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Churg-Strauss syndrome

Churg-Strauss syndrome — also known as eosinophilic granulomatosis (gran-u-loe-muh-TOE-sis) with polyangiitis (pol-e-an-jee-I-tis) — is a disorder marked by blood vessel inflammation. This inflammation can restrict blood flow to vital organs and tissues, sometimes permanently damaging them.

Asthma is the most common sign of Churg-Strauss syndrome, but Churg-Strauss syndrome can cause a variety of problems, ranging from hay fever, rash and gastrointestinal bleeding to severe pain and numbness in your hands and feet. The wide range of signs and symptoms and their similarity to those of other disorders makes Churg-Strauss syndrome challenging to diagnose.

Churg-Strauss syndrome is rare and has no cure. But, your doctor can usually help you control symptoms with steroids and other powerful immunosuppressant drugs.

Symptoms Causes Risk factors Complications

Churg-Strauss syndrome, which used to be known as allergic granulomatosis and allergic granulomatous angiitis, is a highly variable illness. Some people have only mild symptoms, whereas others experience severe or life-threatening complications. There are three stages, or phases, of Churg-Strauss syndrome, each with its own signs and symptoms, but not everyone develops all three phases or in the same order. This is especially true when the disease is caught and treated before the most serious damage occurs.

Churg-Strauss stages may include:

Allergic stage

This is usually the first stage of Churg-Strauss syndrome. It's marked by a number of allergic reactions, including:

  • Asthma. The primary sign of Churg-Strauss syndrome, asthma develops on average three to nine years before other signs and symptoms appear. In people with Churg-Strauss syndrome who already have asthma, symptoms usually become worse and may require steroids for control. Other people develop what is known as late-onset asthma. Developing asthma, even later in life, doesn't necessarily mean that you have Churg-Strauss syndrome, however.
  • Hay fever (allergic rhinitis). This affects the mucous membranes of your nose, causing runny nose, sneezing and itching.
  • Sinus pain and inflammation (sinusitis). You may experience facial pain and develop nasal polyps, which are soft, noncancerous (benign) growths that develop as a result of chronic inflammation.

Eosinophilic stage

An eosinophil is a type of white blood cell that helps your immune system fight certain infections. Normally, eosinophils make up only a small percentage of white blood cells, but in Churg-Strauss syndrome, abnormally high numbers of these cells (hypereosinophilia) are found in your blood or tissues, where they can cause serious damage.

Signs and symptoms of hypereosinophilia depend on which part of your body is affected. Your lungs and digestive tract — including your stomach and esophagus — are involved most often.

Broadly speaking, signs and symptoms of the hypereosinophilia phase may include:

  • Fever
  • Loss of appetite
  • Weight loss
  • Asthma
  • Fatigue
  • Cough
  • Abdominal pain
  • Gastrointestinal bleeding

Vasculitic stage

The hallmark of this stage of Churg-Strauss syndrome is severe blood vessel inflammation (vasculitis). By narrowing blood vessels, inflammation reduces blood flow to vital organs and tissues throughout your body, including your skin, heart, peripheral nervous system, muscles, bones and digestive tract. Occasionally, your kidneys may also be affected.

During this phase, you may feel generally unwell and have unintended weight loss, swollen lymph nodes, weakness and fatigue.

Depending on which organs are affected, you may also experience:

  • Rash or skin sores
  • Joint aches and swelling
  • Severe pain, numbness and tingling in your hands and feet (peripheral neuropathy)
  • Severe abdominal pain
  • Diarrhea, nausea and vomiting
  • Shortness of breath (dyspnea) from asthma or congestive heart failure
  • Coughing up blood (hemoptysis)
  • Chest pain
  • Irregular heartbeat
  • Blood in your urine (hematuria)

When to see a doctor

See your doctor anytime you develop signs and symptoms such as breathing difficulties or a runny nose that doesn't go away, especially if it's accompanied by persistent facial pain. Also see your doctor if you have asthma or hay fever that suddenly worsens. Churg-Strauss syndrome is rare, and it's more likely that these symptoms have some other cause, but it's important that your doctor evaluate them.

The exact cause of Churg-Strauss syndrome is unknown. It's likely that an overactive immune system response is triggered by a combination of genes and an environmental trigger, such as allergens or certain medications. Instead of simply protecting against invading organisms such as bacteria and viruses, the immune system overreacts and targets healthy tissue, causing widespread inflammation.

Some people have developed Churg-Strauss syndrome after using an asthma and allergy medication called montelukast or after switching from low-dose oral systemic steroids to inhaled steroid medications. However, no clear connection between Churg-Strauss syndrome and any medication has been proved.

Churg-Strauss syndrome is rare, affecting as few as 1 to 3 people per million.

Possible risk factors for Churg-Strauss syndrome include:

  • Age. On average, people with Churg-Strauss syndrome are diagnosed in their mid- to late 40s.
  • History of asthma or nasal problems. Most people diagnosed with Churg-Strauss syndrome have a history of nasal allergies, chronic sinusitis or asthma, which is often severe or hard to control. It's important to note that while almost everyone with Churg-Strauss syndrome has asthma or allergies, most people with those conditions will never develop Churg-Strauss syndrome.

Churg-Strauss syndrome can affect many organs, including your lungs, skin, gastrointestinal system, kidneys, muscles, joints and heart. Without treatment, the disease may be fatal. Complications depend on the organs involved and may include:

  • Peripheral nerve damage. Peripheral nerves extend throughout your body, connecting your organs, glands, muscles and skin with your spinal cord and brain. Churg-Strauss syndrome can damage peripheral nerves (peripheral neuropathy), especially those in your hands and feet, leading to numbness, burning and loss of function. Peripheral nerve problems often improve with treatment.
  • Skin scarring. The inflammation may cause sores to develop that can leave scars.
  • Heart disease. Heart-related complications of Churg-Strauss syndrome include inflammation of the membrane surrounding your heart (pericarditis), inflammation of the muscular layer of your heart wall (myocarditis), heart attack and heart failure.
  • Kidney (renal) damage. If Churg-Strauss syndrome affects your kidneys, you may develop glomerulonephritis, a type of kidney disease that hampers your kidneys' filtering ability, leading to a buildup of waste products in your bloodstream (uremia). Kidney failure is uncommon.
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