Churg-Strauss syndrome (CSS), also known as Eosinophilic Granulomatosis with polyangiitis is a form of vasculitis characterised mainly by inflammation of the smaller blood vessels such as arteries, arterioles and venules.
This form of vasculitis (CSS) particularly affects in patients with a history of severe asthma and allergy. The blood vessels of the lungs, skin and abdomen are usually affected. A severe form of CSS can result in the cutting off of the blood supply to the vital organs and tissues.
CAUSES OF CSS
Churg-Strauss syndrome is a rare disorder and its causes are relatively unknown. However, they involve an abnormally overactive immune system coupled with pre-existing lung disease. CSS Syndrome can affect people of all ages, but predominantly figures among people aged between 15 to 70 years, although patients as young as 9 years have been reported with Churg-Strauss. Men are found to get affected slightly more than women.
SYMPTOMS OF CSS
The CSS disorder usually manifests in three stages; in the allergic stage, blood vessels get inflamed and the patient suffers from allergic asthmatic attacks or rhinitis. If already suffering from asthma, the condition may get aggravated with the onset of churg-strauss vasculitis. For others, it could be a first-time attack. Occasional cases of sinusitis are also reported during this stage.
The second stage of churg-strauss vasculitis is marked by hypereosinophilia or an abnormally high level of eosinophils (white blood cells) in the blood, which often bring about tissue damage. The eosinophils form clusters and release harmful granules that form lesions in different parts of the body. This is known as granulomatosis.
The third stage of CSS Syndrome culminates in severe vasculitis or inflammation of blood vessels, leading to reduced blood supply to the organs. Thrombosis or formation of blood clots in arteries occurs particularly in the abdominal region. This eventually results in necrosis, or death of cells and tissue, a condition that is life-threatening.
More serious complications like cardiac and renal disorders due to Churg-Strauss syndrome, also occur in the vasculitis stage.
DIAGNOSIS OF CSS
Churg-Strauss syndrome is a rare disorder that is difficult to diagonise, in most cases. It can vary in severity from patient to patient. In some people, it comes in the milder form, while in others it manifests in its severe form, bringing about major organ complications. Regrettably, churg-strauss vasculitis goes unnoticed in its early stages.
Late onset of asthma or a worsening asthmatic condition accompanied with pain and numbness of extremities, sinus issues, prolonged cough and abdominal pain, are all indications of the onset of the CSS syndrome.
The American College of Rheumatology has listed criteria for diagnosing the syndrome. Asthma, Eosinophilia, Neuropathy, mono or poly Pulmonary infiltrates, non-fixed Paranasal sinus abnormality and extravascular Eosinophils are the criteria out of which, the presence of at least four of the above confirms the onset of churg strauss vasculitis.
Lab tests of blood, kidney functioning, urine as well as chest X-Ray and CT Scan are carried out to look for signs and symptoms of the Churg-Strauss syndrome.
TREATING CHURG STRAUSS SYNDROME
This disorder is treated by prescribing glucocorticoids and similar immunosuppressive drugs. These work on toning down the inflammation of the blood vessels and also suppress the overactive immune system. Cycotoxic drugs are provided to those who fail to positively respond to steroids.
Churg-strauss syndrome currently has no cure; hence, treatment mandates careful monitoring and periodic testing, as remissions can occur frequently. Patients also need to be closely watched for drug tolerance and the side effects.
Any new symptoms should be promptly reported for timely action. Churg-Strauss vasculitis is a chronic disorder and can be life threatening, but with a well-monitored and effective medical treatment, the lives of patients can be made easier and less painful.
There are many well-established vasculitis centers across the globe. The basic aim of such centres is to raise awareness among people about this rare disorder (CSS), and help them cope with it in a better way.
Knowing facts about the disorder and understanding it gives the patient control over their ailment. Explaining in simple, easy to understand language instead of heavy medical jargon goes a long way in conveying some positivity in newly diagnosed patients.
Popular vasculitis centers rope in the best doctors to form a supportive and qualified medical team to diagnose, test, monitor and prescribe the most suited treatment for individuals affected with this syndrome.
In addition to the medical expert panel, people with vasculitis are also part of the interactive sessions, where they talk about their experiences and opinions, which can be of great help to allay fears of new patients diagnosed with Churg Strauss Syndrome.
A strong, dedicated team functioning in a good vasculitis centers usually comprises a team of qualified doctors, nurses, patient care coordinators and assistants to help in the correct diagnosis, care, treatment and follow up of strauss vasculitis-affected patients.
Many vasculitis centers also involve anxious members of the immediate families of patients to extend guided care across to patients’ homes.
Apart from individual patient care, interactive group sessions, talks and seminars, these centres also arrange for community programmes to increase awareness about the disorder. Potential Churg-Strauss syndrome patients can be alerted, identified and treated in the initial stages so as to provide maximum relief. Many community programmes involve patients in fun activities where they can display their inherent skills. This way, the patients learn to stay positive and have a quality life, instead of sulking about the disorder.
Vasculitis centres also focus on research activities to develop new strategies to tackle the disorder, and advance to newer and better diagnostic and therapeutic choices.