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CAPS Autoinflammatory Diseases and IL-1β

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How does the immune system normally work without these genetic diseases?

Normally, the body identifies foreign invaders such as germs and viruses. The body alerts your immune system to protect you from these foreign materials. In response, the immune system calls a number of different substances into action until the threat is gone.

For example, when a healthy person has a cold, the immune system turns on like a light switch. When that person is better, the immune system switches back off.

What happens in people with CAPS?

CAPS are genetic autoinflammatory diseases. In people with autoinflammatory diseases, the immune system’s switch is broken—it turns on and gets stuck. Instead of responding to threats from foreign invaders, the immune system becomes too active. This excessive activity can cause inflammation in the body.

How does IL-1β fit in these genetic diseases?

One of the substances that the immune system uses to protect you is called interleukin-1 beta (IL-1β).

However, in people with Cryopyrin-Associated Periodic Syndromes (CAPS) hereditary diseases, the body makes too much IL-1β, even when there is no threat from foreign invaders.

This happens because people with CAPS autoinflammatory diseases have an abnormal gene. This gene makes it so that a certain protein, called cryopyrin, is overactive. This protein gives the condition its name.

When cryopyrin is overactive, the body makes more IL-1β than usual. Without something to hold it back, this extra IL-1β causes inflammation. For unknown reasons, this activity often begins with exposure to cold temperatures.

  • The mutations (accidental changes) in the gene cause the protein cryopyrin to be overactive
  • This causes too much IL-1β to be produced, which results in inflammation in the body

Inflammation from IL-1β happens all over the body. Inflammation produces the symptoms seen in the subtypes of CAPS, including

Scientists have made great progress in understanding CAPS rare genetic diseases. This research has helped scientists develop treatments for these hereditary autoinflammatory disorders. In fact, some patients have seen their symptoms relieved for periods of time.