Churg-Strauss syndrome

Description

Churg-Strauss syndrome is a disorder that is characterized by inflammation of the blood vessels. This inflammation can restrict blood flow to organs and tissues, sometimes permanently damaging them. This condition is also known as granulomatosis, eosinophilic with polyangiitis (EGPA).

Adult-onset asthma is the most common sign of Churg-Strauss syndrome. The disorder can also cause other problems, such as nasal allergies, sinus problems, skin rash, gastrointestinal bleeding, pain and numbness in the hands and feet.

Churg-Strauss syndrome is rare and has no cure. The symptoms can usually be controlled with the use of steroids and other potent immunosuppressive drugs.

Symptoms

Churg-Strauss syndrome varies greatly from person to person. Some people have only mild symptoms. Others have serious or life-threatening complications.

Also known as EGPA, the syndrome tends to occur in three stages and it gets progressively worse. Almost everyone with the condition of asthma, chronic sinusitis, and high counts of white blood cells called eosinophils.

Other signs and symptoms may include:

  • Loss of appetite and weight loss
  • Muscle and joint pain.
  • Abdominal pain and gastrointestinal bleeding
  • Weakness, fatigue or a general feeling of discomfort
  • Rashes or sores on the skin
  • Pain, numbness, and tingling in the hands and feet

When to see a doctor

Consult your doctor if you have shortness of breath or a runny nose that won't go away, especially if it is accompanied by the persistence of facial pain. Also consult with your doctor if you have asthma or nasal allergies that get worse suddenly.

Churg-Strauss syndrome is rare, and it is more likely that these symptoms have other causes. But it is important for your doctor to evaluate them. The early diagnosis and treatment to improve the chances of a good result.

Causes

The cause of Churg-Strauss syndrome is largely unknown. It is likely that a combination of genes and environmental factors, such as allergens or certain medications, causes hyperactivity of the immune system's response. In place to protect against the invasion of bacteria and viruses, the immune system of the objectives of healthy tissue, causing inflammation.

Risk factors

While anyone can get the Churg-Strauss syndrome, people are usually around 50 years of age when diagnosed. Other possible risk factors include chronic asthma or nasal problems. Genetics and exposure to environmental allergens may also play a role.

Complications

Churg-Strauss syndrome can affect many organs, including the lungs, sinuses, skin, gastrointestinal system, kidneys, muscles, joints and the heart. Without treatment, the disease can be fatal.

Complications depend on the organs involved, may include:

  • Damage to the peripheral nerves. Churg-Strauss syndrome can cause damage to the nerves in the hands and feet, causing numbness, burning, and loss of function.
  • Diseases of the heart. Heart-related complications of Churg-Strauss syndrome include inflammation of the membrane surrounding the heart, inflammation of the muscular layer of the heart wall, heart attack, and heart failure.
  • The kidney damage. If the Churg-Strauss syndrome affects the kidneys, may develop glomerulonephritis. This disease impairs your kidneys ability to filter, which leads to an accumulation of waste products in the blood.

Churg-Strauss syndrome

Diagnosis

To diagnose the Churg-Strauss syndrome, doctors usually order several types of tests, including:

  • Blood tests. A blood test can detect certain antibodies in the blood that may suggest but not confirm a diagnosis of Churg-Strauss syndrome. You can also measure the level of eosinophils, although other diseases, such as asthma, may increase the number of these cells.
  • Imaging tests. X-rays and ct scans can reveal abnormalities in the lungs and sinuses. If you develop symptoms of heart failure, your doctor may also suggest regular echocardiograms.
  • The biopsy of the affected tissue. If other evidence suggests Churg-Strauss syndrome, you could have a small sample of tissue is removed for examination under a microscope. The fabric can come from the lungs or other body, such as skin or muscle, to confirm or rule out the presence of vasculitis.

Treatment

There is No cure for the Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medicines can help control your symptoms.

Corticosteroids

Prednisone, which reduces inflammation, is the most commonly prescribed drug for the Churg-Strauss syndrome. Your doctor may prescribe a high dose of corticosteroids or an increase in their current dose of corticosteroids to obtain your symptoms under control quickly.

