Vasculitis and Vasculitis treatment, refers to a cluster of rare autoimmune diseases, associated with inflammation of the body's vascular system of arteries and veins. Approximately 3,000 new cases appear each year in the UK. The exact cause of vasculitis is unknown. It's probable that the inflammation and damage, which is caused by autoantibodies attacking healthy tissue, is triggered by the patient's own genes combined with exposure to certain environmental factors, such as chemicals, or the use of specific medications. It can also occur in individuals who already have another autoimmune disease, such as rheumatoid arthritis or Crohn's disease.
One type of vasculitis is Churg-Strauss syndrome, first identified in 1951 by Jacob Churg and Lotte Strauss. In 2011, Churg-Strauss syndrome was renamed as Eosinophilic Granulomatosis with Polyangiitis or EGPA. EGPA is referred to as an ANCA positive form of vasculitis, because of the presence of anti-neutrophil cytoplasmic antibodies in the patient. This chronic multi-system syndrome affects the small and medium sized blood vessels of individuals, aged 30 to 45 with more men affected than women. Usually diagnosed in patients, presenting with asthma and an increase in the presence of eosinophils, a type of white blood cell, EGPA affects the lungs and peripheral nervous system. It can also affect the heart, kidneys and the gastrointestinal system.
The treatment for vasculitis varies greatly depending on the duration and severity of the symptoms and the underlying cause of the disease. The Birmingham Vasculitis Activity Score (canvasc.ca/pdf/bvasv3.pdf) and the Vascular Disease Index are clinical tools used to assess and measure the extent, severity and damage to the body caused by vasculitis.
The British Society for Rheumatology and the British Health Professionals in Rheumatology have guidelines for the treatment of EGPA and the other ANCA positive vasculitis syndromes, which are Wegener's granulomatosis and microscopic polyangiitis.
Because there is no cure for vasculitis, the goal of treatment is first to bring the disease under control, using Remission Induction therapy, and then to keep it under control, with Remission Maintenance therapy. Both therapies use a combination of drugs, which vary based on the severity and type of vasculitis, to suppress the immune system, and reduce the inflammation. During the Remission Induction phase of treatment, prednisolone, a steroid, is prescribed together with cyclophosphamide, an immunosuppressive agent, or methotrexate, which controls the activity of immune system enzymes. For some types of vasculitis, newer drugs like rituximab, which destroys certain types of white blood cells or infliximab, a monoclonal antibody which partly suppresses the immune system and changes the inflammation process, replace cyclophosphamide. Side effects of these powerful drugs include the possibility of various infections due to their immunosuppressive effects.
Once the disease symptoms have improved, the vasculitis treatment is directed toward maintaining the improved health status of the patient. The dose of prednisolone is lowered, and cyclophosphamide, if used, maybe switched for a drug with fewer toxic effects, such as azathioprine. The duration of Remission Maintenance therapy depends on the individual patient, the type of vasculitis and the severity of the disease. Like other autoimmune or chronic diseases, good nutrition, appropriate exercise and stress reduction techniques can help patients coping with vasculitis.