Vasculitis

Vasculitis is inflammation of the blood vessels and there are many types. The rash from being allergic to a medicine is a type of vasculitis. Most types are rare, but some can cause severe damage to the parts of the body the blood vessel supplies with blood. Some types of vasculitis are associated with autoimmune diseases, medicines, and infections, but we do not know the causes. Some types only occur in children, some only in the old.

The diagnosis is usually established with a biopsy, although blood tests and angiography x-ray tests can help. The types of vasculitis are based on the size of the blood vessels involved. The large artery types are Giant cell arteritis, Takayasu’s arteritis, and aortitis, which can be associated with Cogan’s syndrome or spondyloarthropathies. Medium sized artery types are Kawasaki’s disease and Polyarteritis nodosa. Small to medium sized artery types are ANCA associated, Granulomatosis with polyangiitis, Microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis, and Primary angiitis of the central nervous system. Small artery types are IgA vasculitis, Vasculitis of rheumatoid arthritis, lupus, Sjogrens and other autoimmune diseases, Cryoglobulinemic vasculitis, Anti-glomerlular basement membrane disease, and drug induced vasculitis. Vasculitis of arteries and veins of various sizes are Behcet’s disease and Relapsing polychondritis. There are other forms of vasculitis related to infection and cancer that are not listed.

Treatment is with corticosteroids, often at high dose. Other drugs are used to minimize the dose of corticosteroids such as Methotrexate, Imuran, Cellcept, Cytoxan, Rituxan and some patients need plasma exchange of their blood (plasmapheresis) or intravenous immunoglobulin infusions, IVIg. Vasculitis can be brief or life long and damage to organs may require surgery for grafting arteries to replace blood suppy. Side effects from the corticosteroids or other medicines require monitoring for complications. The Vasculitis Foundation www.vasculitisfoundation.org/education/forms is available for more information.