What Is Vasculitis? What Causes Vasculitis?

Vasculitis, which may also be referred to as angiitis and arteritis refers to a large group of diseases involving inflammation of the blood vessels, specifically the walls of the blood vessels - including arteries, veins and capillaries (tiny veins). The plural of vasculitis is vasculitides.

Vasculitis causes alterations in the walls of blood vessels, which may include:

• Scarring
• Weakening
• Narrowing
• Thickening

Vasculitis can be acute (short-term) or chronic (long-term). In some cases organs in the body may be affected, especially if they do not receive enough nutrient and oxygen-rich blood, resulting in organ damage, and sometimes death.

According to Medilexicon's medical dictionary:

Vasculitis or angiitis is Inflammation of a blood vessel (arteritis, phlebitis) or lymphatic vessel (lymphangitis).

Examples of vasculitis include:

• Behcet's Disease - a refractory (not yielding readily to treatment) systemic (affecting the entire body) inflammatory disease characterized by repeated episodes of relapses and recoveries, such as oral ulcers on the mucous membrane, dermatitis, uveoretinitis, and ulcers on the vulva. It is sometimes associated with inflammatory symptoms in the intestines, nerves, and blood vessels.
• Buerger's Disease - a disease of the arteries and veins in the arms and legs. The blood vessels swell and become blocked with thrombi (blood clots), eventually damaging or destroying skin tissues. Sometimes this may lead to infection and gangrene. Buerger's disease risk is linked to regular smoking.
• Central Nervous System Vasculitis - a type of vasculitis that also involves the brain and spinal cord.
• Churg-Strauss Syndrome - inflammation of small arteries and veins in people with a history of allergy or asthma.
• Cryoglobulinemia - linked to hepatitis C virus infections. Blood becomes abnormally thick, and there is inflammation of blood vessels.
• Giant Cell Arteritis - inflammation of the walls of the arteries.
• Henoch-Schönlein Purpura - inflammation of capillaries (tiny blood vessels) in the skin and frequently the kidneys, resulting in skin rash, especially over the buttocks and behind the lower extremities. It is associated with arthritis and sometimes cramping pain in the abdomen.
• Kawasaki disease - a rare syndrome of unknown origin that causes high fever, reddening of the eyes (conjunctivitis), lips and mucous membrane of the mouth, gingivitis (ulcerative gum disease), swollen neck glands and a bright red rash over the skin of the hands and feet, in young children. Kawasaki disease causes inflammation in the walls of arteries throughout the body, including the coronary arteries, which supply blood to the heart muscle.
• Rheumatoid Vasculitis - a complication of severe rheumatoid arthritis (RA), an autoimmune disease that causes painful inflammation of the joints. Approximately 2%-5% of RA patients develop RV, an extraarticular (occurring outside the joint) manifestation of rheumatoid arthritis, affecting small and medium-size arteries in the body. RV can involve many body organs including the skin, eyes, heart, lungs, nerves to the hands and feet, as well as blood vessels in the fingers and toes.
• Takayasu's Arteritis - a rare type of vasculitis. Inflammation damages the aorta (large artery that carries blood from the heart to the rest of the body), as well as the aorta's main branches. The patient experiences arm or chest pain, hypertension (high blood pressure, and eventually stroke or heart failure.
• Wegener's Granulomatosis - a type of inflammation and injury to blood vessels that affects several organs, including the lungs, kidneys and upper respiratory tract. It is a life-threatening disorder that requires long-term immunosuppression. Some patients die due to toxicity of treatment.

What are the signs and symptoms of vasculitis?

A symptom is something the patient senses and describes, while a sign is something other people, such as the doctor notice. For example, drowsiness may be a symptom while dilated pupils may be a sign.

