Vasculitis refers to inflammation of the blood vessels and can affect any blood vessel or organ in the body. The immune system mistakenly attacks the blood vessels, resulting in inflammation. Of the 15 types, most are rare with generally unknown causes. Symptoms range from poor blood flow, to lung damage that causes difficulty breathing and nerve damage resulting in numbness or weakness in a hand or foot. The disease can be quite mild or very serious. The diseases are: Behcet’s disease. Buerger’s disease, Central Nervous System, Churg Strauss syndrome, Cryoglobulinemia, Giant Cell Arteritis, Henoch- Schönlein purpura, Hypersensitivity Vasculitis, Kawasaki disease, Microscopic Polyangiitis, Polyarteritis nodosa, Polymyalgia rheumatica, Rheumatoid Vasculitis, Takayasu’s arteritis and Wegener’s Granulomatosis.
The range of symptoms varies according to the specific blood vessels and organs involved.
- Behcet’s syndrome causes eye inflammation, mouth and genital ulcers and skin lesions that resemble acne.
- Buerger’s disease affects the extremities, causing pain in the hands, arms, feet and legs and ulcers on the fingers and toes.
- Churg-Strauss syndrome often results in asthma.
- Cryoglobulinemia causes a rash on the lower extremities, arthritis, weakness and neuropathy (nerve damage).
- Giant cell arteritis is an inflammation of the arties of the head, which can result in headache, scalp tenderness, jaw pain, vision problems and blindness.
- Henoch-Schönlein purpura affects the vessels of the skin, joints, bowel and kidneys. Symptoms include abdominal pain, blood in the urine, joint pain and a rash on the buttocks, legs and feet. It usually affects children.
- Hypersensitivity Vasculitis is characterized by red spots on the skin.
- Kawasaki disease commonly affects young children, causing fever, skin rash and eye inflammation.
- Microscopic polyangiitis affects small blood vessels of the kidneys, lungs and skin. Signs of the disease include skin lesions, fever, weight loss, inflammation of the small blood vessels in the kidneys and nerve damage.
- Polyarteritis nodosa is an inflammation of various mid-sized vessels. Symptoms include rash, skin ulcers, pain and kidney problems.
- Takayasu’s arteritis involves large arteries including the aorta with a feeling of numbness in the extremities, decreased or absent pulses, high blood pressure, headaches and vision problem.
- Wegener’s granulomatosis involves the blood vessels of the nose, sinuses, throat, lungs and kidneys. It causes nasal stuffiness, chronic sinus infections and nosebleeds and, often, kidney problems.
Risk factors vary by the specific vasculitis disease. Some are linked to reactions to medications. And some chronic infections, especially with hepatitis C or hepatitis B, are risk factors for the disease. Vasculitis can also be associated with other rheumatic diseases including lupus, rheumatoid arthritis and Sjögren’s syndrome. Vasculitis is not hereditary and most people who get it have no underlying cause.
Vasculitis cannot be prevented. If your doctor determines that a particular medication caused the outbreak, avoiding that drug can help prevent further involvement.
Your WellStar rheumatologist will review symptoms and the results of a physical exam and/or lab tests. The doctor will look for possible damage to one or more organ systems with no other likely causes. This can indicate vasculitis.
Tools and Tests
Diagnosis methods depend on the size of the vessel involved. A biopsy is typically recommended to detect small-vessel vasculitis (involving the skin or kidney). Vasculitis involving medium vessels (skin, nerve or brain) is detected by biopsy or angiography – use of an x-ray with dye to look for abnormal blood vessels. Large-vessel vasculitis is also detected by angiography.
Blood tests can diagnose some types of vasculitis such as Behcet’s syndrome and Kawasaki’s disease. A positive blood test for anti-neutrophil cytoplasmic antibodies (ANCA) can lead to a diagnosis of Wegener’s granulomatosis, microscopic polyangiitis or Churg-Strauss syndrome. Blood tests can also detect organ damage.
Although some types of vasculitis tend to improve on their own, therapy usually involves drugs. Your physician will talk with you about these and their possible role in your treatment plan:
- Glucocorticoids (like prednisone) are a type of steroid used to control inflammation in many types of vasculitis. Dose and duration of the treatment depends on the individual condition.
- Immunosuppressive agents may be an option when steroids are not enough, or because of their long-term side effects. Examples include methotrexate and azathioprine. These work to prohibit activity of the immune system.
- Plasma exchange and intravenous immunoglobulin are used in some severe cases.
- Surgery (sinus, vascular bypass or kidney transplant) may be an option in cases of severe vasculitis.
- A primary treatment focus is on preventing permanent damage to organs including the lungs, kidneys, brain and nerves. Your doctor will also work with you to relieve the pain, arthritis and fatigue associated with vasculitis.
Early identification and treatment often contribute to a good prognosis. Patients should work in partnership with their doctors and take steps including the following:
- Become informed. Learn all you can about your disease including the possible side effects of medications you are taking.
- Let your doctor know if you experience any changes in your health or have other concerns.
- Eat well. A diet rich in fruits and vegetables, whole gains, low-fat diary products and lean protein can help prevent possible side effects of medications. These can include high blood pressure, diabetes and thinning bones.
- Discuss the need for vitamins with your doctor depending on the types of medicines you are taking.
- Exercise regularly. Aerobic exercise can help prevent bone loss, high blood pressure and diabetes associated with taking steroids. Exercise also helps improve mood and an overall sense of wellbeing.
- Talk with your WellStar rheumatologist about the need to see other specialists (like a dermatologist, neurologist or ophthalmologist) depending on the nature of your vasculitis.