What is Churg-Strauss Syndrome?
This disease was first described by Dr. Churg and Or. Strauss in 1951, and is characterised by asthma, an increased eosinophil (a type of white blood cell) count in the blood and vasculitis (inflani,mation) of the blood vessels. Eosinophils are also present in greater numbers in affected tissue.
Who are affected?
Churg-Strauss Syndrome predominantly 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.
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 affecting discrete areas of their arms or legs. Less often, nerves in the head can be affected, and patients may notice hearing loss or blurred 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 fi Iteri ng 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. There are links with a predisposition to allergic disorders such as hay fever and asthma.
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 organ, such as the kidneys or lungs may show blood vessel inflammation, and accumulations of eosinophils and other blood cells.
Treatment
High dose steroids, either given as a tablet 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 practitioners 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.