Signs and symptoms of vasculitis vary, and depend on which blood vessels are affected, and if organs are damaged, which ones. Most cases of vasculitis include the following symptoms:

• Fever
• Tiredness
• Weight loss, loss of appetite
• Joint pain
• Muscle pain
• Numbness, and possibly weakness

Signs and symptoms of some vasculitis conditions:

Behcet's Disease - symptoms vary, they may disappear and return. Possible signs and symptoms include:

• Mouth - painful sores, like canker sores. They start as raised, round lesions in the mouth and soon turn into painful ulcers.
• Skin - some may develop acne-like sores, while others may have red, raised and tender nodules (especially on the lower limbs)
• Genitals - most commonly on the scrotum in males and the vulva in females. They appear as red, round, ulcerated lesions.
• Eyes - inflammation of the eye (uveitis). Eyes become red and sometimes painful. There may be blurred vision in either one or both eyes. Inflammation in the retina blood vessels can lead to serious complications.
• Joints - typically swelling and pain in the knee; sometimes the ankle, elbow or wrist as well.
• Blood vessels - inflammation of the veins and large arteries cause painful and swollen limbs.
• Gastrointestinal - abdominal pain, diarrhea. Possible bleeding in the digestive system.
• Brain - inflammation in the brain and nervous system can result in headaches, fever, poor balance, and disorientation.

Buerger's Disease - initial symptoms often include pain in the feet and/or hands during exercise, caused by insufficient blood flow; sometimes the pain may also be present at rest. Usually the pain starts in the extremities, but may radiate to other parts of the body. The patient may also experience:

• Numbness in the limbs
• Tingling in the limbs
• Fingers, toes, hands and feet turn white when exposed to the cold
• Skin ulcerations
• Gangrene of fingers and toes are also possible
• The affected areas may be extremely painful

Central Nervous System Vasculitis - signs and symptoms may include:

• Mental Changes
• Headache
• General Confusion
• Paralysis and/or Muscle Weakness
• Visual Problems
• Dysphasia, Coma
• Seizure
• Depressed Level of Consciousness
• Myelopathy (disorder of the spinal cord)

Churg-Strauss Syndrome - asthma is the main feature, which may begin a long time before the onset of vasculitis. Other early signs and symptoms may include allergic rhinitis and nasal polyps. In the next phase the patient typically has eosinophilia - excessive number of eosinophils in blood and/or tissues. An eosinophil is a white blood cell subtype. The third phase is a vasculitis, usually involving the skin, lungs, nerves, kidneys and other organs. There is frequent devastation of the nerves (mononeuritis multiplex) which causes severe tingling, numbness, severe muscle wasting in hands and feet, and shooting pains.

Cryoglobulinemia - symptoms vary according to the type and which organs are affected. Typically, signs and symptoms include:

• Breathing problems
• Fatigue
• Gomerulonephritis - a problem in the kidneys
• Joint pain
• Muscle pain
• Purpura - purple spots and patches on the skin, organs and mucous membranes
• Fingers, toes, hands and feet turn white when exposed to the cold
• Skin ulceration

Giant Cell Arteritis - the most common symptoms are:

• Headache
• Shoulder pain
• Pain the hips
• Pain in the jaw after chewing
• Fever
• Blurred vision
Other symptoms may include


• Scalp tenderness
• Cough
• Throat pain
• Tongue pain
• Weight loss
• Depression
• Pain in the arms during exercise

Henoch-Schönlein Purpura - the most common symptoms are:

• A skin rash that looks like small bruises or small red spots on the buttocks, elbows and legs.
• Joint pain, especially the knees and ankles
• Stomach pain
• Nausea
• Vomiting
• Diarrhea
• Blood in stool (feces) if the vessels in the bowel and kidneys are inflamed

Kawasaki disease

Takayasu's Arteritis - approximately half of all patients develop an initial systemic (all over the body) illness with symptoms of:

• Malaise
• Fever
• Night sweats
• Weight loss
• Arthralgia
• Fatigue

Wegener's Granulomatosis - Wegener's granulomatosis signs and symptoms can develop either gradually or suddenly. Initial symptoms can vary widely, and diagnosis may sometimes be delayed because of their non-specific nature. The first symptom in most patients is rhinitis - runny and stuffy nose. Signs and symptoms may include:

• Persistent runny nose
• Cough - sometimes this may include blood
• Ear infections
• Joint aches
• Loss of appetite
• Malaise - a general feeling of being unwell
• Nosebleeds
• Shortness of breath
• Sinusitis
• Skin sores
• Swelling of joints - often initially diagnosed as rheumatoid arthritis; but is usually arthritis
• Eye pain
• Burning sensation in the eyes
• Eye redness
• Vision problems
• Fever
• Weakness, which is often due to anemia
• Rapidly progressive glomerulonephritis - a syndrome of the kidney that, if left untreated, rapidly progresses into acute renal (kidney) failure.

