Granulomatosis with Polyangiitis: A Clinical Case

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is characterized by inflammation and the destruction of small-and medium-caliber blood vessels in the presence of circulating ANCAs. Anti-neutrophil cytoplasmic antibody-associated vasculitis predominantly affects the lung and kidney with a multifactorial pathogenesis. This case refers to a 55-year-old woman with constitutional symptoms, hypoacusis, cough, and bloody sputum. Physical examination revealed polypnea and decreased lung auscultation at the bases; blood work showed elevated inflammatory parameters, acute kidney injury, and hematuria; pulmonary computed tomography revealed areas of peribronchial thickening in both lungs; immunological study resulted in positive ANCA-PR3. Corticotherapy and double immunosuppression with cyclophosphamide and rituximab were initiated, which resulted in clinical and analytical improvement. This case of granulomatosis with polyangiitis (GPA), with pulmonary, renal, cutaneous, and ear involvement, allows us to demonstrate the importance of timely clinical suspicion and initiation of immunosuppression for a favorable disease prognosis.