Vasculitis

"Vasculitis" is a general term for a group of diseases that involve inflammation in blood vessels.  Blood vessels of all sizes may be affected, from the largest vessel in the body (the aorta) to the smallest blood vessels in the skin.  The size of blood vessel affected varies according to the specific type of vasculitis. 

The causes of most vasculitides are currently unknown.  It is clear, however, that the immune system plays a critical role in the tissue damage caused by vasculitis.  The immune system, normally a protective organ of the body, becomes "hyperactive" in vasculitis because of some unknown stimulus, leading to inflammation within the body’s tissues.  Inflammation in blood vessel walls leads to narrowing of the vessels.  The resulting inadequate blood supply to a particular tissue or organ results in damage.

Symptoms include:

• A variety of rashes, the most classic of which is "palpable purpura" – purplish-red spots, usually found on the legs.  These spots can usually be felt by the examiner's fingertips, hence the description "palpable".
• Symptoms ranging from full-blown arthritis to aches in the joints without obvious swelling (arthralgias).
• Cough (particularly coughing up blood), shortness of breath, a pneumonia-like appearance to a patient's chest X-ray lung "infiltrates", and the development of cavities in the lungs.
• Red blood cells (usually invisible to the naked eye), clumps of red blood cells (known as "casts", also invisible to the naked eye), and loss of protein in the urine.   May lead to renal insufficiency, requiring dialysis.
• Abdominal pain, bloody diarrhea, perforation of the intestines.
• Anemia (low hematocrit or red blood cell count) and/or a slightly elevated white blood cell count.
• Chronic sinus congestion and "infections" that persist for longer than they should; inflammation of the nasal septum, sometimes resulting in a perforation or collapse of the bridge of the nose; hearing loss.
• May affect either blood vessels to the eyes, causing the sudden loss of vision, or small blood vessels within the eyes, leading to retinal problems, thinning of the sclera (the white part of the eyes), inflammation within the eye’s different chambers, and conjunctivitis ("pinkeye").
• Headaches, strokes, changes in mental status, difficulty with coordination.
• Shooting pains in the arms and legs, numbness, and asymmetrical weakness (i.e., weakness that involves one side of the body more than the other).

Because vasculitis is caused in part by an overly active immune system, the treatment usually involves the use of medications that suppress parts of the immune system.

Several things can happen to an inflamed blood vessel. If it is a small vessel, it 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 is inflamed, it may swell and produce a nodule which may be felt if the blood vessel is close to the skin surface. The inside of the vessel tube may become narrowed so that blood flow is reduced, or the inside may become totally closed (usually by a blood clot which forms at the site of inflammation). If blood flow is reduced or stopped, the tissues which receive blood from that vessel begin to die. For example, a person with vasculitis of a mediumsized artery in the hand may develop a cold finger which hurts whenever it is used; occasionally this can progress to gangrene.

What Causes Vasculitis?

Vasculitis can be caused by (1) infection of the blood vessel walls, or (2) an immune or "allergic" reaction in the vessel walls.

The first cause is rare. When it occurs, bacteria, viruses or fungi infect the blood vessel. White blood cells move in to destroy the infectious agents and damage the blood vessel in the process. This is a serious condition and requires prompt antibiotic treatment.

The second cause of vasculitis, an immune reaction, is more common. Substances which cause allergic reactions are called "antigens." They cause the body to make proteins called "antibodies" which bind to the antigen for the purpose of getting rid of it. Antigen and antibody bound together are called "immune complexes." Two primary ways in which immune complexes destroy antigens are: (1) by attracting white blood cells to digest the antigen, and (2) by activating other body substances to help destroy the antigens.

Unfortunately, some immune complexes do not serve their purpose of destroying antigens. Instead, they remain too long in the body and circulate in the blood and deposit in tissues. They commonly accumulate in blood vessel walls, where they cause inflammation.

It is likely that some white blood cells which kill infectious agents ("cytotoxic" cells) can also accidentally damage blood vessels and cause vasculitis.

In the vasculitis caused by lupus, the antigens causing the immune complexes are often not known. In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens. A recently discovered antibody, ANCA (anti-neutrophil cytoplasm antibody), can cause vasculitis in some individuals.

Diseases Associated with Vasculitis Vasculitis can occur in many different illnesses. Some of the illnesses that can cause vasculitis are:

• Infections
• Autoimmune Diseases

  - Lupus

  - Rheumatoid Arthritis

  - Polymyalgia Rheumatica

  - Scleroderma

  - Wegener's Granulomatosis

  - Temporal Arteritis

  - Cryoglobulinemia

• Erythema Nodosum
• Tumors

  - Leukemia

  - Lymphoma

  - Others

Symptoms of Vasculitis

• Systemic symptoms: Fever, generally feeling bad ("malaise"), muscle and joint pain, poor appetite, weight loss, and fatigue. This set of complaints can occur in many illnesses and is not specific to vasculitis.

