Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis is an autoimmune disease that causes blood vessels to swell. Several types of ANCA vasculitis exist due to numerous causes, so patients diagnosed with ANCA vasculitis may display varied symptoms. The disease affects about 1 in 50,000 people and is more prevalent in middle-age Caucasian men and women.
What is an autoimmune disease?
In healthy people, the immune system identifies foreign bodies and produces antibodies that attack them before they can harm the body. Autoimmune diseases occur when the immune system produces abnormal antibodies, called autoantibodies, that target healthy cells and tissue within the body. In ANCA vasculitis, ANCAs attack a type of white blood cells called neutrophils. When under attack, the neutrophils break through the blood vessel containing them, causing vascular damage and swelling.
Types of ANCA vasculitis
Types of ANCA vasculitis are grouped by the organs whose vasculature is being damaged. Doctors who diagnose patients with this disorder must also identify the specific type of ANCA vasculitis in order to treat the patient effectively.
Renal limited vasculitis
In this type of ANCA vasculitis, the capillaries in the glomeruli, which are the filtering units in the kidneys, swell. This impedes kidney function, potentially causing kidney failure. This type of ANCA vasculitis is also known as ANCA glomerulonephritis and is characterized by blood and protein in the urine.
Microscopic polyangiitis (MPA)
In MPA patients, vascular swelling affects multiple tissues and organs including kidneys, skin, nerves, lungs and joints. Patients usually experience a range of symptoms such as kidney inflammation, skin lesions and nerve damage depending on which areas are affected. Weight loss and fevers frequently occur as well.
Granulomatosis with polyangiitis (GPA)
As with MPA, patients with GPA can experience swelling in various tissues, typically in the lungs, kidneys, sinuses, nose, eyes and ears. However, the swelling is specifically caused by granulomas that form in the vasculature. Granulomas are masses of immune cells that form at the site of infections. This type of ANCA vasculitis was formerly known as Wegener’s granulomatosis.
Eosinophilic granulomatosis with polyangiitis (EGPA)
EGPA is typically limited to the lungs and respiratory tract. It is caused by granulomas primarily made up of a single type of white blood cell called eosinophils. Patients may experience asthma-like symptoms for many years before other symptoms of vasculitis appear. This type of ANCA vasculitis is also known as Churg-Strauss syndrome.
How is ANCA vasculitis diagnosed and treated?
Physicians use a series of tests to identify vasculitis and classify a patient’s specific type of ANCA vasculitis. Blood, urine and tissue samples are collected to test for ANCA concentrations in different parts of the body. Imaging may also be recommended to specifically look at the lungs or head and neck regions.
Methods of treatment vary but may include corticosteroids, immunosuppressive drugs or antibiotics. These treatments reduce, and in some cases cure, the vascular swelling that triggers uncomfortable symptoms for patients.
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