What is vasculitis?

  • Vasculitis is the inflammation of the body’s blood vessels .
  • Vasculitis can affect very small blood vessels (capillaries), mediumsizeblood vessels, or large blood vessels such as the aorta (themain blood vessel that leaves the heart).
  • These vessels include arteries and veins.
  • Vasculitis can result in poor blood flow to tissues throughout the body, such as the lungs, nerves and skin.
  • Thus, vasculitis has a wide range of signs and symptoms (what you see and feel), such as:
    • Shortness of breath and cough
    • Numbness or weakness in a hand or foot
    • Red spots on the skin (“purpura”), lumps (“nodules”) or sores (“ulcers”)
    • When inflamed, the blood vessels may become weakened and stretch in size, which can lead to The vessels also may become so thin that they rupture resulting in bleeding into the tissue.
  • On the other hand, vasculitis of the kidneys may produce no symptoms at first but is still a serious problem due to renal failure.
  • Vasculitis can be mild or disabling, or even lead to death. Patients can have one episode of vasculitis or have repeated episodes over several years.
  • Most types of vasculitis are rare.

What causes vasculitis?

  • In most cases, the exact cause is unknown; however, it is clear that the immune system (the system that keeps the body healthy) plays a big role.
  • While the immune system usually works to protect the body, it can sometimes become “overactive” and end up attacking parts of the body.
  • In most cases of vasculitis, something causes an immune or “allergic” reaction in the blood vessel walls.
  • Substances that cause allergic reactions are called antigens.
  • Sometimes certain medicines or illnesses can act as antigens and start this process.
  • Genetic factors (different genes) appear be somewhat important in the disease.
  • Some cases of vasculitis are caused by reactions to medicines.
  • Also, some chronic (long-term) infections, including with hepatitis C or hepatitis B virus, can cause vasculitis.
  • Vasculitis can be a part of other rheumatic diseases, mainly systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome

What are the symptoms of vasculitis?

Vasculitis affects persons of both sexes and all ages.Common symptoms include:

  • Skin rashes
    • If a blood vessel in the skin with vasculitis is small, the vessel may break and produce tiny areas of bleeding in the tissue.These areas will appear as small red or purple dots on the skin.
    • If a larger vessel in the skin is inflamed, it may swell and produce a nodule (lump or mass of tissue), which may be felt if the blood vessel is close to the skin surface.
  • Fatigue
  • Weakness
  • Fever
  • Joint pains
  • Abdominal pain
  • Kidney problems (including dark or bloody urine
  • Nerve problems (including numbness, weakness and pain)
  • Cough and/or shortness of breath
  • Additional symptoms can occur, depending on the area of the body affected by vasculitis.

How is vasculitis diagnosed?

  • The diagnosis of vasculitis is based on a person’s medical history, current symptoms, complete physical examination and results of specialized laboratory tests like ANA         ( antinuclear antibodies), ANCA (antineutrophil cytoplasmic antibodies),complements, RF, viral markers
  • A doctor can test for blood abnormalities, which can occur when vasculitis is present. These abnormalities include:
    • Anemia (low red blood cell count)
    • A high white blood cell count
    • A high platelet count
    • Signs of kidney or liver problems
  • Blood tests also can identify immune complexes or antibodies (ways the body fights off what it thinks is a threat) that can be associated with vasculitis.
  • Additional tests may include Xrays,tissuebiopsies , blood vessel and heart scans.

How is vasculitis treated?

  • The precise treatment of vasculitis depends on the specific type of vasculitis and the areas/organs that are involved.
  • Some measures that may be necessary include the use of corticosteroids
  • For more serious types of vasculitis, other medications that suppress the immune system are also used like cyclophosphamide, azathioprine and methotrexate  These medicines have their own side effects and these treatments must be watched very closely.
  • Newer drugs like rituximab effectively treats severe cases of certain forms of vasculitis. These include granulomatosis with polyangiitis, microscopic polyangiitis and cryoglobulinemicvasculitis.
  • Some patients with the most severe cases of these diseases may receive plasma exchange (“plasmapheresis”) or intravenous immunoglobulin (often called “IVIg”).
  • Surgery: Damage from severe vasculitis sometimes requires surgery. This may involve vascular bypass grafting (a surgery to redirect blood flow around a blockage in a blood vessel). Depending on where the damage is, other possible operations are sinus surgery or a kidney transplant.

Living with Vasculitis

  • Vasculitis can be short term or lifelong.
  • Doctors often focus, with good reason, on preventing permanent damage to vital organs (such as the lungs, kidneys and brain) and the nerves.
  • It is crucial, of course, to prevent death and long-term disability from vasculitis.
  • Yet, other issues often trouble patients. These include fatigue (feeling very tired), pain, arthritis, nose and sinus problems.
  • Side effects from medications, especially glucocorticoids, also can be troubling.
  • Patients taking immunosuppressants are at an increased risk of infections. Follow your doctor’s advice on how to reduce your infection risk.
  • Fortunately, with current treatments, the outcome for patients with vasculitis is often good