Wegeners Granulomatosis
The History
In 1931, Heinz Klinger, who worked at the University of Berlin, first reported patients
who had died with prolonged sepsis with inflammation of the blood vessels throughout the
body.
Approximately five years later Dr. Frederick Wegener found a distinct syndrome in three
patients. This being necrotizing (gradually degenerating) granulomas involving the upper
an lower respiratory tract. A further seven patients were described in 1954
resulting in a definite criteria for the diagnosis of a specific disease attributed with
the name, Wegener's Granulomatosis.
The Disease
Wegener's Granulomatosis (W.G.) is a very rare disease known as a form of vasculitis -
inflammation of the blood vessels. It is also an auto-immune disease meaning that it
attacks its own body tissues. There is no known cause for W.G. although it is known
not to be contagious or hereditary. It is a systemic condition, that is, it affects
the body as a whole, including all major organs, eyes, nose, throat and skin. People
without renal (kidney) involvement are said to have Limited Wegener's. The blood
vessels become inflamed and cell debris (granuloma) appears. If diagnosed early,
treatment can affect early remission and prevent organ failure.
Who gets W.G. and What Age Group?
Patients are divided equally between male and female and are of any age from 0 to
90+. However, it is a very rare disease.
Symptoms of W.G.
Patients show symptoms of a heavy cold or sinusitis which fails to respond to medication
and lasts considerably longer than normal. Nasal membrane ulceration and crusting in
the nose can also be evident.
Additional symptoms may include:
- Saddle nose deformity
- Heavy nose bleeds
- Cough with/without blood
- Pleuritis(inflammation of the lining of the lungs)
- Shortness of breath
- Skin eruptions
- Fever/Bad night sweats
- Lack of Energy/Lethargy
- Loss of Appetite
- Weight Loss
- Severe Joint Pains
- Blood in/colour change in, urine
IMPORTANT - PLEASE NOTE
All patients do not experience all of these symptoms. Please be aware that different
patients experience different symptoms depending on the severity of the disease.
Diagnosis
Diagnosis is established by a Blood Test known as ANCA and can be confirmed by biopsy.
Treatment
Treatment normally consist of a combination of drugs, mainly steroids, to suppress the
disease and these are used in varying degrees dependent on the severity of the disease.
The main drugs at present are Prednisolone which is an anti-inflammatory
drug and Cyclophosphamide, a drug originally used to treat cancer but now has been found
to be very effective for suppressing diseases of the immune system. Azathioprine is
used as an alternative to Cyclophosphamide in cases where bad side effects are
experienced.
Newer Regimes
Lower dose weekly methotrexate therapy, monthly intravenous or oral pulses of
Cyclophosphamide and high dose intravenous immunoglobulins are now being tried with
considerable success. Long term remission of intractable ANCA negative vasculitis has been
achieved with humanised monoclonal antibodies and this therapy has now been tried for
treating W.G. which otherwise had shown little response to conventional Cyclophosphamide
and steroid treatment, again with long term benefit being conferred for patients with
otherwise intractable disease.
In addition, Oxypentifylline has found a role for the treatment of
moderately severe vasculitis.
Side Effects
Steroids
- Weight gain/moon shaped face
- Thinning of the skin/bones
- Thrush in the mouth
- Collet Diabetes
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Cyclophosphamide
- Nausea/Sickness (in high doses)
- Reduced blood cells in bone marrow
- Thinning of the hair
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NOTE
The ANCA blood test will monitor the success of any treatment as it is sometimes difficult
to tell if symptoms are caused by the disease or the drugs used to treat it. |
Remission
As yet there is no cure for W.G. but with early diagnosis the correct treatment will be
effective in putting the disease into remission. However it is essential to maintain
regular ANCA Blood Tests to ensure the disease is under control.
Drugs are reduced very slowly under the watchful eye of your Consultant or
G.P. and you must remember to advise of any changes experienced as a patient so that any
modification of treatment can be actioned.
If you are unhappy with any of your dealings, please approach
The Stuart Strange Trust for additional information and support.
Acknowledgements
Dr. Wai Tse
The University of Birmingham Medical School
Prof. Caroline Savage
Senior Lecturer(Nephrology)
The University of Birmingham Medical School
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