Disease Overview
Synonyms
Signs & Symptoms
Causes
Affected Populations
Disorders with Similar Symptoms
Diagnosis
Standard Therapies
Clinical Trials and Studies
Resources
References
Programs & Resources
Complete Report

Vasculitis

Last updated: July 28, 2015
Years published: 1989, 1992, 1994, 1997, 1998, 1999, 2008

Disease Overview

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.

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Synonyms

Angiitis

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Signs & Symptoms

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.

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Causes

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.

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Affected populations

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.)

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Disorders with Similar Symptoms

The following are diseases that are associated with vasculitis:

Polyarteritis nodosa is a rare multisystem disorder characterized by widespread inflammation, weakening, and degeneration of small- and medium-sized arteries. Blood vessels in any organ or organ system may be affected including arteries supplying the kidneys, heart, intestine, nervous system, and/or skeletal muscles. Damage to affected arteries may result in abnormally increased blood pressure (hypertension), “ballooning” (aneurysm) of an arterial wall, the formation of blood clots (thrombosis), obstruction of blood supply to certain tissues, and/or tissue damage and loss (necrosis) in certain affected areas. In many cases, affected individuals experience weight loss, fever, a general feeling of ill health (malaise), fatigue, weakness, headache, muscle aches (myalgias), and/or abdominal pain. Although the exact cause of polyarteritis nodosa is not known, many researchers suspect that the disorder is due to disturbances of the body’s immune system. (For more information on this disorder, choose “polyarteritis nodosa” 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.

Wegener’s granulomatosis is an uncommon collagen vascular disorder that usually 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 kidney (glomerulonephritis). Other symptoms may include ulcerations of the mucous membranes in the nose with secondary bacterial infection, middle ear infection (otitis media) with hearing loss, cough, expectoration of blood (hemoptysis), and inflammation of the thin membrane lining the outside of the lungs and the inside of the chest cavity (pleuritis). The exact cause of Wegener’s granulomatosis is not known. (For more information on this disorder choose “Wegener’s granulomatosis” as your search term in the Rare Disease Database.)

Lymphomatoid granulomatosis is a rare, progressive, vascular disease characterized by infiltration and destruction of the veins and arteries by lesions. These lesions can affect various parts of the body, especially the lungs. It can be a benign or malignant condition. (For more information on this disorder, choose “Lymphomatoid Granulomatosis” as your search term in the Rare Disease Database.)

Buerger’s disease, also known as thromboangiitis obliterans, is a rare disorder that, in most cases, affects young or middle-aged male cigarette smokers. It is characterized by narrowing or blockage (occlusion) of the veins and arteries of the extremities, resulting in reduced blood flow to these areas (peripheral vascular disease). The legs are more often affected than the arms. In most cases, the first symptom is extreme pain of the lower arms and legs while at rest. In addition, affected individuals may have sores (ulcers) on the extremities, numbness and tingling and a lack of normal blood flow to the fingers and/or toes when exposed to cold temperatures (Raynaud’s phenomenon), and/or inflammation and clotting of certain veins (thrombophlebitis). The exact cause of Buerger’s disease is not known; however, most affected individuals are smokers. (For more information on this disorder, choose “Buerger’s” as your search term in the Rare Disease Database.)

Churg-Strauss syndrome is a rare disorder that affects multiple organ systems and is characterized by the formation and accumulation of an abnormally large number of certain white blood cells (eosinophils), inflammation of blood vessels (angiitis or vasculitis), and inflammatory nodular lesions (granulomatosis). Other symptoms of Churg-Strauss syndrome include fever, a general feeling of weakness and fatigue (malaise), loss of appetite (anorexia) and weight loss. It is often preceded by asthma or allergies. The exact causes of Churg-Strauss syndrome is not known. (For more information on this disorder, choose “Churg Strauss” as your search term in the Rare Disease Database.)

Henoch-Schonlein purpura is a rare inflammatory disease of the small blood vessels (capillaries) and is usually a self-limited disease. It is the most common form of childhood vascular inflammation (vasculitis) and results in inflammatory changes in small blood vessels. The symptoms of Henoch-Schonlein purpura usually begin suddenly and may include headache, fever, loss of appetite, cramping abdominal pain, and joint pain. Red or purple spots typically appear on the skin (petechial purpura). Inflammatory changes associated with this disorder can also develop in the joints, kidneys, digestive system, and, in rare cases, the brain and spinal cord (central nervous system). The exact cause of Henoch-Schonlein purpura is not fully understood, although research suggests that it may be an autoimmune disease or, in some cases, an extreme allergic reaction to certain offending substances (e.g., foods or drugs). (For more information on this disorder, choose “Henoch Schonlein Purpura” as your search term in the Rare Disease Database.)

Behcet’s syndrome is a rare chronic relapsing inflammatory disorder characterized by inflammation of the eyes and ulcers of the mouth and genitals. The cause is unknown. Symptoms include areas of abnormal skin changes (ulcers or lesions) of the mouth and genitals that tend to recur spontaneously. Other systems of the body may also be effected including the joints, blood vessels, central nervous system, and/or digestive tract. (For more information on this disorder, choose “Behcet” as your search term in the Rare Disease Database.)

Kawasaki syndrome, also known as mucocutaneous lymph node syndrome, is an inflammatory disease of childhood characterized by fever, skin rash, swollen lymph nodes (lymphadenopathy), inflammation of arteries (polyarteritis), and inflammation of the blood vessels (vasculitis). Inflammatory changes cause destructive lesions in blood vessels and may lead to complications involving the liver, gall bladder, and especially the heart. Kawasaki syndrome is the main cause of acquired heart disease in children. The exact cause of Kawasaki syndrome is unclear although it may be related to two previously unknown strains of staphylococcus and streptococcus bacteria. (For more information on this disorder, choose “Kawasaki” as your search term in the Rare Disease Database.)

Hypocomplementemic urticarial vasculitis is a rare disorder characterized by recurrent episodes of vasculitis and (urticaria) associated with fever, arthritis, and abdominal pain. Other symptoms include swelling of (angioedema), ocular inflammation, and/or inflammation and degeneration of the clusters of capillaries (renal glomeruli) that filter the blood passing through the kidneys (glomerulonephritis). In some cases a chronic obstructive lung disease may develop. The exact cause of hypocomplementemic urticarial vasculitis is unknown.

Primary angiitis of the central nervous system (CNS), also known as (isolated) vasculitis of the central nervous system, is an extremely rare disorder characterized by vasculitis of the CNS that occurs as a primary condition (i.e., not secondary to another disorder). Physical findings and symptoms associated with this disorder depend upon the specific blood vessels of the central nervous system that are affected.

There are additional disorders that may be characterized by inflammation of certain blood vessels (vasculitis). These disorders include giant cell arteritis, polymyalgia rheumatica, lupus, essential mixed cryoglobulinemia, cutaneous necrotizing vasculitis, Cogan’s syndrome type I, Takayasu’s arteritis, and rheumatoid arthritis. (For more information on these disorders, choose the exact disease name in question as your search term in the Rare Disease Database.)

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Diagnosis

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.

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Standard Therapies

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.

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Clinical Trials and Studies

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:

www.centerwatch.com

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.

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Resources

RareConnect offers a safe patient-hosted online community for patients and caregivers affected by this rare disease. For more information, visit www.rareconnect.org.

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References

TEXTBOOKS

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.

JOURNAL ARTICLES

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.

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Programs & Resources

RareCare^® Assistance Programs

NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.

Additional Assistance Programs

MedicAlert Assistance Program

NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.

Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/

Rare Disease Educational Support Program

Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.

Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/