Granulomatosis with Polyangiitis: clinical case

Abstract

Introduction: Granulomatosis with poliangeitis, previously known as Wegener’s granulomatosis, is a multisystemic disease characterized by necrosis in the upper or lower portions of the respiratory system, glomerulonephritis and necrotizing vasculitis. Though a rare disease, its presentation is acute and rapidly progressive. The radiological pattern includes parenchymal alteration in 50% of the patients, with lung consolidation seen on the CT SCAN or ground glass opacities secondary to diffuse pulmonary hemorrhage, usually bilateral. Currently, the median survival time with treatment is longer than 20 years. Case: This is the case of a female patient younger than 40 years-old who had a cavitated mass in her lungs. She was transferred from another hospital after receiving several antibiotic schemes without improving her clinical condition. She also received antituberculous treatment. At CAM Hospital, the diagnosis was made on the ground of histopatologic reports and immunologic tests. Corticoisteroids and inmunosupressive treatment improved definitively her clinical and radiologic picture. Discussion: Granulomatosis with Poliangiitis is a necrotizing inflammation involving the respiratory tract, lungs and kidneys. It is defined by the presence of ANCA PR3 positive antibodies in most patients. Its evolution varies and often tends to be chronic. It is treated with corticosteroids and immunosuppressors. After remission, the probability of recurrence goes from 50 to 70%. This could be be prevented with adequate follow-up and maintenance treatment.