Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases characterized by inflammation and damage to small blood vessels.
This inflammation is caused by white blood cells called neutrophils that erroneously attack the cells lining blood vessels. In most cases, neutrophils go haywire because patients produce self-reactive antibodies called ANCAs that attach to these immune cells and wrongly activate them.
Depending on the organ or tissue where blood vessel damage occurs, AAV can cause many different symptoms. If left untreated, the prognosis of AAV patients is poor, but with the introduction of immunosuppressive treatments and high-dose steroids to manage the condition, outcomes have improved dramatically.
In some cases, vasculitis can be cured quickly; in others, the disease becomes chronic and is often marked by temporary states of remission, in which treatments markedly reduce disease activity, followed by periods in which therapies lose efficacy and symptoms reappear.
Factors that affect the prognosis of ANCA vasculitis
The prognosis of ANCA vasculitis is difficult to define because the disease can affect a variety of organs and body systems. Forms of vasculitis that cause damage to major organs, such as the kidneys or the cardiovascular system, can have a negative impact on overall life expectancy.
The few studies that have reviewed the long-term survival of ANCA vasculitis patients indicate that mortality is higher than in the general population, despite recent advances in diagnosis and treatment.
Mortality is particularly higher in the first year after diagnosis, especially for patients with severe kidney problems and advanced age at diagnosis, with infection and active vasculitis as the main causes of death. Such infections sometimes arise from the use of immunosuppressive medications to manage AAV, which suppress the body’s immune system and its ability to fight off threats.
Recent studies indicate that the kind of ANCA a patient has is also an important determinant of a patient’s prognosis — a disease’s likely course — including relapse rate, response to treatment, and other outcomes. ANCA antibodies against the proteinase 3, or PR3, protein are associated with twice as many relapses as those against myeloperoxidase (MPO).
Periodic checkups with a physician
Regular checkups are important for patient safety because the symptoms can change significantly over the disease’s course. Some treatments for vasculitis, particularly corticosteroids, also can suppress the immune system and increase the risk of infection.
Understanding which symptoms are associated with the disease and which ones may have arisen due to treatment is also important for managing ANCA vasculitis.
Last updated: Dec. 7, 2021
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