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Churg-Strauss syndrome (CSS)    

What is the cause of Churg-Strauss syndrome?
What organs are affected by Churg-Strauss syndrome?
How is the diagnosis of Churg-Strauss syndrome made?
What is the treatment of Churg-Strauss syndrome?
What is the prognosis for Churg-Strauss syndrome?

Churg-Strauss syndrome is a small-vessel vasculitis resulting in asthma, increased eosinophils (a type of white cell) in the blood and inflammation of the vessels. Drs. Jacob Churg and Lotte Strauss at the New York Mount Sinai Hospital first described it in 1951. CSS belongs to a family of small-vessel vasculitides called ANCA-associated vasculitides. These include granulomatosis with polyangiitis, microscopic angiitis and Churg-Strauss syndrome.

What is the cause of Churg-Strauss syndrome?

CSS is an autoimmune condition caused by the overproduction of ANCA antibodies by the immune system. What triggers the immune system to make these antibodies and attack the vessels remains unknown. However, researchers agree that it is a combination of a genetic susceptibility and an environmental factor. Some medications like Montelukast (an asthma medication) or illicit drugs like cocaine have been shown to cause CSS in susceptible patients. 

What organs are affected by Churg-Strauss syndrome?

The first stage of the disease usually consists of new-onset or worsening of sinus disease and allergies. In the second stage, late-onset asthma develops. Finally, in the third stage other organs become involved.

CSS may affect multiple organs:

  • Rash of red spots, skin bumps or nodules (leucocytoclastic vasculitis)
  • Lacy violaceous deep rash (livedo reticularis)
  • Asthma
  • Allergies
  • Anemia
  • Weakness or numbness of extremities due to nerve damage
  • Headaches or seizures (rarely)
  • Joint pain and inflammation
  • Kidney inflammation (and failure if not treated)
  • Heart rhythm abnormalities or failure
  • Abdominal pain, fever, diarrhea and blood in the stool

How is the diagnosis of Churg-Strauss syndrome made?

Patient history, physical exam and laboratory testing usually make the diagnosis of CSS. The doctor will check the level and type of ANCA antibodies, test blood and urine, and obtain chest X-rays and CAT scans. Often a biopsy (a piece of tissue) is necessary to establish the diagnosis prior to using immunosuppressive medications. The organs that are often biopsied are the lungs, skin, kidneys and nerves. Even though a biopsy is an invasive procedure, with recent technologies it is relatively safe and carries minimal risks for patients.

What is the treatment of Churg-Strauss syndrome?

CSS is treated by a class of drugs called immunosuppressives. These medications suppress the immune system and prevent it from further attacking the body and causing damage. The drugs that are often used for the treatment of CSS are: corticosteroids (prednisone or methylprednisolone), cyclophosphamide (Cytoxan), azathioprine (Imuran), methotrexate (Methotrex) and mycophenolate mofetil (Cellcept). Other medications like inhalers and airway dilaters may be required to control the asthma. These medications require constant monitoring of the blood and urine to prevent toxicity.

The treatment of CSS depends on the severity of the disease. If asthma is the predominant manifestation, then corticosteroids and inhalers may be the only required treatment. In case of other organ involvement, treatment will consist of two phases: the “induction phase” and the “maintenance phase.” During the induction phase, strong medicines like corticosteroids and Cytoxan (pills or intravenous) are administered to completely control the disease. The induction phase may last three to six months. Later, during the maintenance phase, less-toxic drugs like methotrexate or Imuran are used to maintain disease control. This phase may last up to two years or longer.

What is the prognosis for Churg-Strauss syndrome?

With recent advances in treatment, the current prognosis for CSS patients is considered to be good. Remission of the disease is usually attained in the majority of cases. The sooner the diagnosis is made and the less vital organs involved, the better the outcome is.

Medication toxicity remains a major concern in Churg-Strauss syndrome. Therefore, it is very important that patients get regular check-ups, be vaccinated and be constantly monitored for any infection or side effects of therapy. All patients affected by Churg-Strauss syndrome should be under the supervision of a skilled health team that is familiar with these conditions, their treatment and the side effects of immunosuppressive medications. 

Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
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Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612

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