Vasculitis

Vasculitis is commonly an inflammation of the blood vessels. It happens when the body's immune system attacks the blood vessel by mistake and causes the blood vessels walls to become thicker and weaker. It is a rare disease. Vasculitis can affect arteries, veins and capillaries, in this way it may restrict blood flow and cause damage to some tissues and organs as well. While the condition remains for a short term in some cases, it can be a long lasting disease too and in few cases it doesn't even require specific treatment.

There are 3 types of vasculitis based on the size of vessel inflamed, which varies in symptoms, severity and duration:

  • Large vessel vasculitis - Takayasu arteritis, giant cell arteritis

  • Medium vessel vasculitis - Kawasaki disease, PAN (Polyarteritis Nodosa)

  • Small vessel vasculitis - Wegners (GPA), churgstrauss, HSP etc.

Vasculitis affects persons of both sexes and all ages. Vasculitis can be mild or severe, or even leads to death. A few forms of vasculitis affect certain age groups only. For example; Kawasaki disease occurs only in children, IgA Vasculitis (Henoch-Schönlein) is much more common in children than adults, giant cell arteritis occurs only in adults over 50 years old.

Vasculitis leads to poor blood flow to organs, such as the lungs, nerves, kidney and skin. Thus, vasculitis has a wide range of signs and symptoms which include:

  • • Shortness of breath and cough, blood in sputum

  • • Tingling,numbness or weakness in a hand or foot

  • • Red spots on the skin (purpura), lumps (nodules) or ulcers

  • • Fever, headache, fatigue, weight loss, general aches and pains, night sweats and rash are the other symptoms of vasculitis.

On the other hand, vasculitis of the kidneys may produce no symptoms at first but is still a serious problem.

What causes vasculitis?

Most types of vasculitis are rare, and the exact causes are generally unknown. Some cases of vasculitis are related to the genetic makeups, some are due to autoimmune phenomenon.

Some other causes of vasculitis are:

  • • Reactions to some medicines.

  • • Long-term infections (hepatitis C or hepatitis B virus).

  • • Rheumatic diseases like lupus, rheumatoid arthritis and Sjögren’s syndrome

Diagnosis of vasculitis

Clinical examination that starts by analysing your medical history proceed to the support of lab test and screening:

  • Blood Tests - For a complete blood cell count to check the sign of inflammation.

  • Urine Test - To determine the presence of red blood cells and protein level in your urine.

  • Biopsy - Surgical removal of a small piece of tissue from the affected areas of your body for inspection under a microscope.

  • Angiography - A type of CT/MRI or X-rays of your blood vessels to look for abnormalities of blood vessels.

To find small-vessel vasculitis, we most often go for a biopsy of the skin or a kidney. Detection of medium-vessel vasculitis is done by either biopsy (of skin, nerve or brain) or angiography. Angiography is also the test that is often used to finds large-vessel vasculitis.

Treatment of Vasculitis:

Vasculitis is inflammation of the blood vessels thus the treatment is to control the inflammation and prevent immune system from causing damage to blood cells affecting tissues. Treatment of vasculitis includes:

Steroids : These are main drugs for treating most forms of vasculitis to reduce inflammation. The dose and length of treatment depend on how severe the disease is and how long the patient has had it.

Immune-Suppressive Drug : - Rheumatologist use these drugs to decrease the dose of steroids to minimise the side effects. This is called “steroid-sparing” treatment.

  • • For less serious vasculitis; we use methotrexate, azathioprine, Mycophenolate, tacrolimus or other immune-suppressing drugs.

  • • For severe disease endangering vital organs; we use Cyclophosphamide or Rituximab infusion.

  • • Some patients with the most severe diseases often requires receive plasma exchange (Plasmapheresis) or Intravenous immunoglobulin (IVIg).

Surgery : Damage from severe vasculitis sometimes require surgery like 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.

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