Wegener's granulomatosis is a rare autoimmune disease that causes inflammation of the blood vessels. It affects many tissues in the body, including the lungs, kidneys, and skin. While the causes are unknown, it affects about 1 in 20,000 to 30,000 people equally among both sexes. Diagnosis involves biopsies of affected tissues like the lungs and kidneys. Treatment consists of high doses of steroids and immunosuppressive drugs to control symptoms and prevent relapses.

1. Jackie Ward

2. A disease of “abnormal immune
function”.
Affects many of the body’s tissues,
including blood vessels (vasculitis).
Although causes are unknown, it
affects about one in every 20,000 or
one in every 30,000 people.

3.  Not contagious
 No evidence that the
disease is hereditary
 Systemic : affects the
body as a whole

4.  Equally distributed between sexes
 Slight male dominance
 May affect anyone, but typically in middle aged men or
women.
 Unusual to occur in childhood, but not unusual for
diagnosis to be detected in 70’s or 80’s.

5.  X-rays may show a pneumonia-like
symptoms on lungs
 Because the disease normally
involves the upper respiratory
tract, doctors biopsy these tissues
for diagnosis.
 There are also lung and kidney
biopsies, with lung biopsies being
the best way of diagnosis.

6.  The most recent and simple
procedure in diagnosis, are ANCA’s
(anti-neutrophil cytoplasmic
antibodies)
 Antibodies are directed against the
cytoplasm of white blood cells.
 Pros: when done correctly, may
lead to early diagnosis
 Cons: can often lead to
misdiagnosis and incorrect
treatment.

7.  Ear: infections slow to resolve;
decrease in hearing.
 Eye: inflammation in parts of eye,
can cause protrusion of one eye.
 Nose: Nasal crusting and nosebleeds
may occur. The bridge of the nose
can collapse, along with erosion of
the septum (saddle-nose deformity).
 Sinuses: Chronic sinus inflammation
which leads to the destruction of
surrounding tissues.
 Trachea: narrowing of windpipe –
“subglottic stenosis”

8.  Lungs: Pneumonia-like symptoms seen on x-rays.
Bleeding may occur.
 Kidneys: inflammation leads to small or large amounts
of protein in blood. If this is not treated, it can lead to
kidney failure and renal masses can occur.
 Skin: rashes appear (small purple/red dots on lower
extremities) “palpable purpura”.
 Joints: swelling pain
 Nerves: numbness, tingling, shooting pains

9.  High dose steroid - cortisone (prednisone)
 Immunosuppressive drug, cyclophosphamide (cytoxan)
 Because over one half of patients with Wegener’s
experience relapses, Bactrim is also prescribed to
prevent these reoccurrences.

10.  http://www.medicinenet.com/wegeners_granulomatosi
s/page2.htm
 http://vasculitis.med.jhu.edu/typesof/wegeners.html
 http://www.vasculitisfoundation.org/wegenersgranulom
atosis