1. Definition Vasculitis is an inflammation of blood vessels, which can also be called vasculitis, and can lead to lesions of the vessel wall, including thickening, swelling, narrowing, and sclerosis of the vessel wall. Inflammation can manifest itself in the short term (acute) or long term (chronic), and severe inflammation can lead to ischemia of tissues and organs, resulting in tissue and organ damage and even death. There are many types of vasculitis, and certain age groups are more susceptible to specific types of vasculitis. Some vasculitis can heal on its own, while the rest require treatment, usually with long-term medication. The clinical manifestations of vasculitis depend on the affected blood vessels and organs, but most vasculitis has signs and symptoms that include fever, fatigue, weight loss, muscle and joint pain, loss of appetite, and neurological abnormalities such as numbness and weakness. Specific clinical manifestations exist for different types of vasculitis. Typical signs and symptoms include genital ulcers and inflammation of the eyes. Buerger’s disease: Also known as thrombo-occlusive vasculitis, it can lead to inflammation of the blood vessels in the limbs and thrombosis. The onset of the disease is closely related to smoking. Mound-Schönig’s syndrome: Also known as allergic granulomatous vasculitis, it usually involves the pulmonary vasculature and is closely associated with asthma. Cryoglobulinemia: usually associated with hepatitis C virus infection, typical signs and symptoms include a hemorrhagic rash (purpura) of the lower extremities, arthritis, malaise, and nerve damage. Macrocytic arteritis: usually develops in people over 50 years of age and is an inflammation of the blood vessels in the neck, upper body, upper extremities and head, especially the temporal region. It can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. The onset is usually associated with rheumatic polymyalgia. Hen-Schönlein purpura (allergic purpura): usually causes inflammation of blood vessels in the skin, joints, intestines, and kidneys. Typical signs and symptoms include abdominal pain, blood in the urine or stool, joint pain, and a hemorrhagic rash (purpura) on the buttocks, legs, and feet. Allergic purpura is usually more common in children, but can develop at any age. Allergic vasculitis: The most prominent symptom is red patches of skin. It can be triggered by an allergic reaction, usually due to a drug allergy or an infection. Kawasaki disease: Also known as mucocutaneous lymph node syndrome, it has a high incidence in children under 5 years of age. Typical signs and symptoms include fever, rash, and ophthalmia. Microscopic small vessel vasculitis: This type of vasculitis involves small vessels in the kidneys, lungs, and skin. Typical signs and symptoms include: skin ulcers, fever, weight loss, glomerulonephritis – inflammation of small vessels in the kidneys, and nerve damage. Polyarteritis nodosa: This type of vasculitis involves multiple small and medium-sized blood vessels in the body, including the skin, heart, kidneys, peripheral nerves, muscles, and intestines. Typical signs and symptoms include purpura, skin ulcers, muscle and joint pain, abdominal pain, and kidney damage. Rheumatic polymyalgia: This type of vasculitis is usually seen in the elderly and causes pain and inflammation in large joints, such as the shoulder, knee, and hip joints. Typical signs and symptoms include pain and stiffness in the hip, thigh, shoulder, upper arm, and neck. It is usually closely associated with giant cell arteritis. Rheumatic vasculitis: This type of vasculitis can usually be complicated by rheumatoid arthritis, usually with a high incidence in patients with severe rheumatoid arthritis, and can involve multiple parts of the body, such as the eyes, skin, hands, and feet. Takayasu(s) arteritis: This type of vasculitis usually involves the large arteries of the body, such as the aorta, and often develops in young women. Typical signs and symptoms include back pain, arm weakness, intermittent claudication, diminished or absent pulse, dizziness, headache, and visual disturbances. Wegener’s granulomatosis: This vasculitis often leads to inflammation of blood vessels in the nose and sinuses, as well as the throat, lungs, and kidneys. Typical signs and symptoms include: shortness of breath, nasal congestion, chronic sinusitis, rhinorrhea, and frequent ear infections. 