High doses of corticosteroids can cause serious side effects, so your doctor will reduce the dose gradually until you are taking the smallest quantity that will keep your disease under control. Even lower than the doses taken over long periods of time can cause side effects.

Side effects of corticosteroids include bone loss, high blood sugar, weight gain, cataracts, and hard-to-treat infections.

Other immunosuppressive medications

For people with mild symptoms, a corticosteroid alone may be sufficient. Other people may need to add another medication to help suppress the immune system.

Mepolizumab (Nucala) is currently the only medication approved by the Food and Drug Administration for the treatment of Churg-Strauss syndrome. However, depending on the severity of the disease and the organs involved, other drugs may be needed. Examples include:

  • Azathioprine (Azasan, Imuran)
  • Benralizumab (Fasenra)
  • Cyclophosphamide
  • Methotrexate (Trexall)
  • Rituximab (Rituxan)

Because these drugs affect the body's ability to fight the infection and may cause other serious side effects, your condition will be monitored closely while you are taking.

Self-care

The long-term treatment with corticosteroids may cause a number of side effects. You can minimize these problems by taking the following steps:

  • Protect your bones. Ask your doctor how much calcium and vitamin D you need in your diet, and discuss whether you should take supplements.
  • Exercise. The exercise can help you maintain a healthy weight, which is important when you are taking corticosteroid medications that can cause weight gain. Strength training and weight-bearing exercises, such as walking and jogging also help to improve the health of the bones.
  • Eat a healthy diet. Steroids can cause high blood sugar levels and, finally, type 2 diabetes. Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains.

Coping and support

Here are some tips for dealing with the Churg-Strauss syndrome:

  • Educate yourself. To learn more about the disease can help to prepare you to deal with complications or recurrences.
  • Build a support system. Family and friends can help a lot. You might also want to speak with a counselor or social worker, doctor who is familiar with the disease. Or you may find it helpful to talk with other people with the Churg-Strauss syndrome.

Preparing for your appointment

If you have common signs and symptoms of Churg-Strauss syndrome, make an appointment with your doctor. The early diagnosis and treatment significantly improve the prognosis of this condition.

You may be referred to a doctor who specializes in disorders that cause inflammation of the blood vessels (vasculitis), such as a rheumatologist or immunologist. Also you can see a pulmonologist from Churg-Strauss affects the respiratory tract.

Here's some information to help you prepare for your appointment.

What you can do

When making an appointment, ask if you need to do anything in advance, such as restrict your diet. Also, ask if you need to stay in the doctor's office for observation after their tests.

Make a list of:

  • Your symptoms and when they started, even those that seem unrelated to Churg-Strauss syndrome
  • Key medical information, including other conditions with which you have been diagnosed
  • All the drugs, vitamins, and other supplements that you take, including over-dose
  • Questions to ask your doctor

If you've seen other doctors for his condition, bring a letter summarizing their findings and copies of recent X-rays of the chest or breast of x-rays. Have a family member or a friend to help you remember the information that you receive.

Basic questions to ask your doctor include:

  • What is the most likely cause of my condition?
  • What are other possible causes?
  • What diagnostic tests do I need?
  • What treatment do you recommend it?
  • What changes can I make to help control or reduce the symptoms?
  • How often will you see me for follow-up tests?

What to expect from your doctor

A doctor attends to a possible Churg-Strauss syndrome is likely to ask you questions, such as:

  • The symptoms, especially those related to asthma, worsened over time?
  • Do your symptoms include shortness of breath or wheezing?
  • Do your symptoms include sinus problems?
  • Do your symptoms include gastrointestinal problems such as nausea, vomiting, or diarrhea?
  • Have you ever had numbness, pain or weakness in an arm or a leg?
  • You have lost weight without trying?
  • Have you been diagnosed with any other medical condition, including allergies or asthma? If so, how long have you had it?
Symptoms and treatment of Churg-Strauss syndrome