CD-ROM Today (UK) (Spanish) 15/CDRT.iso/dp/0378/03786.txt

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$Unique_ID{BRK03786}
$Pretitle{}
$Title{Granulomatosis, Wegener's}
$Subject{Granulomatosis Wegener's Necrotizing Respiratory Granulomatosis
Pathergic Granulomatosis}
$Volume{}
$Log{}

Copyright (C) 1986, 1988, 1989 National Organization for Rare Disorders,
Inc.

270:
Granulomatosis, Wegener's

** IMPORTANT **
It is possible the main title of the article (Wegener's Granulomatosis)
is not the name you expected.  Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Necrotizing Respiratory Granulomatosis
     Pathergic Granulomatosis

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.


Wegener's Granulomatosis is an uncommon 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 kidney (glomerulonephritis).

Symptoms

Onset of Wegener's Granulomatosis may be gradual or acute.  Initial symptoms
usually occur in the upper respiratory tract and appear as a severe common
cold, paranasal sinusitis, 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 pleuritis.  The
nasal mucous membrane appears red with a raised granular appearance, and it
may bleed easily.

Other initial symptoms may include fever, malaise, loss of appetite,
weight loss, and migratory disease of the joints (polyarthropathy).  Skin
lesions and eye problems such as obstruction and bulging of the tear duct may
be present.  Inflammation of the ear cartilage (chondritis), myocardial
infarction caused by the inflammation of the blood vessels (vasculitis),
aseptic meningitis and non-healing granuloma of the central nervous system
may also occur.

After a few weeks or months, a disseminated vascular phase characteristically
develops.  Necrotizing inflammatory skin, lung and kidney lesions
(glomerulitis) may progress, causing hypertension and kidney failure (uremia).

Kidney disease is the hallmark of this disorder in the generalized phase.
Urinalysis shows protein (proteinuria), blood (hematuria), and red blood cell
casts in the urine.  Functional kidney impairment is inevitable without
immediate appropriate therapy.

Occasionally, the disease is limited to the lungs.

The levels of the complex series of enzymatic proteins in the blood serum
(serum complement) are normal or elevated.  The sedimentation rate of red
blood cells (erythrocyte sedimentation rate or ESR) is elevated.  An increase
in white blood cells (leukocytosis) is present.  Antinuclear antibodies and
lupus erythematosus cells are not found in patients with Wegener's
Granulomatosis.

Causes

The exact cause of Wegener Granulomatosis is unknown.  Though the disorder
resembles an infectious process, no viral, bacterial or other causative agent
has been isolated.  Because of the characteristic histologic tissue changes,
an allergic reaction has been suggested as a possible basis for the disorder.

Affected Population

Wegener's Granulomatosis can occur at any age.  It affects males about twice
as often as females.

Related Disorders

Polyarteritis is a disorder characterized by inflammation and necrosis of
medium-sized muscular arteries, with secondary lack of oxygen in the tissues
supplied by the affected vessels.  Differential diagnosis of Polyarteritis is
ruled out by biopsy of the skin lesions and pathologic examination of the
vascular lesions.  The presence of cells that are easily stained with eosin
(eosinophilia), is not a feature of Wegener's Granulomatosis, but it is often
found in polyarteritis.  Nasal and pulmonary granulomatous inflammation are
absent in polyarteritis.

The vascular renal phase of Subacute Bacterial Endocarditis (SBE) can be
differentiated from Wegener's Granulomatosis by characteristic blood cultures
and changing heart murmurs.

Systemic Lupus Erythematosus can be differentiated from Wegener's
Granulomatosis by the presence of antinuclear antibodies and Lupus
Erythematosus cells in the serum.  Additionally, the serum complement level
is depressed.  (For more information on this disorder, choose "Lupus" as your
search term in the Rare Disease Database.)

Glomerulonephritis is a disorder characterized by diffuse inflammatory
changes in the kidney glomeruli, by the abrupt appearance of protein and
blood in the urine, and, by red blood cell casts.

In Midline Malignant Reticulosis vascular granulomatous inflammation is
absent.

Therapies:  Standard

The complete syndrome of Wegener's Granulomatosis can progress rapidly to
kidney failure once the diffuse vascular phase begins.  Persons with the
limited form of the disorder have only nasal and pulmonary lesions with
little or no systemic involvement.  Pulmonary symptoms may improve or worsen
spontaneously.

Early diagnosis and treatment are important in view of the potential
success of drugs that have a toxic effect on certain cells (cytotoxic drugs).
The prognosis for this disease has been improved in recent years by treatment
with immunosuppressive cytotoxic agents.  Cyclophosphamide is the drug of
choice, but azathioprine and chlorambucil are also useful.

Duration of therapy depends on the patient's response.  White blood cell
(leukocyte) counts are closely monitored.  Dosages are reduced gradually to
prevent severe deficiency of white blood cells.  Attempts should be made to
discontinue therapy if symptoms of the disorder have been absent for one
year.  The possibility of kidney disease relapse is carefully monitored when
tapering medication dosage or discontinuing the drug.  Long-term, complete
remissions are often achieved with drug therapy, even with advanced disease.

Kidney transplantation has been successful for kidney failure resulting
from Wegener's Granulomatosis.

Corticosteroids can be used intermittently for non-renal symptoms in
conjunction with the above drugs.

Research at the National Institute of Allergy and Infectious Diseases
(NIAID) on regulation of the immune function has benefited patients with such
inflammatory vascular diseases as Wegener's granulomatosis, lymphatoid
granulomatosis and polyarteritis nodosa.  Before the introduction of
treatment regimens developed at the NIAID, the majority of those with
Wegener's granulomatosis died within one year after the onset of the
disorder.  Today, ninety-three percent of those treated with cyclophosphamide
and prednisone show complete remission.

Therapies:  Investigational

The National Institute of Allergy and Infectious Diseases (NIAID) is
conducting a study on a treatment for Wegener's Granulomatosis.  Patients who
wish to participate in the clinical trials must be at least 14 years of age
and had a recent onset of the illness.  For further information, physicians
can contact Dr. Gary S. Hoffman or Dr. Randi Y. Levitt at NIH/National
Institute of Allergy and Infectious Diseases, Bldg. 10, Rm. 11813, 9000
Rockville Pike, Bethesda, MD  20892 or phone [phone].  Travel expenses
are usually covered by the NIH and all treatment is free.

This disease entry is based upon medical information available through
September 1989.  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 Wegener's Granulomatosis, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     Wegener's Granulomatosis Support Group, National Chapter
     P.O. Box 1518
     Platte City, MO  64079
     [phone] or [phone]

     Wegener's Foundation
     9000 Rockville Pike
     Bldg. 31A, Rm. B1W30
     Bethesda, MD  20892

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

     The National Kidney Foundation
     30 East 33rd St.
     New York, NY  10016
     [phone] or [phone]

     American Lung Association
     1740 Broadway
     New York, NY  10019
     [phone]

References

The Merck Manual of Diagnosis of Therapy:  Berkow et al., eds.:  Merck Sharp &
Dohme (1982).