels and presenting with systemic manifestations, including those in the kidneys and respiratory tracts. Diagnosis involves clinical assessment and specific serological tests. This paper presents the case of a 68-year-old morbidly obese woman with chronic sinusitis, hypertension, and hyperlipidemia who developed a saddle nose deformity. Despite the absence of typical respiratory and renal symptoms, her laboratory results showed positive antineutrophil cytoplasmic antibodies (ANCA) and antinuclear antibodies (ANA), with a nasal septal biopsy confirming GPA. She was treated with methotrexate and folic acid. This case underscores the variability of GPA presentations and the critical importance of early diagnosis and treatment to prevent irreversible damage.