3. Etiology The vascular system is composed of an intricate network of blood vessels, including arteries, veins and capillaries. The arterial blood vessels carry oxygen-rich arterial blood to all parts of the body, while the venous vessels carry carbon dioxide and metabolic waste products back to the heart. The capillaries are the smallest vessels, connecting the arterioles and venules, and also perform the function of fluid and nutrient exchange with the tissues. In the case of vasculitis, the blood vessels are attacked by inflammation, resulting in thickening of the vessel walls and narrowing of the lumen, which reduces the blood flow to the tissues of all parts of the body, with a corresponding decrease in oxygen and vital nutrients. In some cases, thrombus may also form in the involved starved vessels, completely blocking blood flow. Sometimes the involved blood vessels do not show narrowing, but instead may dilate locally into tumors due to the weakness of the vessel walls, in which case there is a potentially fatal risk. 4. Primary or secondary vasculitis In many cases, the cause of vasculitis is not known, and these vasculitis of unknown etiology are called primary vasculitis. In some types of vasculitis, infection may be the cause. In these cases, it has been determined that the vasculitis is caused by a pre-existing condition called secondary vasculitis. For example, most cryoglobulinemia is due to hepatitis C virus infection. Hepatitis B virus infection can lead to polyarteritis nodosa. Vasculitis can also occur secondary to certain immune diseases such as rheumatoid arthritis and lupus erythematosus. Drug allergies can also lead to vasculitis in some cases, such as antibiotic or diuretic allergies. 5. When to seek medical attention If you think you have vasculitis, you need to seek medical attention. Some types of vasculitis involving vital organs are very serious and can lead to death if not treated promptly. If you have been diagnosed with vasculitis, remember that the signs and symptoms of a recurrence are usually similar to those of the first episode of the disease. Also, be alert for new signs and symptoms that indicate a possible recurrence of the disease or a complication that occurred during treatment, such as an infection. The signs and symptoms of vasculitis are similar to those of many other diseases, making a definitive diagnosis more difficult. Therefore, your doctor may try to rule out other diseases that may be causing your current symptoms. Your doctor will ask about your symptoms and past history, and do a complete physical examination. You may undergo some of the following tests. Hematology: If your doctor suspects vasculitis, he or she will give you a hematocrit test, also known as the erythrocyte sedimentation rate, which measures the rate at which red blood cells settle in a test tube. You may also be tested for C-reactive protein, a protein synthesized by the liver during inflammation. Your doctor may also test your blood cells for anemia and platelets. Platelets are colorless cells that help stop bleeding when the body is injured. Some types of vasculitis can cause an abnormal increase or decrease in these cells. The doctor may also check for a significantly higher white blood cell count, which may indicate an infection or inflammation. In addition, the doctor may check the blood for anti-neutrophil cytoplasmic antibodies, as well as other antibodies such as rheumatoid factor and antinuclear antibodies. Anti-neutrophil cytoplasmic antibodies may indicate Wegener’s granulomatosis or microscopic vasculitis, while increased rheumatoid factor and anti-nuclear antibodies may indicate rheumatoid arthritis or connective tissue disease. Imaging: The doctor may decide to perform non-invasive imaging tests such as ultrasound, CT or MRI to rule out involvement of large arteries such as the aorta and its branches. In some cases, an invasive angiogram may also be required. During an angiogram, a catheter is inserted into an artery or vein, and a special dye (contrast) is injected through the catheter into the vessel, and the filling of the artery or vein is photographed. Biopsy: Although hematologic and imaging tests can provide some useful information, a biopsy of an involved vessel is still the best means of confirming a diagnosis of vasculitis. This test is usually performed on an outpatient basis under local anesthesia and usually involves only minor discomfort and minimal scarring. The specimen obtained from the biopsy is examined microscopically in the laboratory. If scarring is visible microscopically, the disease may be chronic or long term. Urinalysis: Urinalysis may reveal abnormalities such as increased urine red blood cells or protein. If the kidneys are involved, the prognosis is poor. Many vasculitides have mild symptoms that resolve spontaneously, and in some cases, such as Hen-Schein purpura (allergic purpura), medication can effectively control the condition. However, in some cases of vasculitis that are severe and involve major organs, damage to the organs may have occurred prior to drug treatment, or the disease may be so severe that treatment is ineffective and may lead to failure of major organs or death. Even after the vasculitis has been controlled by treatment, it may recur and require further treatment. Some types of vasculitis such as giant cell arteritis, Wegener’s granulomatosis, and Takayasu’s arteritis are prone to recurrence after the first treatment is in remission, and some vasculitis is difficult to cure and requires long-term continuous treatment. 