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. The gene discussed is PRTN3; the disease is acute kidney injury.