What is Churg-Strauss syndrome?
Churg-Strauss syndrome is a rare disease characterised by generalised inflammation of small to medium blood vessels in the body. The syndrome was described by 2 doctors – Churg and Strauss – in 1951.
Increased levels of certain cells in the blood (eosinophils) are seen. The etiology of the syndrome remains unknown and it is thought to be autoimmune in nature – meaning that antibodies are produced against one’s own body cells. The organs that are usually affected are
- Lungs – asthma, sinusitis and rhinitis
- Joints – arthritis
- Skin – nodules and rash
- Heart – myocarditis (inflammation of hear muscle), pericarditis (inflammation of covering of the heart), involvement of coronary arteries (arteries which supply blood to heart muscle)
- Bowels – inflammation of stomach and intestines presenting as diarrhoea, tummy pain, passing blood in stools
- Inflammation of nerves
- Kidneys
Age at onset
Varies from 15-70 years, with mean age being third to fourth decade.
Sex
Males are affected slightly more frequently than females
Symptoms related to the heart are
- Shortness of breath
- Chest pain
- Palpitations (awareness of ones own heart beat)
- Syncope
- Swelling of legs due to collection of water
- Very rarely can present as sudden cardiac death due to rhythm disturbances or even myocardial infarction (heart attack)
The most common cause of death is from heart involvement in the form of myocardial infarction or heart failure.
Treatment
The mainstay of treatment is use of steroids, either orally as tablets or as injections into veins depending on the severity of the condition. In most cases steroids are adequate, but in complicated cases and in patients who do not respond to steroids, more powerful and toxic drugs are used like cyclophosphamide.
Patients should be under the care of specialists depending on the organs involved – e.g. cardiologist when the heart is involved
