Churg-Strauss Syndrome

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Churg-Strauss Syndrome is a disorder that causes inflammation in blood vessels, which restricts blood flow to various organs. Although the disease may involve any organ, most commonly it affects your lungs and skin. The restricted blood flow to these organs can cause temporary or permanent damage.

Asthma is a common feature of this disease, which is also known as allergic granulomatosis and allergic angiitis. Besides inflamed blood vessels, it produces a type of inflammatory response known as granuloma, which can destroy normal tissue.

Churg-Strauss syndrome is an uncommon disorder, and it affects males and females equally. The disease can occur at any age, but it's more commonly diagnosed in middle-aged people. People older than 65 are unlikely to develop this disease.

There's no cure, but, with medication, it's possible to achieve long periods with no signs or symptoms.

There are three stages of Churg-Strauss syndrome, each with its own signs and symptoms, depending on which organs are affected. This diseasee is progressive, but not everyone develops all three phases, and the phases don't always develop in order.

. . . Allergic
This usually is the first phase of the disease. In this phase, you may develop a number of allergic reactions, including:

  • Asthma. If you already have asthma, it may worsen and become harder to treat. Or you may develop late-onset asthma.
  • Hay fever. This affects the mucous membranes of your nose, causing runny nose, sneezing and itching.
  • Sinus pain and inflammation. You may experience facial pain and develop nasal polyps, which are soft, benign growths that develop as a result of chronic inflammation.
  • . . . Hypereosinophilia
    An eosinophil is a type of white blood cell that normally helps your immune system fight certain types of infections. In this phase, your body produces abnormally high numbers of eosinophils.

    The signs and symptoms of this phase depend on the area of your body that's affected. Overproduction of eosinophils in your lungs and overproduction of eosinophils in your digestive tract are common. During this phase, the signs and symptoms may worsen and improve. General signs and symptoms include:

  • Fever
  • Weight loss
  • Fatigue
  • Night sweats
  • Cough,
  • Abdominal pain
  • Gastrointestinal bleeding

  • . . . Systemic vasculitis
    With treatment in an earlier stage, you may not progress to this stage. The vasculitis commonly affects blood vessels in your skin, heart, lungs, central nervous system, muscles and bones, and gastrointestinal tract. It can also affect your kidneys. Signs and symptoms depend on which organs are affected. General signs and symptoms of this phase include:
  • General ill feeling
  • Swollen lymph nodes
  • Weight loss
  • Weakness and fatigue, possibly associated with low red blood cell count

  • Depending on which of your organs are affected, you may experience:
  • Rash or skin sores
  • Joint aches and swelling
  • Numbness and tingling in your hands and feet
  • Severe abdominal pain
  • Diarrhea, nausea and vomiting
  • Shortness of breath
  • Coughing up blood
  • Chest pain
  • Irregular heartbeat
  • Blood in your urine

  • Causes:
    Churg-Strauss syndrome is likely due to an overzealous immune system response. In an autoimmune reaction, white blood cells, which normally protect you from unwanted invaders, such as bacteria, viruses and allergens, attack healthy tissue, causing inflammation.

    There's no cure for Churg-Strauss syndrome, but some medications may help you achieve remission. Early diagnosis and treatment may prevent the more serious complications of the disease.

    Corticosteroids, such as prednisone, are most often prescribed. Your doctor may prescribe a high dose — 40 milligrams (mg) to 60 mg a day — of corticosteroids to get your disease into remission as soon as possible. However, high doses of corticosteroids can cause serious side effects, such as bone loss and high blood sugar. So after a month or so, your doctor may begin to decrease the dose gradually until you're taking the smallest amount that will keep your disease under control.

    For some people, a corticosteroid may be enough. However, other people require another immunosuppressive drug, such as cyclophosphamide (Cytoxan) azathioprine (Imuran) or methotrexate (Rheumatrex), to reduce the body's immune reaction.

    Because these drugs suppress your immune system and hamper your body's ability to fight off infection, your doctor must monitor your condition while you're taking them.

    Depending on which of your organs the disease affects, your doctor may recommend that you also see a specialist, such as a cardiologist if you have heart involvement.

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