Vasculitis is a rare condition, characterized by inflammation of the blood vessels. It’s the umbrella term for a group of approximately twenty diseases–the “vasculitides” –which differ depending on which veins or arteries are affected, and in which areas of the body. Some forms of the disease are mild, and resolve on their own without medical therapy, others go into remission with treatment. More serious forms may involve critical organs and can be life-threatening.
Vasculitis is considered an autoimmune disease, in which the body’s immune responses go out of control, attacking its own healthy tissues–in this case, the lining of blood vessels. In many cases, it’s unknown what causes this aggressive immune response and inflammation. In some instances, the disease may be triggered by an infection or an allergic reaction to medication, but can also be a complication of another autoimmune disease, such as rheumatoid arthritis or lupus.
Vasculitis can affect small, medium or large blood vessels, such as listed below:
Giant cell arteritis (also known as temporal arteritis)
Granulomotosis with polyangiitis
The inflammation of blood vessels may cause a number of problems, depending on the type of vasculitis. The veins or arteries can narrow, sometimes reducing or even halting the normal flow of blood. As a result, dysfunction of nearby tissues, formation of blood clots or even irreversible necrosis (tissue death) may occur. In some cases, the inflammation causes widening, stretching and weakening of a major artery wall, causing, in extreme cases, a “ballooning” artery deformity (an aneurysm) which, if ruptured can be life threatening. The symptoms of vasculitis are many and various, since so many body systems can be affected. Patients can experience headaches, muscle or joint pain, fever, cough, shortness of breath, general weakness, high blood pressure, skin rash or ulcerating lesions, inflammation of the lung tissue and/or lining of the chest, blindness or kidney failure.
Approximately 100,000 Americans per year are hospitalized for treatment of vasculitis. The majority of people are diagnosed in middle age or older, though the disease also occurs in younger people and infants. A few forms of vasculitis are specific to certain groups of people; Kawasaki disease occurs predominantly in children. IgA Vasculitis (Henoch-Schönlein) is also more common in youngsters. Giant cell arteritis, on the other hand, affects adults who are over 50 years of age. Vasculitis may affect only a single organ system (i.e the kidneys), or it may affect multiple areas of the body at the same time, which is known as “systemic” vasculitis.
Because vasculitides are rare and present with so many varying symptoms, they may take years to be diagnosed. Physicians suspect vasculitis when they see a cluster of abnormal symptoms, “telling” findings on physical examination, abnormal test results, and diagnostic imaging studies (i.e. vascular MRI). It initially takes a thorough medical history, physical examination, and often a biopsy, to detect vasculitis. An angiography—an X-ray that looks for abnormalities in the blood vessels—can also help physicians arrive at a diagnosis. Certain blood tests are used to find out whether a patient has “antineutrophil cytoplasmic antibodies,” or ANCA, which are present in some forms of the disease.
Rheumatologists are the specialists with the most overall knowledge about vasculitis, but patients may need to see other physicians who specialize in the area that a specific type of vasculitis affects—dermatologists, neurologists, ophthalmologists, pulmonologists and others. The treatment for vasculitis also varies with the type. Most forms are treated with steroids (glucocorticoids). Physicians may prescribe immune-suppressing drugs in some cases. Occasionally, damage from severe vasculitis may require surgery. In some forms of the disease, new classes of drugs that target certain immune cells may also help control symptoms of vasculitis.
Once diagnosed, the outlook for patients with these rare diseases depends on the type and severity of vasculitis. Some people live with the disease without it affecting their day-to-day activities, while others require consistent medical attention. In the past, people with the most severe forms might have been expected to survive only weeks or months. It is currently a goal of researchers and physicians that with proper treatment, and emerging new therapies, normal life spans and desired quality of life are possible for many people with vasculitis.