Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases in which the immune system erroneously attacks and damages small blood vessels.
This misled immune attack is caused by self-reactive antibodies, known as ANCAs, which bind to and activate white blood cells called neutrophils.
Depending on the organ or tissue where blood vessel damage occurs, AAV can cause many different symptoms. These include problems in the lungs, kidneys, nervous system, and skin.
When inflammation affects the kidneys, it usually means that blood vessels responsible for filtering water, electrolytes, and waste products have been damaged. As a result, patients experience poor kidney function that can progress to kidney failure, in which case a transplant or frequent dialysis is needed.
Symptoms of kidney problems include the presence of blood in the urine, which makes it appear red or brownish, a foamy urine due to the presence of proteins, and high blood pressure. Blood levels of a metabolic waste product called creatine may also be increased.
Kidney failure often leads to other symptoms such as fatigue, vomiting, and metallic taste.
Patients may have inflammation of blood vessels in the lungs, and particularly damage in the capillaries surrounding alveoli (small air sacs in the lungs where gases are exchanged).
Consequently, they experience several respiratory problems, including shortness of breath, coughing up blood, cough with mucus, and chest pain that can worsen when taking a deep breath.
Inflammation in the trachea may also lead to a hoarse voice and cough.
Neurological symptoms involving the nerves outside the brain and spinal cord are common in people with ANCA vasculitis, affecting up to 70% of patients. These symptoms include numbness, tingling, burning sensation, and weakness in different parts of the body.
Symptoms of the central nervous system (the brain and spinal cord), such as headaches, cognitive impairment, and memory deficits, may also appear but they are rare. Seizures, paralysis, and loss of consciousness may also be experienced by patients with AAV.
Eye, ear, nose, and throat symptoms
People living with AAV may experience sinusitis, which is pain, pressure, or congestion in the sinus (small air spaces behind the forehead, nose, cheekbones, and in between the eyes) caused by inflammation.
There may be a greenish, brown, or bloody discharge from the nose that may form a crust, which causes patients to have trouble breathing through the nose.
Inflammation may occur in the eyes, which become red and irritated, usually causing pain. Vision may be blurred and progressively lost. In rare cases, patients can also exhibit bulging or protruding eyeballs, as a result of inflammation in the back of the eye.
A ringing or other noise, a condition dubbed tinnitus, may be experienced. Patients may also have ear pain and infection (otitis) that are not solved within normal healing time, and they may experience hearing loss.
Before other symptoms of vasculitis appear, some patients may have asthma-like symptoms, nasal polyps (painless soft growths inside the nose), and allergic rhinitis (also called hay fever).
Inflammation in the skin associated with AAV may be confused easily with other inflammatory diseases. Patients may develop purplish skin lesions due to bleeding into the tissues under the skin, a rash with hives, and small bumps, particularly on the elbows.
Skin lesions, such as sores or ulcers, may also occur.
Joint and muscle symptoms
Inflammation in muscles and joints may be confused with other inflammatory conditions. These symptoms usually develop later and include a shooting pain in the muscles (myalgia) and joints (arthralgia), as well as intense muscle loss in the hands and/or feet.
Patients with AAV can develop a number of digestive symptoms, including blood in their stools, diarrhea, nausea, vomiting, and abdominal pain. They may also have high levels of liver enzymes in the blood, indicating liver damage.
Although AAV symptoms are based on where blood vessel inflammation is located, patients may experience other symptoms, generally called “constitutional” complaints. These include weight loss, lack or loss of appetite, fever, night sweats, flu-like symptoms, and a feeling of general weakness or discomfort (a condition known as malaise).
Last updated: May 13, 2021
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