ANCA, or anti-neutrophil cytoplasmic autoantibody vasculitis, is a group of autoimmune diseases that are characterized by vascular inflammation.There are four types of ANCA vasculitis. In all four types, autoantibodies target white blood cells and trigger them to attach to and destroy the surrounding blood vessels. The symptoms vary based on the location(s) of vascular damage in the body.

Renal limited vasculitis

Renal limited vasculitis refers to ANCA vasculitis that affects blood vessels only in the kidneys. This disorder interferes with regular kidney function. 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 a number of the body’s organ systems, including but not limited to the kidneys, peripheral nervous system, skin, and lungs. The most common symptoms of MPA are kidney inflammation and weight loss (affecting about 80 percent of patients), and skin lesions, nerve damage, and fevers (seen in about 60 percent of patients).

Patients will have various symptoms based on where blood vessel inflammation is located, but weight loss, fevers, fatigue, and malaise are common complaints, generally called “constitutional” complaints, across all MPA patients. When respiratory capillaries are damaged to the point of causing bleeding into the air sacs that make up the lungs, called alveoli, patients also will cough up blood.

The manifestation of MPA in the eyes, muscles, and joints can cause other illnesses related to inflammation in those areas.

Granulomatosis with polyangiitis (GPA)

Like MPA, patients with GPA can experience swelling of blood vessels in nearly any part of the body, with the most common areas being the lungs, kidneys, and respiratory tract (sinuses, nose, trachea, or ears). GPA patients also may experience similar constitutional symptoms to MPA patients. Inflammation in less common parts of the body, such as the skin and joints, can be confused easily with other inflammatory diseases.

Indications of damage to the lungs generally involve heavy coughing and occasionally coughing up blood. Symptoms of kidney damage, primarily brown and bloody urine, also are identified easily.

Respiratory tract symptoms may include some or all of the following:

  • Ear infections that do not resolve within normal healing time, or reduced hearing abilities.
  • Inflammation in various parts of the eye, sometimes resembling a recurring pink eye infection, blurry vision, and pain in the eye region.
  • Worsening runny nose, nose pain, and trouble breathing through the nose. Nosebleeds and nasal crusting may be more common.
  • Shortness of breath.

Eosinophilic granulomatosis with polyangiitis (EGPA)

EGPA is typically limited to the lungs and respiratory tract. People with undiagnosed EGPA may experience asthma-like symptoms, and possibly nasal polyps and allergic rhinitis (also known as hay fever) for many years before other symptoms of vasculitis appear. Characteristics that develop later on include tingling, numbness, shooting pains, and extreme muscle degradation in the hands and/or feet. EGPA patients also may share some GPA symptoms associated with damage to the lungs, kidneys, and skin.

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