Vasculitis is an autoimmune connective tissue disease that targets blood vessels in the body, leading to inflammation and potential damage to vital organs. Living with vasculitis can be challenging for patients as there are periods when the disease is more active and often unpredictable in nature. Although there is no cure for vasculitis, improved understanding of the disease process and new treatments can improve quality of life for patients. This article provides an overview of the disease, particularly anti-neutrophil cytoplasmic antibody positive vasculitis, including diagnostic difficulties and the role of the nurse specialist in providing support and advice to patients and their families.