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Vasculitis (Symptoms, Causes, Diagnosis, Treatment, Types)

  • Medical Author: William C. Shiel Jr., MD, FACP, FACR
  • Medical Editor: Melissa Conrad Stöppler, MD

Vasculitis definition and facts

  • Vasculitis is a group of uncommon diseases, which result in inflammation of the blood vessels.
  • Vasculitis symptoms and signs vary greatly and depend upon the organs affected and the severity of the disease, and may include:
  • Researchers don't know the cause of vasculitis; however, common features of the condition include inflammation of blood vessels and immune system abnormalities. Examples of diseases and conditions in which vasculitis occurs include:
  • Vasculitis also may occur with infections, drugs (cocaine, amphetamines), cancers, lupus, and rheumatoid arthritis.
  • Vasculitis is diagnosed with blood tests, biopsies, and angiogram.
  • Treatment for vasculitis is directed toward decreasing the inflammation of the blood vessels and improving the function of affected organs.

What is vasculitis?

Vasculitis is a general term for a group of uncommon diseases that feature inflammation of the blood vessels. The blood vessels of the body are referred to as the vascular system. The blood vessels are comprised of arteries that pass oxygen-rich blood to the tissues of the body and veins that return oxygen-depleted blood from the tissues to the lungs for oxygen. Vasculitis is characterized by inflammation in and damage to the walls of various blood vessels.

Each of the vasculitis diseases is defined by certain patterns of distribution of blood vessel involvement, particular organ involvement, and laboratory test abnormalities. As a group, these diseases are referred to as vasculitides.

The word vasculitis is derived from the Latin "vasculum", vessel + "- itis," inflammation. Another term for vasculitis is angiitis. When arteries are the inflamed blood vessels, the condition is also referred to as arteritis. When the veins are inflamed, it is referred to as venulitis.

What causes vasculitis, and what are examples of diseases with vasculitis?

The actual cause of these vasculitis diseases is usually not known. However, immune system abnormality and inflammation of blood vessels are common features. Each form of vasculitis has its own characteristic pattern of symptoms, much of which depends on what particular organs are affected.

Examples of vasculitis include:

  • Kawasaki disease,
  • Behçet's disease,
  • Polyarteritis nodosa,
  • Granulomatosis with polyangiitis,,
  • Cryoglobulinemia,
  • Takayasu's arteritis,
  • Churg-Strauss syndrome,
  • Giant cell arteritis (temporal arteritis), and
  • Henoch-Schönlein purpura.

Vasculitis can also accompany:

What are the symptoms of vasculitis?

The symptoms of vasculitis vary greatly from person to person, and depend upon the organs affected and the severity.

  • Generally, vasculitis causes fatigue and malaise.
  • Sometimes vasculitis can lead to weakness and weight loss.
  • Vasculitis affecting the skin can cause rashes, skin discoloration, and ulcers.
  • Vasculitis affecting the muscles can cause muscle pain.
  • Vasculitis affecting the lungs can cause shortness of breath and cough.
  • Vasculitis affecting the heart can cause congestive heart failure.
  • Vasculitis affecting the brain can cause headaches, confusion, seizures, stroke, paralysis, numbness, and lightheadedness.
  • Vasculitis affecting the kidneys can cause kidney failure.

What tests diagnose vasculitis?

Laboratory testing of blood or body fluids in a patient with active vasculitis generally indicates inflammation in the body. Depending on the degree of organ involvement, a variety of organ function tests can be abnormal.

The diagnosis of vasculitis is definitively established after a biopsy of involved tissue demonstrates the pattern of blood vessel inflammation. Examples of tissues used for biopsy include skin, sinuses, lung, nerve, and kidney. Depending upon the situation, an alternative to biopsy can be an X-ray test of the blood vessels called an angiogram, which can demonstrate characteristic patterns of inflammation in affected blood vessels.

What is the treatment for vasculitis?

The treatment of the various forms of vasculitis is based on the severity of the illness and the organs involved. Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, cortisone-related medications, such as prednisone , are used. Additionally, other immune suppression drugs, such as cyclophosphamide (Cytoxan) and others are considered. Additionally, affected organs (such as the heart or lungs) may require specific medical treatment when the disease is active.

The management of vasculitis is an evolving field in medicine. The ideal programs for monitoring and treatment will continue to improve as disease patterns and causes are defined by medical research.

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Reviewed on 4/6/2018

REFERENCES:

Luca, NJC, MD. Vasculitis and Thrombophlebitis. Medscape. Updated: Oct 26, 2017.
<https://emedicine.medscape.com/article/1008239-overview>

Kelley and Firestein's Textbook of Rheumatology 10th edition. 2016.

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