F.A.Q.
What is Wegener's Granulomatosis?
Wegeners
Granulomatosis is an uncommon disease, a form of vasculitis (inflammation
of the blood vessels.), and an auto immune disease (meaning the bodies own
immune system attacks its own body tissues). There is no known cause for
WG; but it is not contagious, and there is no evidence it is hereditary.
It is systemic, meaning it affects the body as a whole. It affects the
upper (sinuses and nose) and lower (lungs) respiratory system and may
involve kidneys, eyes, ears, throat, skin and other body organs. For
reasons not clear, blood vessels in those areas become inflamed and piles
of debris appear (granuloma).
Patients who do not have a renal (Kidney) involvement are said to have
limited Wegeners.As awareness of WG grows, more patients are diagnosed in
the early stages when treatment can affect early remission and prevent
organ failure.
Who
gets Wegener's Granulomatosis?
Patients are divided equally
between male and female. They range in age from 3 to 89, with the peak of
the disease in the 4 th
or 5 th decade of
life. It is definitely an uncommon disease and can occur at any age.
What are some of the signs or symptoms?
Onset of Wegeners
Granulomatosis may be non apparent, that is, does not exhibit early
symptoms of its advent, or may have a rapid severe onset. About 90% of
patients have symptoms of a 'cold' or 'sinusitis' that fail to respond to
usual therapeutic measures and last considerably longer than the usual
upper respiratory tract infection. Other symptoms include nasal membrane
ulceration's and crusting, saddle nose deformity, inflammation of the ear
with hearing problems, cough (with or without blood), pleuritis
(inflammation of the lining of the lung), allergy like symptoms, skin
eruptions, fever, lack of energy, loss of appetite, weight loss, joint
pain, night sweats, changes in colour of urine, and weakness. All patient
do not experience all of these symptoms. Be aware that different symptoms
and the severity of the disease vary in each. If any symptoms persist
consider WG and have the ANCA test.
How is
it diagnosed ?
Diagnosis is
established by clinical and laboratory findings such as the ANCA test (a
blood test). WG is often confirmed by biopsy. Antineutrophil Cytoplasmic
Antibody (ANCA) is manufactured by the bodies immune system. ANCA was
found in the majority of patients and its level correlates with the
disease activity. Since most people with WG produce ANCA, they usually
have a positive ANCA test, especially when symptoms worsen. The ANCA test
is also helpful in monitoring treatment, since the amount of ANCA in the
blood tends to fall in some patients as the disease gets better.
What
is the treatment?
Treatment of WG
usually consists of cytoxic agents (Cytoxan) with or without a
glucocorticoid (prednisone). Although basic treatment is the same, it will
vary with increased symptoms and involvement of the disease in each
individual. You must maintain a good relationship with your doctor, and
understand the following instructions carefully. It is imperative to have
a close, continuous, long-term follow up, even when in remission and off
therapy.
Is there a cure ?
There is no known
cure for WG, but early diagnosis and proper treatment means therapy will
be affective and the disease can be brought into remission. Long term
remission can be induced and maintained with medications, close
management, and frequent ANCA tests for monitoring the disease while
Wegeners is inactive. Although treatment can produce symptom free
intervals of 5 to 20 years or more, it is important to follow up with ANCA
tests to catch a relapse at its earliest and most treatable stage.