What are the causes of vasculitis?

When the body attacks its own blood vessels as if they were undesirable foreign pathogens, such as harmful bacteria or viruses, vasculitis occurs. In other words, vasculitis occurs when our body's immune system mistakenly attacks our blood vessels. Experts are not sure why this happens. We do know there are some triggers, including:

• Infections
• Some cancers
• Some immune system disorders
• An allergic reaction

Typically, when blood vessels are affected by vasculitis they become inflamed; the blood vessel walls thicken, resulting in a narrowing of the blood vessel and less bloodflow. Less bloodflow means less oxygen and nutrients getting to organs and body tissues.

The affected blood vessel is more susceptible to blood clots, partly because it is narrower. If the blood vessels weaken an aneurysm (a bulge) may form.

There are two main categories of vasculitis:

• Primary vasculitis - this is vasculitis with no known cause.
• Secondary vasculitis - the vasculitis occurred because of another disease, such as an infection, an immune system disorder, an allergic reaction, or a blood cancer.
  
  
  • Infection - Hepatitis C virus infection can cause cryoglobulinemia.
  • Immune system disorder - rheumatoid arthritis or lupus can cause vasculitis to occur.
  • Allergic reaction - an allergic reaction to some medications can cause vasculitis to occur.
  • Some cancers - leukemia or lymphoma can cause vasculitis to occur.

Diagnosing vasculitis

The GP (general practitioner, primary care physician) or specialist doctor will ask the patient about their symptoms, medical history and carry out a physical examination. The following diagnostic tests may be ordered too:

Blood tests - these may include:

• An Erythrocyte Sedimentation Rate (ESR) test - a sample of red blood cells is placed into a test tube of liquid. The amount of time the red blood cells take to fall to the bottom is measured at a rate of millimeters per hour. If they fall faster than normal it could mean the child has an inflammatory condition. However, it does not tell us what is causing the inflammation.
• A C Reactive Protein (CRP) test - this test measures how much C reactive protein there is in the blood. CRP is produced by the liver. A higher-than-usual blood level of CRP means there is an inflammation in the body. However, this test, like the ESR one, does not tell us what is causing the inflammation.
• Platelet count - platelets are cells in the blood that clump together when there is a lesion to stop bleeding; they form part of our blood's clotting (coagulation) system.
• A complete blood cell count, and anti-neutrophil cytoplasmic antibodies test may also be ordered.

Urine tests - these may reveal the amount of protein in the urine, or if there are any red blood cells. Urine tests can determine whether there is a kidney problem.

Imaging scans - these may be used to have a look at the larger arteries and their branches. X-rays, ultrasounds, CT scans or MRI scans may be used.

Angiogram - this is an X-ray of the blood vessels. A long, thin, flexible tube (catheter) is inserted into a large vein or artery. A dye is injected into the blood vessels through the catheter. This dye shows up in X-ray images, allowing the doctor to see the outlines of the blood vessels.

Biopsy - the doctor surgically removes a small sample of the affected blood vessel. This is then examined for signs of vasculitis.

What are the treatment options for vasculitis?

Treatment options for vasculitis depend of several factors, including what type of vasculitis the patient has, the severity of symptoms, their age and general health. Sometimes, as may be the case with Henoch-Schonlein purpura, the condition resolves without medical therapy.

Most commonly used medications for vasculitis:

Steroids - used to reduce inflammation. Examples include prednisone or methylprednisolone (Medrol). Side effects may be severe if taken over the long-term.

Side effects may include:

• Weight gain
• Diabetes
• Osteoporosis (thinning of bones)

Patients with vasculitis will usually receive very low doses.

Immune system medications - if the patient does not respond well to steroid therapy, the doctor may prescribe drugs that stop the immune system cells that cause inflammation - cytotoxic drugs. Examples include azathioprine (Imuran) and cyclophosphamide (Cytoxan).

What are the possible complications of vasculitis?

Complications can vary, depending on the type of vasculitis. The following are the most common:

• Organ damage - poor oxygen and nutritional (blood) supply to organs can result in their damage.
• Recurrence - even if treatment is successful, there may be recurrent episodes. Some patients require long-term treatment.