Wegener's Granulomatosis
Medical Author: William C. Shiel Jr., MD, FACP, FACR
What is Wegener's granulomatosis?
Wegener's granulomatosis is a uncommon type of inflammation of
small
arteries and veins (vasculitis). It classically involves
inflammation of
the arteries that supply blood to the tissues of the lungs, the
nasal
passages
(sinuses), and the kidneys. "Incomplete" forms exist
that only
involve one of these areas. When both lungs and kidneys are affected, the condition is sometimes referred to as generalized Wegener's granulomatosis. When only the lungs are involved, the condition is sometimes referred to as limited Wegener's granulomatosis.
Wegener's granulomatosis usually
affects young
or middle-aged adults. Although it is uncommon in children, it
can affect people at any age. The cause of Wegener's granulomatosis
is not known.
What are symptoms of Wegener's granulomatosis?
Symptoms of Wegener's granulomatosis include fatigue, weight
loss,
fevers, shortness of breath, bloody sputum, joint pains, and
sinus
inflammation (sinusitis). Nasal ulcerations and even bloody
nasal discharge
can occur. Other areas of the body that can also become
inflamed in
patients with Wegener's granulomatosis include the eyes, the
nerves
(neuropathy), the middle ear (otitis media), and the skin resulting in skin nodules or ulcers.
How is Wegener's granulomatosis diagnosed?
Abnormal lab findings in patients with Wegener's
granulomatosis include
urine tests that detect protein and red blood cells in the
urine (not
visible to the naked eye) and x-ray tests of the chest and
sinuses which
detect abnormalities resulting from lung and sinus
inflammation. Blood
tests that detect the abnormal inflammation include the
sedimentation rate (sed rate) and
C-reactive
protein. A more specific blood test
used to
diagnose and monitor Wegener's granulomatosis is the
antineutrophil
cytoplasmic antibody (ANCA test), which is commonly elevated when the disease is active.
The diagnosis of Wegener's granulomatosis
is
confirmed by detecting both abnormal cellular formations, called
granulomas, and vasculitis in a biopsy of tissue involved
with the
inflammatory process. For examples, an open lung biopsy or a kidney biopsy are commonly used in making a diagnosis of Wegener's granulomatosis.
Next: How is Wegener's granulomatosis treated? »
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Last Editorial Review: 2/14/2008
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