Wegener’s Granulomatosis
What is it?
Wegener's granulomatosis is a rare form of
vasculitis, or inflammation of the blood vessels,
that begins in the respiratory system. As the
disease progresses, it can cause complete kidney
failure.
Who gets it?
Wegener's granulomatosis is so rare that only
around 500 new cases are diagnosed each year. It
is slightly more common in men than women, with
most patients being Caucasian. Wegener's
granulomatosis can occur at any age, but is more
likely to affect adults in their thirties and
forties.
What causes it?
The cause of Wegener's granulomatosis has not
been identified. However, it appears to be an
immune system response to an unknown trigger. The
bodies’ immune system produces white blood cells
and proteins called antibodies that attack and
kill foreign invaders – usually bacteria, viruses,
and fungi. Wegener's granulomatosis seems to be an
autoimmune disease, which means something causes
the immune system to see the body’s own tissues as
foreign invaders. In this case, the antibodies
actually attack the immune system’s own white
blood cells causing inflammation of the blood
vessels and inflamed tumor-like masses called
granulomas that interfere with blood flow.
Wegener's granulomatosis is not hereditary or
contagious, which means one person cannot pass it
on to another.
What are the symptoms?
In a person with Wegener's granulomatosis,
inflammation in the blood vessels causes them to
narrow and reduces blood flow throughout the body.
This can damage tissues and major organs. Symptoms
typically appear first in the upper respiratory
tract – the ears, nose, sinuses, and trachea. A
patient may seem to have a cold with runny nose,
cough, and sore throat, but the symptoms become
worse and can progress to pneumonia. Patients may
have frequent and severe nosebleeds, ear
infections, and sinus infections. They may have a
chronic cough and even cough up blood. Other
symptoms include fever, joint pain, loss of
appetite, anemia, general weakness, and fatigue.
Some patients may also develop sores on the skin.
In the most severe cases, patients develop renal,
or kidney, disease that can become life
threatening. Wegener's granulomatosis without
renal disease is called limited Wegener’s.
How is it diagnosed?
Because untreated Wegener's granulomatosis can
progress to renal failure, it is important to
diagnose and treat it as soon as possible. Your
doctor will study your medical history and perform
a complete physical examination. Diagnosis is
based primarily on a pattern of symptoms, but
blood tests can help rule out other possible
causes and support a diagnosis. Most people with
Wegener's granulomatosis will have a high level of
antineutrophil cytoplasmic antibodies (ANCAs) in
the blood. These antibodies are the ones that
attack the immune system’s own white blood cells.
The amount of ANCA in the blood can help measure
the progression of the disease. Blood tests may
also show a low red blood cell count (anemia) and
a high white count. Patients with renal disease
may also have a build-up of wastes in the blood,
called uremia, because the kidneys are not working
as they should. Urinalysis also may show red blood
cells in the urine. If symptoms indicate renal
disease, your doctor may order a kidney biopsy, in
which a small sample of kidney tissue is removed
for study. Other diagnostic tests include lung
x-rays and computed tomography (CT) scans of
affected organs.
What is the treatment?
Wegener's granulomatosis is treated with
immunosuppressive drugs, which inhibit the
activity of the immune system in order to control
the disease. One commonly used drug is
cyclophosphamide, also used in cancer treatment.
Wegener's granulomatosis is also treated with
corticosteroids that reduce inflammation. Blood
tests to measure the levels of antibodies in the
blood help your doctor determine the dosage
needed. Immunosuppressive drugs also compromise
the body’s ability to fight infection, so it is
important to guard against infections by receiving
vaccinations against the flu and pneumonia while
under treatment. Once fatal in most cases, people
with Wegener's granulomatosis now have a good
response to treatment when diagnosed early.
Self-care tips
Researchers have not yet found a way to prevent
Wegener's granulomatosis. If you have cold
symptoms that persist and progress, see a doctor
as soon as possible. Early diagnosis can help
control the progression of this disease.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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