Vasculitis is inflammation of blood vessels. In individuals with vasculitis, inflammation damages the lining of affected blood vessels, causing narrowing, the formation of blood clots (thrombosis), and/or blockage. As a result, there may be restriction of oxygenated blood supply to certain tissues (ischemia), potentially resulting in pain, tissue damage, and, in some cases, malfunction of certain affected organs. Vasculitis may affect veins and arteries of any type or size; may involve a single organ or many organs and tissues of the body; and may be a primary disease process or occur due to or in association with a number of different underlying disorders. Therefore, the range and severity of symptoms and findings associated with vasculitis may vary greatly. The specific underlying cause of vasculitis is not fully understood. However, in most cases, it is thought to be due to disturbances of the body’s immune system.
Vasculitis is a general term that means inflammation of blood vessels. This inflammation causes a narrowing of the inside of the vessel and can obstruct the flow of blood to the tissues (ischemia). The lack of blood may result in damage to nearby tissues (necrosis), formation of blood clots (thrombosis), and, in rare cases, a weakening or ballooning that may rupture of the vessel wall (aneurysm).
Arteries and veins of all sizes and in all parts of the body may be affected. Vasculitis may be limited to only one location or certain organs (localized or isolated) such as the skin, brain, or specific internal organs. In other cases, vasculitis may affect multiple areas or organs of the body at the same time (systemic or generalized). It may occur alone or as a complication of many other disorders.
The symptoms of vasculitis are many because of the wide variety of body systems it can affect. Depending on the system involved there may be muscle pain, joint pain, fever, weight loss, loss of appetite (anorexia), headache, or generalized weakness. There may also be ulcers of the mouth, hoarseness, night sweats, high blood pressure (hypertension), abdominal pain, diarrhea, blood in the urine (hematuria), or kidney (renal) failure. Eye inflammation and blurred vision are also symptomatic, and in very severe cases blindness can occur. When the respiratory system is involved there may be an inflammation of the sinuses, runny nose, asthma, a cough with or without bleeding (hemoptysis), shortness of breath (dyspnea), nosebleeds (epistaxis), or an inflammation of the membranes of the lungs.
When vasculitis affects the skin there may be lesions that are flat and red (macules), nodules, and hemorrhages under the skin (purpura). These lesions may occur on any area of the body but are seen more frequently on the back, hands, buttocks, the inside area of the forearms and the lower extremities. These skin symptoms may occur only once or at regular intervals. They will usually last for several weeks and may leave darkened spots or scarring. In some cases of vasculitis there may be wheel-like lesions that cause intense itching (urticaria), or ring-shaped lesions and ulcers. Blister-like lesions (vesicles, bullae) may develop in severe cases.
The specific underlying cause of vasculitis is not fully understood. However, in most cases, vasculitis is thought to be due to disturbances of the body’s immune system. Some forms of vasculitis may be due to allergic reactions or hypersensitivity to certain medications such as sulfur drugs, penicillin, propylthiouracil, other drugs, toxins, or other inhaled environmental irritants. Other forms may occur due to fungal, parasitic, or viral infections. In some instances, it is thought that vasculitis may be an autoimmune disorder. Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.
Vasculitis usually affects males and females in equal numbers. It is most commonly seen in the elderly.
There are a large number of disorders that may be characterized by or associated with vasculitis. These disorders are often referred to as the Vasculitic syndromes or vasculitides. (For more information on these disorders, see the Related Disorders section below.)
Because of the wide range of symptoms and body systems involved, an extensive history and physical exam is needed before a clear diagnosis of the type of vasculitis can be made. In some cases, an x-ray of the blood vessels using dye (angiogram), or a biopsy of the affected organ may be recommended to give an accurate diagnosis and to insure proper treatment.
Treatment of vasculitis depends on the cause and symptoms of the underlying disease and the specific organs of the body that are affected. The drugs prednisone, cyclophosphamide, methylprednisolone and pentoxifylline have proven to be successful in treating the autoimmune form of vasculitis. Other treatment is symptomatic and supportive.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
For information about clinical trials sponsored by private sources, contact:
At the present time, research is being conducted on the use of high-dose intravenous gamma-globulin for some forms of vasculitis. Other studies are examining a combination of cytotoxic agents, including cyclophosphamide and steroids, as possible treatments for certain types of vasculitis.
