What is vasculitis?

Causes and Symptoms

There is no definite cause of vasculitis. Some types may be autoimmune in nature, in which the body attacks its own tissues. Some types of vasculitis may be caused by an allergic reaction to a medication, exposure to a toxic chemical, or a virus, such as the hepatitis B and hepatitis C viruses. Vasculitis may occur with connective tissue
disease such as rheumatoid arthritis, Sjögren’s syndrome, and lupus, or with a blood cell cancer such as leukemia or lymphoma.

These conditions are often classified by the size of the blood vessels involved in the condition or by the type of cells involved in the inflammation of the blood vessels. Large vessel vasculitis includes Takayasu arteritis, Behçet’s syndrome, polymyalgia rheumatica, and giant cell arteritis. Medium vessel vasculitis includes Buerger’s disease, polyarteritis nodosa, Kawasaki disease, cutaneous vasculitis, and primary central nervous system vasculitis. Small vessel vasculitis includes Wegener’s granulomatosis, Churg-Strauss arteritis, microscopic polyarteritis/angiitis, hyperallergic vasculitis, Henoch-Schonlein purpura, essential cryoglobinemic vasculitis, hypersensitivity vasculitis, and vasculitis secondary to connective tissue disorders. The types of cells that may be observed in the various vasculitis types are neutrophils, lymphocytes, leukocytes, eosinophils, or granulomatous cells. The two classification systems tend to overlap, and the cell type can change in a type of vasculitis as the condition progresses.

The first symptoms that are reported are usually systemic. They include fatigue, fever, night sweats, weakness, weight loss, anorexia, muscle and joint pain, and numbness. More specific symptoms depend on the type of vasculitis. Some of the types demonstrate only skin
lesions, which can be purple spots (purpura), areas of necrosis, or skin ulcers. They include hypersensitivity vasculitis, Buerger’s disease, and cutaneous vasculitis. Each type of vasculitis then has its own symptoms based on the part of the body that is affected. Symptoms can include a skin rash, joint or extremity pain, neuropathy, ulcerations of the skin, headache, visual problems, abdominal pain, vomiting, diarrhea, anemia, coughing up blood, muscle pain, conjunctivitis, weakness, heart failure, palpitations, sinus problems, bleeding into the lungs, and abnormal kidney function. Some types of vasculitis are self-limiting, but most are chronic conditions. The damage that vasculitis can cause can be life-threatening.

Vasculitis is diagnosed by blood tests, including a complete blood count (CBC), general chemistry, liver function tests, and kidney function tests. These tests demonstrate what body organs are affected by the vasculitis. Some tests that are commonly abnormal with vasculitis are erythrocyte
sedimentation rate (ESR), C-reactive protein (CRP), antinuclear antibody (ANA), and antineutrophil cytoplasmic antibody (ANCA). These tests indicate the presence of inflammation in the body, which is a sign of vasculitis. The most specific test for vasculitis is a biopsy of an affected area of the body, such as the skin or a kidney. The biopsy actually demonstrates the presence of vasculitis. Sometimes, angiograms (X-rays of the blood vessels) are performed to look for vasculitis. If kidney problems are suspected, then urine is tested for the presence of microscopic blood and protein.

Treatment and Therapy

The treatment for vasculitis is based on the type of disease. The most common treatment is the administration of corticosteroid drugs, such as prednisone or solumedrol. They can be given orally or intravenously. Corticosteroids
are used to control the inflammation in the blood vessels. Often, other immune system suppressant drugs are administered with the corticosteroids. They include cyclophosphamide, azathioprine, and methotrexate. These medications can also be administered orally or intravenously. Both of these groups of medications have a number of side effects. Corticosteroids can cause weight gain, diabetes, osteoporosis, insomnia, hypertension, increased risk of infection, mood changes, stomach upset, and cataracts. The immune system suppressant drugs are cytotoxic substances that are also used to treat some types of cancer and to prevent the rejection of transplanted organs. They, too, have many side effects. They can cause hair loss, fatigue, bladder cancer, hemorrhagic cystitis, increased risk of infections, nausea, vomiting, diarrhea, and liver damage.

Usually, vasculitis responds well to one or both of these medications, particularly if it is diagnosed early. If vasculitis does not respond well to this treatment, plasmapheresis can be performed or the medication interferon alpha can be given. Plasmapheresis is a procedure in which plasma is removed from blood previously taken from the body. Removal is done by centrifuging the blood, which is separated into plasma with other immune cells and the red blood cells. The red blood cells are then returned to the patient. Eliminating many of the white blood cells can interfere with the immune response. Interferon alpha is a biologic drug that can affect the immune response. This drug is still being studied for its role in treating vasculitis.

Prospective and Prospects

Although symptoms of conditions that sound like vasculitis have appeared in ancient medical writings, vasculitis was not identified until 1866 by Adolf Kussmaul. Kussmaul noted that the affected patient had nodules under his skin, but on autopsy, he was able to see that there were nodules on the patient’s arteries. He called this condition periarteritis nodosa. Kussmaul attributed the arterial nodules to inflammation of the blood vessels, and he considered it to be a novel condition that had not been described previously.

After Kussmaul’s description of periarteritis nodosa, other cases of apparent vasculitis were compared to it. However, many of these conditions were actually different types of vasculitis. There are approximately twenty different types of vasculitis. Giant cell arteritis was described in 1890. Giant cell arteritis is inflammation of the large temporal arteries of the head. It causes localized pain and tenderness, and it can lead to blindness if untreated. It is diagnosed by biopsy of a temporal artery. Polymyalgia rheumatica was first described in 1957, and it occurs in roughly 50 percent of those who develop giant cell arteritis. Polymyalgia rheumatica affects the large arteries of the shoulders and hips and causes muscle pain in the arms and legs.

Takayasu’s arteritis, first described in 1908, is inflammation of the aorta and its major branches, the optical arteries. This vasculitis affects young women and can lead to heart failure. Buerger’s disease was also described in 1908. It is characterized by severe lack of blood flow to the hands and feet, causing severe pain, blue fingers and toes, and tissue death. Buerger’s disease is caused by cigarette smoking. Kawasaki disease was described in 1939. It demonstrates inflammation of the medium-sized arteries of the mucous membranes, lymph nodes, and coronary arteries. This condition occurs only in young children and causes swollen glands in the neck, conjunctivitis, inflammation around the mouth and on the palms of the hands and the bottom of the feet, a skin rash, and aneurysms of the coronary arteries.

Three of the more serious forms of vasculitis are Wegener’s granulomatosis, Churg-Strauss syndrome, and microscopic polyarteritis. These conditions can be rapidly fatal unless treated aggressively. All three are associated with the presence of antineutrophil cytoplasmic antibodies in the blood. Wegener’s granulomatosis was first described in 1936, and it affects the small blood vessels of the skin, lungs, eyes, sinuses, and kidneys. Churg-Strauss syndrome was described in 1951. It begins with the development of asthma and then progresses to affect the nerves, skin, heart, lungs, gastrointestinal tract, and kidneys. Microscopic polyarteritis was first described in 1948. It affects the smallest blood vessels of the lungs and kidneys. All three conditions can lead to bleeding by the small blood vessels in the lungs and lead to kidney failure.

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