8. Medication The treatment measures for vasculitis depend on the type of vasculitis, the severity of the condition, and the general condition of your body. Your doctor may ask about your past history of drug allergies, nutritional intake, and whether you get sun exposure, all of which may cause an allergic rash in some people. Although some types of vasculitis are self-limiting and can heal on their own, such as Hen-Schein’s purpura (allergic purpura), the remaining types require treatment with one or more of the following medications Corticosteroids: Many types of vasculitis require the use of corticosteroids, such as prednisone or methylprednisolone, for treatment. After using corticosteroids for a period of time, you may feel significantly better, but you will need to continue the medication for a longer period of time. After the first month of corticosteroid use, your doctor may begin to taper the dosage to the lowest maintenance level of control, and some signs and symptoms will improve with treatment. Your doctor may use medications to counteract the side effects of long-term corticosteroid use, such as risedronate, to treat corticosteroid-induced osteoporosis, which can also be used to prevent the onset of osteoporosis. Cytotoxic drugs: Some vasculitis is severe and corticosteroid therapy is not effective, then cytotoxic drugs are needed, such as azathioprine and cyclophosphamide, which inhibit inflammation of the blood vessels. Nonsteroidal anti-inflammatory drugs: such as aspirin and ibuprofen can be effective in treating those vasculitides with milder symptoms, such as rheumatic polymyalgia and Kawasaki disease. However, for many patients, NSAIDs are difficult to achieve a complete cure, and long-term application of NSAIDs may cause bleeding in the gastrointestinal tract. While you are being treated, your doctor may order regular hematology tests to test the effectiveness of your treatment and your response to treatment. Some other drugs that researchers are studying for the treatment of vasculitis include primidone, tumor necrosis factor blockers, rituximab, and others. Response and support If vasculitis can be diagnosed and treated early, the prognosis is good. The biggest challenge you face will be in coping with the side effects that occur during treatment with rational drugs. The following suggestions may help you: Know your condition. Learn as much as you can about vasculitis and its treatment, understand some of the possible side effects of the medications you are taking, and inform your doctor of any changes in your body. Eat a healthy diet. A healthy diet can help prevent potential problems associated with medications such as osteoporosis, high blood pressure and diabetes. Fresh fruits and vegetables, cereals, fat-free fish, and a low-smoking, low-sugar diet with limited alcohol intake should be emphasized. If you are finding it difficult to get enough calcium in your daily diet, it is because you are not consuming enough dairy products, such as vitamin D. Proper calcium supplementation is effective, inexpensive and well tolerated and absorbed. Sometimes calcium supplementation may lead to constipation. If this is a problem, increase the amount of water you drink and increase your fiber supplementation. Also, check the type of calcium supplement you are using. Calcium phosphate and calcium citrate are less likely to cause constipation than other types of calcium supplements. Calcium-rich foods include milk, low-fat yogurt, cheese, kale, canned bone-in salmon, orange juice, and some other foods such as calcium-rich tofu. Regular exercise. Regular aerobic exercise, such as walking, can prevent bone loss, high blood pressure and diabetes. It’s also good for your heart and lungs. In addition, many people find that exercise can improve their mood and make them feel good about themselves. If you are not in the habit of exercising regularly, start and gradually build up. Your doctor will help you develop an exercise program that works for you.