Plasmapheresis may be of benefit in some cases of vasculitis. This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient’s plasma is then replaced with other human plasma and the blood is retransfused into the patient. More research must be conducted to determine long-term safety and effectiveness of these drugs and procedures.
Studies are underway to determine the use of monoclonal antibody therapy as a possible treatment for systemic vasculitis in those individuals who are not responsive to immunosuppressive drugs. More research is needed to determine the long-term safety and effectiveness of monoclonal antibody therapy as a treatment for vasculitis.
RareConnect offers a safe patient-hosted online community for patients and caregivers affected by this rare disease. For more information, visit www.rareconnect.org.
Harrison’s Principles of Internal Medicine, 14th Ed.: Anthony S. Fauci, M.D. et al., Editors; McGraw-Hill, Inc., 1998. P. 167
Internal Medicine, 4th Ed.: Jay H. Stein, Editor-In-Chief; Mosby-Year Book, Inc., 1994. Pp. 2430-41.
The Merck Manual, Volume 2, 15th Ed.: Robert Berkow, M.D. ed.-in-chief; Merck, Sharp & Dohme Laboratories., 1987. Pp. 1272-73.
Textbook of Rheumatology, 4TH Ed.: William N. Kelley et al., Editors; W.B. Saunders Company, 1993. Pp. 1077-1102.
Cecil Textbook of Medicine, 19th Ed.: James B. Wyngaarden and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1992. Pp. 2025-28.
Pulmonary Diseases and Disorders, Volume 2, 2nd Ed.: Alfred P. Fishman M.D., Ed.-in-chief; McGraw-Hill Book Co., 1980. P. 1127.
Belizna CC, Kerleau JM, Heron F, Cailleux N, Lévesque H. Vasculitis and myelodysplasia. Isr Med Assoc J. 2008 Feb;10(2):156-7
Guedes-Barbosa LS, Batista EV, Martins DC, Neder L, Crepaldi N, Martins EV.
Necrotizing cutaneous vasculitis in multibacillary leprosy disease (Lucio’s phenomenon).
J Clin Rheumatol. 2008 Feb;14(1):57-9.
Cerebral Angiography as a Guide for Therapy in Isolated Central Nervous System Vasculitis. R. Stein et al.; JAMA, (Apr 24, 1987; 257 (16)). P. 2193.
Diagnostic Studies for Systemic Necrotizing Vasculitis. Sensitivity, Specificity and Predictive Value in Patients with Multisystem Disease. P.J. Dahlberg et al.; ARCH INTERN MED, (Jan 1989; 149 (1)). Pp. 161-65.
Severe Leukocytoclastic Vasculities of the Skin in a Patient with Essential Mixed Cryoglobulinemia Treated with High-Dose Gamma-Globulin Intravenously. B.W. Boom et al.; ARCH DERMATOL (Oct 1988; 124 (10)). P. 1550.
Central Nervous System Vasculities in Behcet’s Syndrome; Angiographic Improvement After Therapy with Cytotoxic Agents. J.D. Zelenski et al.; Arthritis Rheum, (February 1989; Issue 32 (2)). Pp. 217.
Reversal of Progressive Necrotizing Vasculitis with Intravenous Pulse Cyclophosphamide and Methylprednisolone. J.G. Fort et al.; Arthritis Rheum (Sep 1988; 31 (9)). Pp. 1194.
Vasculitis in Older Patients: Presentations and Significance. A. Montonaro; Geriatrics (Mar 1988; 43 (3)). Pp. 75-76, 79-83, 86.
Long-Term Remission on Intractable Systemic Vasculitis with Monoclonal Antiobdy Therapy. C.M. Lockwood et al.; Lancet (Jun 1993; 341(8861)). Pp. 1620-21.
Vasculitis and Antineutrophil Cytoplasmic Autoantibodies Associated with Propylthiouracil Therapy. Th. J. Vervaat et al.; Lancet (Sep 1993; 342(8872)). Pp. 651-52.
Small-Vessel Vasculitis. J.C. Jennette et al.; New Eng J Med (Nov 20 1997; 337(21)). Pp. 1512-23.
The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.
National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 • (203)744-0100