$Unique_ID{BRK04310} $Pretitle{} $Title{Vasculitis} $Subject{Vasculitis Angiitis Group of Systemic Polyarteritis Nodosa Necrotizing Vasculitis Hypersensitivity Vasculitis Wegener's Granulomatosis Lymphomatoid Granulomatosis Giant-Cell Arteritis Thromboangiitis Obliterans Buerger's Disease Mucocutaneous Lymph Node Syndrome Miscellaneous Vasculitides Systemic Lupus Erythromatosus Churg-Strauss Syndrome Purpura Schoenlein-Henoch Behcet's Syndrome Goodpasture Syndrome Kawasaki Disease} $Volume{} $Log{} Copyright (C) 1989, 1992 National Organization for Rare Disorders, Inc. 705: Vasculitis ** IMPORTANT ** It is possible that the main title of the article (Vasculitis) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Angiitis Information on the following diseases can be found in the Related Disorders section of this report: Group of Systemic Polyarteritis Nodosa Necrotizing Vasculitis Hypersensitivity Vasculitis Wegener's Granulomatosis Lymphomatoid Granulomatosis Giant-Cell Arteritis Thromboangiitis Obliterans (Buerger's Disease) Mucocutaneous Lymph Node Syndrome Miscellaneous Vasculitides Systemic Lupus Erythromatosus Churg-Strauss Syndrome Purpura, Schoenlein-Henoch Behcet's Syndrome Goodpasture Syndrome Kawasaki Disease General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Vasculitis is an inflammation of the blood vessel system which includes the veins, arteries and capillaries. It may occur alone or in conjunction with allergic and rheumatic diseases. Symptoms Vasculitis is a common disorder characterized by an inflammation of the blood vessel walls. 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 cause damage to the tissues (necrosis), possible formation of blood clots (thrombosis), a weakening or ballooning which can possibly cause a 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 localized or affect multiple areas of the body with inflammatory and destructive lesions. 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. 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. Causes Since there are many forms of Vasculitis, there are many causes. Some types may be caused by allergic reactions or hypersensitivity to certain medications such as sulfa or penicillin, other drugs, toxins, and other inhaled environmental irritants. Other forms may occur because of a fungal infection, parasites or viral infections, while in some cases there may be no apparent cause. In some instances vasculitis may be an autoimmune disorder. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.) against invading organisms suddenly begin to attack healthy tissue. Affected Population Vasculitis usually affects males and females in equal numbers. It is most commonly seen in the elderly. Related Disorders The following are diseases that can be associated with Vasculitis: Polyarteritis Nodosa is a group of systemic necrotizing vasculitis including the original classic polyarteritis nodosa, allergic granulomatosis, and those disorders that have the characteristics of both (an overlap syndrome). For more information on this disorder, choose "Polyarteritis" as your search term in the Rare Disease Database.) Hypersensitivity Vasculitis includes a wide group of vasculitic syndromes that affect the upper and lower respiratory tract and kidneys. They are usually caused by an allergic reaction to an unknown antigen. Giant Cell Arteritis includes several forms of Vasculitis that characteristically involves one or more branches of the carotid artery, in particular the temporal artery. There may be involvement of other blood vessels. (For more information on these disorders, choose "Arteritis "or "Takayasu" as your search terms in the Rare Disease Database.) Wegener's Granulomatosis is a rare collagen vascular disorder that begins as a localized inflammation of the upper and lower respiratory tract mucosa, and usually progresses into generalized inflammation of the blood vessels (vasculitis) and kidneys (glomulonephritis). (For more information on this disorder choose "Wegener" as your search term in the Rare Disease Database.) Lymphomatoid Granulomatosis is a more severe form of Vasculitis that infiltrates various tissues of the body, especially the lungs. It can be benign or malignant. Thromboangiitis Obliterans (Buerger's Disease) is a rare inflammatory disorder that usually affects the medium and small arteries of the upper lower extremities. It is more common in males and is closely related to smoking. Mucocutaneous Lymph Node Syndrome is an acute, non progressive, inflammatory illness that is unresponsive to antibiotics and affects infants and children. It is characterized by fever, enlarged lymph nodes in the neck (cervical adenitis), swelling of the skin (edema), flushing of the oral cavity, lips and palms of the hands, and shedding of the skin on the fingertips. In more severe cases there may be an inflammation of the arteries of the heart (coronary arteritis). Miscellaneous Vasculitides is