Vasculities

Presentation by Tolulope Sivebukola.

VASCULITIES

What is vasculities?• Vasculitis is an inflammation of the blood vessels. • It causes changes in the walls of blood vessels, including thickening,

weakening, narrowing and scarring. • These changes restrict blood flow, resulting in organ and tissue

damage.• Vasculitis is also known as angiitis and arteritis.

Signs and Syptoms• General signs and symptoms of vasculitis include:• Fever• Headache• Fatigue• Weight loss• General aches and pains• Night sweats• Rash• Nerve problems, such as numbness or weakness• Loss of a pulse in a limb

Cause(s)• The exact cause of vasculitis isn't fully understood. Some types are related to a person's

genetic makeup. Others result from the immune system attacking blood vessel cells by mistake. Possible triggers for this immune system reaction include:

• Infections, such as hepatitis B and hepatitis C• Blood cancers• Immune system diseases, such as rheumatoid arthritis, lupus and scleroderma• Reactions to certain drugs• Blood vessels affected by vasculitis may bleed or become inflamed. Inflammation can

cause the layers of the blood vessel wall to thicken. This narrows the blood vessels, reducing the amount of blood — and therefore oxygen and vital nutrients — that reaches your body's tissues and organs.

Types Different types of vasculitis will usually affect mainly one size of blood vessel. • Takayasu's arteritis and Giant cell or Temporal arteritis are called large vessel

vasculitis because they mainly affect the aorta and the biggest arteries as they branch off. • Polyarteritis nodosa and Kawasaki disease are called medium vessel vasculitis

because they affect the middle sized arteries as they go into the organs.• Most of the other types of vasculitis, such as Wegener's granulomatosis (GPA),

and Microscopic polyangiitis etc, are called small vessel vasculitis because they cause inflammation in the smallest blood vessels, the capillaries, as they supply the insides of the organs.

Takayasu• Takayasu arteritis is a rare, systemic, inflammatory large-vessel

vasculitis of unknown etiology that most commonly affects women of childbearing age.

• Takayasu arteritis can be divided into the following six types based on angiographic involvement (see Workup) [2] :• Type I - Branches of the aortic arch• Type IIa - Ascending aorta, aortic arch, and its branches• Type IIb - Type IIa region plus thoracic descending aorta• Type III - Thoracic descending aorta, abdominal aorta, renal arteries,

or a combination• Type IV - Abdominal aorta, renal arteries, or both• Type V - Entire aorta and its branches

Giant cell • Giant cell arteritis (GCA), or temporal arteritis, is a systemic

inflammatory vasculitis of unknown etiology that occurs in older persons and can result in a wide variety of systemic, neurologic, and ophthalmologic complications. GCA is the most common form of systemic vasculitis in adults. • GCA typically affects the superficial temporal arteries—hence the term

temporal arteritis. In addition, GCA most commonly affects the ophthalmic, occipital, vertebral, posterior ciliary, and proximal vertebral arteries. It has also been shown to involve medium- and large-sized vessels, including the aorta and the carotid, subclavian, and iliac arteries.

Polyarteritis Nodosa• Classic polyarteritis nodosa (PAN or c-PAN) is a systemic vasculitis

characterized by necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcations, resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction.• PAN, like other vasculitides, affects multiple systems and has protean

manifestations, although it most commonly affects skin, joints, peripheral nerves, the gut, and the kidney.The lungs are usually spared with PAN. A typical PAN patient might present with fever, night sweats, weight loss, skin ulcerations or tender nodules, and severe muscle and joint pains developing over weeks or months.

Kawasaki

Diagnosis• Blood Tests

• Blood tests can show whether you have abnormal levels of certain blood cells and antibodies (proteins) in your blood. These tests may look at:

• Hemoglobin and hematocrit. A low hemoglobin or hematocrit level suggests anemia, a complication of vasculitis. Vasculitis can interfere with the body's ability to make enough red blood cells. Vasculitis also can be linked to increased destruction of red blood cells.

• Antineutrophil cytoplasmic antibodies (ANCA). These antibodies are present in people who have certain types of vasculitis.

• Erythrocyte sedimentation rate (ESR). A high ESR may be a sign of inflammation in the body.

• The amount of C-reactive protein (CRP) in your blood. A high CRP level suggests inflammation.

• Biopsy

• A biopsy often is the best way for your doctor to make a firm diagnosis of vasculitis. During a biopsy, your doctor will take a small sample of your body tissue to study under a microscope. He or she will take the tissue sample from a blood vessel or an organ.

• A pathologist will study the sample for signs of inflammation or tissue damage. A pathologist is a doctor who specializes in identifying diseases by studying cells and tissues under a microscope.

• Urinalysis

• For this test, you'll provide a urine sample for analysis. This test detects abnormal levels of protein or blood cells in the urine. Abnormal levels of these substances can be a sign of vasculitis affecting the kidneys.

• EKG (Electrocardiogram)

• An EKG is a simple, painless test that records the heart's electrical activity. You might have this test to show whether vasculitis is affecting your heart.

• Echocardiography

• Echocardiography is a painless test that uses sound waves to create a moving picture of your heart. The test gives information about the size and shape of your heart and how well your heart chambers and valves are working.

• Chest X Ray

• A chest x ray is a painless test that creates pictures of the structures inside your chest, such as your heart, lungs, and blood vessels. Abnormal chest x-ray results may show whether vasculitis is affecting your lungs or your large arteries (such as the aorta or the pulmonary arteries).

• Computed Tomography Scan

• A computed tomography (to-MOG-rah-fee) scan, or CT scan, is a type of x ray that creates more detailed pictures of your internal organs than a standard x ray. The results from this test can show whether you have a type of vasculitis that affects your abdominal organs or blood vessels.

• Magnetic Resonance Imaging

• A magnetic resonance imaging (MRI) test uses radio waves, magnets, and a computer to create detailed pictures of your internal organs.

• Angiography

• Angiography is a test that uses dye and special x rays to show blood flowing through your blood vessels.

• The dye is injected into your bloodstream. Special x-ray pictures are taken while the dye flows through your blood vessels. The dye helps highlight the vessels on the x-ray pictures.

• Doctors use angiography to help find out whether blood vessels are narrowed, swollen, deformed, or blocked.

Treatment• Common prescription medicines used to treat vasculitis include

corticosteroids and cytotoxic medicines.

• Corticosteroids help reduce inflammation in your blood vessels. Examples of corticosteroids are prednisone, prednisolone, and methylprednisolone.

• Prescribe cytotoxic medicines if vasculitis is severe or if corticosteroids don't work well. Cytotoxic medicines kill the cells that are causing the inflammation. Examples of these medicines are azathioprine, methotrexate, and cyclophosphamide.

• Other treatments may be used for certain types of vasculitis. For example, the standard treatment for Kawasaki disease is high-dose aspirin and immune globulin. Immune globulin is a medicine that’s injected into a vein.

• Certain types of vasculitis may require surgery to remove aneurysms that have formed as a result of the condition.

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