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$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
     [phone]

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
     [phone]

     NIH/National Heart, Lung and Blood Institute
     9000 Rockville Pike
     Bethesda, MD 20892
     [phone]

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.