The systemic vasculitides are rare conditions characterized by inflammation and necrosis of blood vessels. Those with predominately small vessel involvement, such as the ANCA-associated vasculitides (AAV), are the only ones to target the lungs with any regularity. However, these conditions can affect almost any organ system, including the cardiovascular system. Obtaining an accurate diagnosis is crucial before committing a patient to long-term immunosuppressive therapy. The choice of treatment is guided by the organ systems involved, the severity of disease, and the presence of comorbid conditions. Newer treatments are emerging which provide more options in the therapeutic armamentarium.