Vasculitis is an inflammation of the wall of blood vessels, arteries, veins, or capillaries. This inflammation causes a weakening and narrowing of the blood vessel lumen that can progress to the point of blood vessel blockage or hemorrhage. There is no known cause, or cure, for the primary types of vasculitis. There are some probable causes, and “cures” in rare drug and bacterial (meningitis), triggered vasculitis.
A result of vasculitis is that the tissues and organs supplied by the affected blood vessels do not get enough blood. This can result in organ and tissue damage, which can be irreversible, thus causing morbidities, and sometimes leads to death. Vasculitis is a family of about 26 separate, but related, diseases within the larger family of more than 110 arthritic diseases. It is also considered an autoimmune disease. An autoimmune disease occurs when the body mistakes its own cells for foreign invaders and produces antibodies to attack the perceived invaders, for example, as a defense against infection.
The different types of vasculitis are classified according to the size and location of the blood vessels that are affected. All are considered rare diseases, and affect people of all ages, gender and ethnicity. Though some specific forms of vasculitis can improve on their own, most require treatment. The duration of treatment varies, with some people using medications for an extended period of time, often years. Vasculitis is more common than you think, and can be more serious than you expect!
Vasculitis can affect any blood vessel or organ in the body, including the skin, joints, lungs, kidneys, gastrointestinal tract, eyes, brain, nerves, sinuses, nose and ears. Signs and symptoms of Vasculitis vary widely in type and severity. Some are specific to a particular organ and others are non specific causing general aches, pains and fatigue. Some types of Vasculitis are more likely to occur in certain populations than others.
Early diagnosis and proper treatment can bring vasculitis into remission. Many patients lead full, productive lives with the right management of their chronic disease, many others do not. Treatment usually consists of a combination of powerful drugs such as Rituximab, Cyclophosphamide, Methotrexate, Azathioprine, or others, with glucocorticoids (prednisone). Although basic treatment is similar, it will vary depending on the specific vasculitis, severity of symptoms, the patient’s general health status and associated conditions/comorbidities.
Treatment is often divided into two stages: firstly, the induction of disease remission, and secondly, the maintenance of disease remission. Patients must follow treatment instructions carefully, for example, oral cyclophosphamide should be followed with plenty of water to flush away harmful by-products, and requires frequent lab monitoring. Ideally, its use will be limited to a 3 to 6 month duration, and a +/-25g lifetime exposure limit, with follow-up and long-term cancer screening via urine dipstick etc. Effective treatment may require a “team” approach with specialists like a: nephrologist (kidney), otolaryngologist (ear, nose/sinus, throat), ophtalmologist (eye), pulmonologist/respirologist (lung), others as needed, and always consult with a vasculitis specialist, usually a rheumatologist/immunologist.
It is imperative to have a close, continuous and long-term follow-up, even when in remission and off drug therapy since the disease, in some patients, can relapse. For example, in ANCA associated vasculitis relapses and “flares” occur in over 50% of patients as time goes on. To help manage their disease, patients must maintain a good relationship with their doctors, understand and follow instructions carefully. Many patients find it useful to maintain a diary listing medications, test results, and notes on any symptoms they are experiencing. These notes can be reviewed during a patient/doctor appointment.