   

• Skin: Red or purple dots ("petechiae"), usually most numerous on the legs. When the spots are larger, about the size of the end of a finger, they are called "purpura." Some look like large bruises. These are the most common vasculitis skin lesions, but hives, itchy lumpy rash, and painful or tender lumps can occur. Areas of dead skin can appear as ulcers (especially around the ankles), small black spots at the ends of the fingers or around the fingernails and toes ("nail fold infarcts"), or gangrene of fingers or toes.

   

• Joints: Aching in joints and a frank arthritis with pain, swelling and heat in joints. Deformities resulting from this arthritis are rare.

   

• Brain: Vasculitis in the brain can cause many problems, from mild to severe. They include headaches, behavioral disturbances, confusion, seizures, and strokes.

   

• Peripheral Nerves: Peripheral nerve symptoms may include numbness and tingling (usually in an arm or a leg, or in areas which would be covered by gloves or socks), loss of sensation, or loss of strength (especially in the feet or hands).

   

• Intestines: Inadequate blood flow in the intestines can cause crampy abdominal pain and bloating. If areas in the wall of the intestine develop gangrene, blood will appear in the stool. If the intestinal wall develops a hole (called a "perforation"), surgery may be required.

   

• Heart: Vasculitis in the coronary arteries is unusual in lupus. If it occurs, it can cause a feeling of heaviness in the chest during exertion ("angina"), which is relieved by rest. Heart attacks rarely occur as a result of vasculitis.

   

• Lungs: Vasculitis in this tissue can cause pneumonia-like attacks with chest x-ray changes that look like pneumonia, and symptoms of fever and cough. Occasionally, inflammation can lead to scarring of lung tissue with chronic shortness of breath.

   

• Kidneys: Vasculitis is not common in kidneys of people with lupus, even those who have lupus nephritis. It may not cause any symptoms, although most patients with renal vasculitis have high blood pressure.

   

• Eyes: Vasculitis involving the small blood vessels of the retina can occur in lupus. The retina is a tissue at the back of the eye which contains cells that have to be activated to form a visual image. Sometimes, vasculitis of the eyes causes no symptoms. Usually, however, there is visual blurring which comes on suddenly and stays, or a person may even lose a portion of their vision. In other non-lupus types of vasculitis, such as temporal arteritis, there is sudden loss of part or all of the vision in one eye, usually accompanied by severe headache.

Consulting Your Physician

Doctors trained in several different specialties are taught to recognize and treat vasculitis. These include rheumatologists, general internists, dermatologists, hematologists, nephrologists, gastroenterologists, infectious disease experts, pulmonologists, cardiologists, geriatricians, neurologists, and ophthalmologists.

Diagnosing Vasculitis

Blood abnormalities which often occur when vasculitis is present include an elevated sedimentation rate, anemia, a high white blood count and a high platelet count. Blood tests can also be used to identify immune complexes or antibodies that cause vasculitis in the circulation and measure whether complement levels are abnormal. These tests take several days to complete. The physician may also order a urine analysis.

If there are any symptoms that suggest heart involvement, tests that may be ordered include: EKG, ECHO cardiogram and heart scans. For lung symptoms, the physician may order a chest x-ray, obtain blood from an artery to measure the oxygen content, and schedule a pulmonary function test. A pulmonary function test uses a specialized machine to measure how well the lungs handle air and oxygen as you breathe into it. If there are abdominal symptoms, the physician may order ultrasound or CAT scans of the organs in the abdomen, or other special x-rays to see the intestines. For brain symptoms, CAT scans and magnetic resonance images are frequently useful.

Sometimes, inflammation in medium and large-size arteries or veins can be seen by injecting dye into them and viewing the outlines of the blood vessels on x-ray. This procedure is called an "angiography." It can be done in any area of the body.

The diagnosis is most firmly made by seeing vasculitis in involved tissue. This is done by taking a biopsy of the involved tissue and examining that tissue under a microscope. Your physician may suggest this procedure.

Finally, it may be important for your physician to consult with other medical specialists about your case. For example, if your physician is a rheumatologist and you have visual complaints which could be indicative of vasculitis, you may be referred to an ophthalmologist. It is very important that one physician be in charge of your case, coordinating your care and helping you with decisions.

Treating Vasculitis

Many cases of vasculitis do not require treatment. For example, a few spots on the skin now and then (if not combined with other symptoms) may not require any medications.

Most physicians recommend cortisone-type medications, such as Prednisone, Prednisolone, or methylprednisolone (Medrol) as the initial treatment for vasculitis.

Some people with severe vasculitis or vasculitis that does not respond well to cortisone-type drugs will need to be treated with cytotoxic drugs. These medications kill the cells that cause inflammation in the blood vessels. The two most frequently used are azathioprine (Imuran) and cyclophosphamide (Cytoxan). They are usually used in combination with Prednisone and are often effective in treating vasculitis.

Experimental procedures that have been helpful in treating some cases of vasculitis include: plasmapheresis, intravenous gamma globulin, and cyclosporin, a medication used to prevent organ rejection in transplant patients. Experimental therapies change frequently. Your physician can provide you with current information.

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