Autoimmune inflammatory vasculitis causes inflammation of blood vessels and can be fatal.
What is Autoimmune Inflammatory Vasculitis?
Vasculitis, in general, refers to a condition where blood vessels are destroyed by inflammation. It can occur in the arteries (arteritis) or veins (phlebitis). It can affect blood vessels of any size and organs throughout the body. Vasculitis can also be a part of other diseases and problems, such as cancer, infections (i.e., hepatitis), exposure to drugs and chemicals, and rheumatic diseases, such as systemic lupus erythematosus or rheumatoid arthritis.1 Symptoms can vary, depending on which blood vessels are affected. The skin may be develop purpura (red spots), nodules (lumps), or ulcers (sores). The hands or feet could have weakness or numbness. Coughing and shortness of breath are other possible symptoms. Certain affected parts of the body may show no signs at all.2 Vasculitis can have one or repeated episodes, most cases are rare, but some lead to death, such as in the case of actor Harold Ramis. Ramis passed away from autoimmune inflammatory vasculitis, which is an extremely rare disease. In this disorder, the immune system attacks the blood vessels, which weakens and narrows the blood vessel walls. The blockages that result can affect organs and tissues to the point where they die from lack of blood supply. Over time, more symptoms accumulate and other organs and tissues get affected. Some cases of this disease can become rapidly serious, while other patients can live with autoimmune inflammatory vasculitis for years.3 Further symptoms can include fever, fatigue, hives, bruises, weight loss, aches and pains, and arthritis in the joints. For some, ruptured intestines, ear infections, burning eyes, headaches, stroke-like symptoms, nerve problems, and pneumonia symptoms can result.4 The arteries can not only be blocked, but they can leak or break. In Ramis’ situation, he developed an infection, and the resulting complications led to him having to relearn how to walk. He passed away “four years after contracting the condition”.5
The typical treatments for vasculitis are aimed at reducing inflammation and suppression of the immune system. Prednisone and other corticosteroids are prescribed, as well as immune suppression drugs (i.e., cyclophosphamide). Cephalexin or other antimicrobial agents could be used if there is an infection.6 For less severe cases, patients may be prescribed Azathioprine or Methotrexate, which are also used for other rheumatic diseases. Newer medications for inflammatory and autoimmune diseases, such as rituximab, may also be effective. Severe cases of vasculitis may need plasmapheresis (plasma exchange) or IVIg (intravenous immunoglobulin). Medications carry side effects and suppression of the immune system leaves patients open to infections.7 Other treatments include the cancer medication Cytoxin and anti-B-cell therapy. Some cases of the disease resolve on their own.8 Prognosis depends on the form of the vasculitis, the affected organs, and how quickly treatment begins. It can go into remission or it can be chronic. If an aneurysm occurs, surgery may be required.9
2, 7 http://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Vasculitis/