The ANCA-Associated Vasculitis is a group of rare diseases that affect blood vessels and are related to the presence of specific antibodies called antineutrophil cytoplasmic antibodies (ANCA). While respiratory and kidney involvement are the most common in ANCA-associated vasculitis and thus are part of the classification criteria, it is important to remember that vasculitis are diseases with the potential to cause multi-organ damage. We present this case to emphasize that a sequential approach is necessary for decision-making regarding treatment to prevent complications and improve quality of life, especially when they are refractory to first-line treatment.