Churg-Strauss Syndrome
What is it?
This disease was first described by Churg and Strauss in 1951, and is characterised by
asthma, an increased eosinophil ( a type of white blood cell) count in the blood and
vasculitis (inflammation) of the blood vessels.
Who Are Affected?
CSS predominately affects patients 30-45 years of age, either male or female.
What Are The Symptoms?
Non-specific symptoms are often found and may include fever, myalgia, arthralgia, fatigue,
red eyes, skin rash, loss of appetite and weight loss.
Specifically, involvement of the lungs is typically seen and patients may
complain of breathlessness due to direct lung involvement by the disease or asthma, a
major feature of CSS. It is worth emphasising that most patients with asthma do not
have and will never develop CSS.
Other symptoms may be present due to blood vessel inflammation leading to
poor blood supply. Inflammation of vessels supplying nerves are common and
more than 60% of patients will notice weakness, or numbness of their arms or legs.
Less often, nerves in the head can be affected, and patients may notice hearing loss or
blurring vision. Other manifestations include high blood pressure, heart failure and
abdominal pain.
Kidney involvement is present in about 40% of patients. The kidneys
are responsible for filtering the blood and removing excess fluid and poisonous
substances. The kidneys have a tremendous functional reserve, and symptoms only appear
when poisonous substances have accumulated in the blood stream. Symptoms are
non-specific and consist of such maladies as fatigue, loss of appetite, nausea, vomiting,
swelling of the legs; but when function is severely diminished, alterations in mental
status and seizures can occur.
Aetiology/Cause
Essentially unknown.
Diagnosis
Urea and creatinine are metabolic waste products which accumulate in the blood when the
kidney function declines, therefore levels of these molecules are useful measurements of
kidney function. The eosinophil count in the blood is usually raised and this can be
measured by a simple blood test. Anti-neutrophil cytoplasmic antibodies (ANCA) are
present in the blood in 50% of patients and can be detected by a blood test.
Biopsy of an involved organ, such as the kidney or lungs may show blood
vessel inflammation, and accumulations of eosinophils and other blood cells.
Treatment
High dose steroids, either given as tablets or injections, are usually administered in
conjunction with other drugs which modify the immune system. The drugs most commonly
used are called prednisolone, azathioprine and cyclophosphamide and work by suppressing
the immune system.
Does The Treatment Have Adverse Effects?
The drugs used modify the immune system, so patients taking these drugs are also more
susceptible to having infections. It is therefore very important that at the
earliest signs of infection, patients should seek medical attention either with their
general practioners or their attending hospital physicians. Steroids can also cause
or aggravate bone thinning, indigestion, sugar diabetes, high blood pressure, thinning and
bruising of the skin and weight gain. Patients will be regularly reviewed and
treatment reduced as quickly as possible to reduce side effects.
Can This Condition Relapse?
Uncommon, but can occur.
Prognosis
Good, with correct early diagnosis and treatment.
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