What is Churg-Strauss syndrome (CSS)

Churg-Strauss syndrome (CSS), or allergic granulomatous angiitis, is a rare disease that affects small to medium-sized arteries and veins.

It is a disease affecting multiple body sites and characterised by the following:

  • Presence of Allergic Rhinitis (hay fever)
  • Asthma
  • Increased eosinophils in the blood (eosinophil is one of the white blood cells in the body)

As it is a multiorgan disease, it can affect any part of the body. However, the commonest site involved is the lung. Other organs involved include the gut, heart, skin, nerve and joints.

Statistics on Churg-Strauss syndrome (CSS)

Little information is available concerning variation in the prevalence or incidence of Churg-Strauss syndrome. This is partly due to diagnosis-related uncertainties.

CSS does not have gender preference, therefore men is affected as do women. The average age of diagnosis remains at 50 years old. It is uncommon in people older than 65 years old.

Risk Factors for Churg-Strauss syndrome (CSS)

Causes of CSS are unknown. CSS possibly is an allergic or autoimmune reaction to an environmental agent or drug.

Progression of Churg-Strauss syndrome (CSS)

CSS is most likely due to an autoimmune process. This means that the antibodies in the body is targeting and destroying the body’s own tissue. The cause of such change in still unknown.

CSS consists of 3 phases:

  • Prodromal phase: this is characterized by Allergic Rhinitis, Asthma and atopic diseases.
  • Eosinophilic phase: this is characterized by rising blood level of eosinophils, which is a type of white blood cells responsible for certain type of allergic reaction. Such reaction can lead to inflammation in the lung or gut.
  • Vasculitic phase: this is characterized by inflammation of the blood vessels. It usually develops within 3 years of the onset of asthma, although it may be delayed for several decades. This may be accompanied by non-specific symptoms such as fever, weight loss, general malaise and lassitude.

Churg-Strauss syndrome is a serious disease that can be fatal. Untreated it is extremely dangerous and threatens the organs that are affected. With aggressive treatment and monitoring it can be controlled fairly well. Total inactivation of the disease (remission) is possible as well.

How is Churg-Strauss syndrome (CSS) Diagnosed?

Blood Examination generally shows elevated levels of a type of white blood cell called eosinophil. Also there is increase of other white blood cells. Kidney function tests and urine test can be abnormal when the kidneys are affected (which is not common).

Prognosis of Churg-Strauss syndrome (CSS)

The prognosis of patients is unclear but treatment has significantly decreased death rate in patients. Before the use of steroids, 50% of untreated patients die within 3 months of the onset of vasculitis. Currently recent clinical studies suggest a survival rate of greater than 70% at 5 years, this could be due to advance of treatment modalities available for patients.

How is Churg-Strauss syndrome (CSS) Treated?

The treatment of patients with Churg-Strauss syndrome is directed toward both immediately reducing the inflammation of the blood vessels (vasculitis) and suppressing the immune system (because it is the body’s own immune system that is attacking the cells). Treatment usually includes high doses of corticosteroids medication (such as prednisone or prednisolone) to suppress the inflammation and suppression of the active immune system with cyclophosphamide (Cytoxan).

If the patient is unresponsive to the above treatment schedule or if the disease is severe, other treatment may be used.

Churg-Strauss syndrome (CSS) References

  1. Churg J, Strauss L: Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951; 27: 277-94.
  2. eMedicine: Churg-Strauss Syndrome [online]. 2004. [Cited 2005 October 14th]. Available from: URL: http://www.emedicine.com/med/topic2926.htm
  3. Green RL, Vayonis AG: Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment. Lancet 1999 Feb 27; 353(9154): 725-6.
  4. Up to Date: Churg-Strauss syndrome (allergic granulomatosis and angiitis) [online]. 2005. [Cited 2005 October 14th]. Available from URL: http://www.utdol.com/application/topic.asp file=int_lung/5519&type=A&selectedTitle=1~22

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