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Churg-Strauss Syndrome (CSS) – Symptoms, Causes, Treatment

What is Churg-Strauss Syndrome?

One uncommon type of vasculitis is Churg-Strauss syndrome or CSS. This is also called as allergic granulomatous angiitis. This is a condition wherein blood vessels both arteries and veins are inflamed. Because of the inflammation, blood flow in the vital organs is restricted causing damage to these organs. It was first described by Dr. Jacob Churg and Dr. Lotte Strauss in 1951 wherein the characteristics of the syndrome are composed of asthma, eosinophilia or an increased number of eosinophils, fever and vasculitis.

The Churg-Strauss syndrome, Wegener granulomatosis and the periarteritis are the three diseases that have been closely related to each other. This is because it affects the medium and small-sized vessels in the body.

Churg-Strauss syndrome has 3 stage where in the first stage, it involves the sinuses and the onset of allergies which the person did not have or the previous allergies worsen. The second stage is involves acute asthma. The last stage involves other organs and this happens to be the most life threatening one. It also becomes painful because the patient develops pain in the legs, arms and hands.

According to statistics, Churg-Strauss syndrome slightly affects more males than females and on the average; it affects adults in their late 30’s usually at 38. However, the syndrome can happen from ages 15-70. In the United States, there are about 1-3 cases per 100,000 adults every year. Internationally about 2.5 cases per 100,000 every year are affected. When diagnosed with Churg-Strauss syndrome, its mortality is mostly because of myocarditis and myocardial infarction. But with proper and religious treatment, 90% survival rate is accounted for a year and the 5-year survival rate is at 62%.

Churg-Strauss Syndrome Symptoms

In this syndrome, there are 3 phases where the patient should identify the signs and symptoms.

1st stage (allergic stage)

In this stage, the patient will experience the following:

  • Asthma
  • Hay fever or allergic rhinitis which includes colds or runny nose, sneezing and itching
  • Sinus pain or sinusitis

2nd stage (Eosinophilic stage)

In this stage, there is a marked increase in the eosinophils, a type of white blood cells which is very helpful in the immune system. Normally, in blood counts, this only makes up a small percentage but with Churg-Strauss syndrome, it is quite high. This usually affects the stomach and esophagus and other symptoms include:

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

3rd stage (vasculitic stage)

This is the hallmark stage for the conditions because of the inflamed blood vessels or vasculitis. The patient will experience the following:

  • General weakness
  • Rashes or skin sores
  • Severe abdominal pain
  • Diarrhea with nausea and vomiting
  • Hemoptysis or coughing up of blood
  • Chest pain
  • Shortness of breath
  • Joint pains and swelling
  • Numbness and tingling sensation of hands and feet.
  • Blood in the urine or hematuria

Churg-Strauss Syndrome Causes

The exact cause of Churg-Strauss syndrome is still unknown. Doctors pointed out that allergies, autoimmune reactions, environmental agents and even drugs may trigger the condition.

Churg-Strauss Syndrome Treatment

Blood tests are taken to check for the immune system. in Churg-Strauss syndrome, proteins called autoantibodies are formed and eosinophils are also measured. Normally, eosinophils are elevated in this condition. aside from that, imaging tests are done like x-rays, CT scans and even magnetic resonance imaging.

Aside from that, the American College of Rheumatology or (ACR) proposed 6 criteria for diagnosing Churg-Strauss syndrome. And the presence of 4 criteria with a sensitivity of 85% and 99.7% is enough to diagnose the problem. the criteria include:

  • Asthma
  • Eosinophilia of more than 10% in the peripheral blood
  • Paranasal sinusitis
  • Pulmonary infiltrates
  • Histological proof of vasculitis
  • Mononeuritis multiplex or polyneuropathy

Churg-Strauss Syndrome Medications

Once diagnosed with Churg-Strauss syndrome, doctors would prescribe the following medications for treatment:


This is to help minimize the symptoms of the condition. Prednisone is the most common drug of choice but doctors can prescribe other steroids of a higher dose. However the problem with high dose corticosteroids is that it can lead to serious side effects. It includes bone loss, high blood sugar, cataracts and even infections that are hard to treat.

Immunosuppressive drugs

Drugs like Cytoxan, Azathioprine, Methotrexate are the common immunosuppressant drugs. This is to prevent the immune system to attack the body. patients taking immunosuppressant drugs impairs the body’s ability to fight off infections because the immune system is suppressed. So it is important to avoid infections.

Intravenous immunoglobulins or IVIg

This is given in IV infusions and this is usually given to patients who don’t respond to other medications. This is not the first line of medications and this drug is very expensive. When this is given, patients are expected to experience flu-like symptoms in a day or 2.

Aside from medications, there are other lifestyle modifications to be done in patients with Churg-Strauss syndrome. This includes:

  • Stop smoking
  • Protecting the bones – Because of corticosteroids, it can greatly affect the bones. With that, it is important to take supplements like vitamin D. it helps increase the calcium absorption and increase the muscle strength. Experts would recommend taking up to 2,000 international units (IU) per day.
  • Exercise – Since corticosteroids can lead to gain weight and increase blood sugar, regular exercise is very important.
  • Healthy diet – Long time use of corticosteroids can lead to full-blown diabetes. With that, patients should keep their blood sugar at a normal level. They should eat foods with whole grain and wheat. Lean meat with lots of vegetables and fruits are also required rather than eating a lot of desserts like pastries. Sodas should also be minimized and they should hydrate with water instead.
  • Keeping up with appointments – It is very important to keep up with appointments from the doctor so that they can monitor the patient’s improvement. They should also have regular check-up to check on their blood sugar, do bone scans, check their blood pressure and involvement off other vital organs like the heart and the kidneys.

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