Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitis is an autoimmune disease which can cause blood vessels to swell and form vasculitis. This disease is rare, affects about one in 50,000 people and is more common in middle-aged, Caucasian men and women.
Causes
ANCAs (the autoantibodies) attach to neutrophils (a type of white blood cell) which makes the neutrophils attack small blood vessels in the body. Thus, vasculitis (inflammation in blood vessels) has formed which makes blood vessels become swollen and inflamed. In fact, most people do not have these ANCAs.
Symptoms & Types
ANCA vasculitis can cause different symptoms depending on its types.
Renal limited vasculitis
Renal limited vasculitis affects blood vessels only in the kidneys. It can interfere with normal kidney functions. It is characterized by blood and protein in the urine and can lead to permanent kidney failure.
Microscopic polyangiitis (MPA)
MPA is associated with inflamed vasculature in many body’s organ systems, including but not limited to the kidneys, peripheral nervous system, skin, and lungs.
The most common symptoms of MPA include:
Other symptoms depend on where blood vessel inflammation is located. For example, when it occurs in respiratory capillaries, blood will flow into alveoli (the air sacs make up the lung) which causes patients to cough up blood.
Granulomatosis with polyangiitis (GPA)
Similar to MPA, swelling of blood vessels can appear in nearly any part of the body. The most commonly parts are lungs, kidneys, and respiratory tract (sinuses, nose, trachea, or ears).
If it occurs on uncommon parts, such as skin and joints, symptoms may be confused with other inflammatory diseases easily.
Symptoms in the lungs usually include heavy coughing and occasionally coughing up blood, while in the kidneys mainly include brown and bloody urine which are easy to identify.
Respiratory tract symptoms may include:
Eosinophilic granulomatosis with polyangiitis (EGPA)
EGPA is mainly limited to the lungs and respiratory tract. Symptoms mainly include:
EGPA patients also may share some GPA symptoms associated with damage to the lungs, kidneys, and skin.
Diagnosis
Doctors use a combination of tests and historical symptoms and physical exam to diagnose ANCA vasculitis. These tests usually focus on the part(s) of the body that are affected.
Following are some tests that are often used to diagnose vasculitis:
Treatment
Treatments of ANCA vasculitis are complicated. Because of different affected part, patients need specialists major in different part give combined therapies.
Treatments and medicines usually depend on following things:
According to some studies, treatment process of ANCA vasculitis mainly divided into two parts:
Some of medicines may be also helpful:
Besides that, plasmapheresis, also known as plasma exchange or PLEX can be used. It is a procedure that can remove antibodies from the bloodstream – including the ANCA autoantibodies.
Diets
According to the condition of disease or treatments, doctors may advise patients changing their diets, such as:
Please remember to consult your doctors for your symptoms and specific treatments.
Keywords: anti-neutrophil cytoplasmic antibodies vasculitis; ANCA vasculitis; ANCA; anti-neutrophil cytoplasmic antibodies; autoimmune disease; vasculitis.