Churg-Strauss syndrome (CSS)

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What is Churg-Strauss syndrome?

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.

Who gets Churg-Strauss syndrome?

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.

Predisposing Factors

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

Progression

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.

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