a group of syndromes in which vasculitis is either the primary disorder or a symptom of another disease. Churg-Strauss Syndrome is a lung disorder often occuring as a complication of other disorders. Allergic blood vessel inflammation (angiitis or vasculitis) is accompanied by many inflammatory nodular lesions (granulomatosis) which may be small or granular, and are made up of compactly grouped cells. The age of onset varies from 15 to 70 years of age. (For more information on this disorder, choose "Churg-Strauss" as your search term in the Rare Disease Database). Systemic Lupus Erythematosus is an inflammatory connective tissue disease that can affect many parts of the body including the joints, skin and internal organs. Lupus is a disease of the body's immune system, most often striking young women between the ages 15 and 35 years. Vasculitis can be a symptom of Lupus. (For more information on this disorder, choose "Lupus" as your search term in the Rare Disease Database). Schoenlein-Henoch Purpura is one of a group of disorders characterized by purplish or brownish red discolorations of the skin. These spots may be large or small. Internal bleeding may occur in various areas of the body. This blood vessel disorder may affect the skin, joints, gastrointestinal system, kidneys, and in a very few cases the central nervous system. Little is known about the cause of this form of purpura although it may be an allergic reaction which more often occurs in children than in adults. (For more information on this disorder, choose "Schoenlein-Henoch" as your search term in the Rare Disease Database). Behcet's Syndrome is a relapsing multi-system inflammatory disease. The most common symptoms include oral and genital ulcers and inflammation of the eyes. The joints, blood vessels, central nervous system, and gastrointestinal tract may also be involved. Attacks may last a week to a month and recur spontaneously. (For more information on this disorder, choose "Behcet" as your search term in the Rare Disease Database). Goodpasture Syndrome is a rare inflammatory disorder involving the membranes of the lungs and kidneys. This disorder can be classified into three groups: autoimmune or antibody induced disease; systemic vasculitis (a vessel disease that may affect the body as a whole); and idiopathic (cause unknown). (For more information on this disorder, choose "Goodpasture" as your search term in the Rare Disease Database). Kawasaki disease is characterized by diseased lymph nodes in the neck, high fever, and a rash primarily over the trunk. This syndrome predominantly affects people of Japanese ancestry. Multiple sites of inflammatory and destructive lesions in the arteries (polyarteritis) occur, and may involve the coronary vessels in twenty percent of those affected. (For more information on this disorder, choose "Kawasaki" as your search term in the Rare Disease Database). Therapies: Standard Treatment of Vasculitis depends on the cause and symptoms of the underlying disease. 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. Therapies: Investigational At the present time research is being conducted on the use of high-dose intravenous gamma-globulin for some forms of Vasculitis. Another study uses a combination of cytotoxic agents 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. Clinical trials are underway to learn about the immunopathogenesis and optimal treatment of Vasculitis. Interested persons may wish to contact: Barton F. Haynes Box 3258 Duke University Medical Center Durham, NC 27710 (919) 684-5093 to see if further patients are needed for this research. This disease entry is based upon medical information available through January 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Vasculitis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Heart, Lung and Blood Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 421-8453 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 1288. THE MERCK MANUAL, Volume 2, 14th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck, Sharp & Dohme Laboratories., 1982. Pp. 798. PULMONARY DISEASES AND DISORDERS, Volume 2, 2nd Ed.: Alfred P. Fishman M.D., ed.-in-chief; McGraw-Hill Book Co., 1980. Pp. 1127. CEREBRAL ANGIOGRAPHY AS A GUIDE FOR THERAPY IN ISOLATED CENTRAL NERVOUS SYSTEM VASCULITIS. R. Stein et al.; JAMA, (April 24, 1987; issue 257 (16)). Pp. 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, (January 1989; issue 149 (1)). Pp. 161-165. SEVERE LEUKOCYTOCLASTIC VASCULITIS OF THE SKIN IN A PATIENT WITH ESSENTIAL MIXED CRYOGLOBULINEMIA TREATED WITH HIGH-DOSE GAMMA-GLOBULIN INTRAVENOUSLY. B.W. Boom et al.; ARCH DERMATOL, (October 1988; issue 124 (10)). Pp. 1550. CENTRAL NERVOUS SYSTEM VASCULITIS 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, (September 1988; Issue 31 (9)). Pp. 1194. VASCULITIS IN OLDER PATIENTS: PRESENTATIONS AND SIGNIFICANCE. A. Montonaro; GERIATRICS, (March 1988; Issue 43 (3)). Pp. 75-76; 79-83; 86.