Churg-Strauss Syndrome

Churg-Strauss syndrome, now known as eosinophilic granulomatosis with polyangiitis, is a rare autoimmune disorder characterized by inflammation of blood vessels.

It often affects small-sized blood vessels like arteries, arterioles, and venules.

All patients with Churg-Strauss syndrome have history of asthma or allergy. Inflammation of blood vessels injures organs like lungs, sinuses, joints, nerves, and intestine.

In some patients it affects heart and kidneys too. Fatigue, weight loss, and weakness are common symptoms of this syndrome.

Suppressing the immune system and controlling inflammation are the treatment strategies for Churg-Strauss syndrome.

Symptoms of Churg-Strauss syndrome range from mild to serious, life-threatening conditions. Disease progresses in three stages – allergic stage, eosinophilic stage, and inflammatory or vasculitis stage. The three stages may occur in different order and may vary from individual to individual.

Allergic stage is characterized by allergic responses including asthma, hay fever, and sinusitis. Asthma is considered to be the first sign of this syndrome. In people who have a history of asthma, symptoms of asthma worsens and steroids are required to control the same. Hay-fever results in running nose and sneezing. Chronic inflammation of the nasal cavity may lead to the growth of nasal polyps. Sinusitis is characterized by pain and inflammation in the sinus cavities.

Churg-Strauss syndrome is characterized by high levels of eosinophils. Increased levels of eosinophils affect different organs. Signs and symptoms in this stage depend on the organ affected and the extent of damage. Most common symptoms of eosinophilic stage are loss of appetite, loss of weight, abdominal pain, fatigue, fever, intestinal bleeding, and cough.

Inflammation of small-sized blood vessel is the characteristic feature of vasculitic stage. Inflammation reduces blood flow to different organs like skin, heart, nerves, muscles, joints, and digestive tract. Unexplained weight loss, swelling in lymph nodes, weakness, and fatigue are the most common symptoms of this stage.

Some other symptoms include:

• Abdominal pain
• Numbness and tingling of hands and feet
• Joint pain
• Diarrhea, nausea, and vomiting
• Irregular heart beat
• Difficulty in breathing
• Blood in urea
• Chest pain
• Skin rashes
• Blood in cough

The definitive cause of Churg-Strauss syndrome is not known. It is associated with an enhanced response of the body’s immune system. Many factors may be involved in triggering the immune response.

Some of the factors implied in activating the immune system include genetic factors, environmental triggers, allergens, and certain medications. The hyperactivity of the immune system produces responses that target the healthy tissues, which leads to inflammation.

Churg-Strauss syndrome is associated with asthma medications like leukotriene modifiers. It is more commonly found in people above 45 years. History of asthma and allergic conditions also increase the risk of developing Churg-Strauss syndrome.

Almost all people with this disease have a history of chronic asthma, nasal allergies, or sinusitis. Although all patients with this disorder have a history of asthma, not all patients with asthma will develop Churg-Strauss syndrome.

Diagnosis of Churg-Strauss syndrome is based on signs and symptoms, organ involvement, and blood tests. The criteria for identifying Churg-Strauss syndrome is given by American College of Rheumatology.

A person is considered to be having this syndrome if he/she has four or more of the following criteria:

• Asthma – most of the people have a history of chronic asthma
• Eosinophilia – high levels of eosinophils, to the order of >10 %, is characteristic of this syndrome
• Neuropathy – peripheral neuropathy resulting in numbness and tingling of the fingers and toes is also found in Churg-Strauss syndrome
• Lesions revealed by X-ray – Lesions resembling that in pneumonia can be noted in the X-ray
• Sinus problems – Most of the patients have a history of sinusitis
• Eosinophils outside the blood vessels – eosinophils may be present outside the blood vessels

Blood tests detect autoantibodies that attack one’s own cells. It also reveals the level of eosinophils in blood. Imaging techniques are used to check out for abnormalities in organs like lungs and sinuses. Skin biopsy confirms inflammation of the blood vessels.

There is no complete cure for Churg-Strauss syndrome and treatment focuses on relieving the symptoms which range from mild to serious.

Early diagnosis and treatment is crucial in reducing the risk of complications. Symptoms are controlled by corticosteroids, the dose of which is based on the extent of damage.

Immunosuppressive drugs like cyclophosphamide, azathioprine, and methotrexate help in reducing the immune response. Immunoglobulins are recommended for people who do not respond to the conventional treatment methods or medications.

Biologic medications like rituximab are also used to reduce the response of immune system. It also helps to reduce the levels of eosinophils.

There are no known ways to prevent Churg-Strauss syndrome as the cause of the disease is not clear. 

A few alternative and homeopathic remedies exist for Churg-Strauss syndrome.

Anti-inflammatory diet that eliminates certain food like milk and milk products, polyunsaturated vegetable oils, and partially hydrogenated oils is suggested for controlling vasculitis.

Anti-inflammatory herbs like ginger and turmeric also help to control inflammation of blood vessels.

Grape seed extract is used to provide protection to blood vessels. Some other methods like hypnosis, psychotherapy, biofeed back and traditional Chinese medicine are also suggested in the treatment of this syndrome.

Healthy diet, regular exercise, and developing a healthy lifestyle help to minimize the symptoms of the disease.

Understand more about the disease and treatment options, and talk to a counsellor or a social worker regarding the same.

Have a good support system at home, including family and friends to cope with this serious disease.

Complications of Churg-Strauss syndrome depend on the organ affected and the extent of damage.