What is autoinflammatory syndrome?

Autoinflammatory syndromes are a diverse group of rare diseases that occur when the body has difficulty turning off inflammation. Many cause fevers in children. The Children’s Health rheumatology experts will work with you and your child to provide the best possible treatment.

What causes autoinflammatory syndrome?

Autoinflammatory diseases are caused by genetic mutations in molecules that are involved in regulating the innate immune response-a “hard wired” defense system that evolved to quickly recognize and act against infectious agents and other danger signals produced by our bodies.

Are autoinflammatory diseases genetic?

These conditions are marked by fevers, rashes, joint and muscle pain, abdominal pain, and systemic (all-over) inflammation that is often evident in bloodwork. Most autoinflammatory diseases are genetic, start in childhood, and will continue into adulthood. They will affect multiple family members.

Are autoinflammatory diseases fatal?

Symptoms of autoinflammatory disease Other common symptoms include inflammation of muscles, joints, skin, the gastrointestinal tract and internal organs. If not properly controlled, repeated inflammation can lead to potentially fatal deposits of amyloid protein in vital organs like the kidney.

How do you treat autoinflammatory disease?

The therapy with the most important and widespread effect on autoinflammatory disorders is colchicine, a medication extracted from the meadow saffron and used since the first century for rheumatologic diseases, such as gout.

What are the symptoms of autoinflammatory disease?

Autoinflammatory diseases refer to problems with the innate immune system’s reactions. Immune cells target the body’s own healthy tissues by mistake, signaling the body to attack them. This can cause intense episodes of inflammation that result in such symptoms as fever, rash, or joint swelling.

What is the difference between autoimmune and autoinflammatory?

Autoimmune diseases involve the adaptive immune system, while autoinflammatory diseases involve the innate immune system.

What is familial cold urticaria?

General Discussion. Familial cold autoinflammatory syndrome (FCAS), also known as familial cold urticaria, is a rare, inherited inflammatory disorder characterized by intermittent episodes of rash, fever, joint pain and other signs/symptoms of systemic inflammation triggered by exposure to cold.

Is Muckle Wells Syndrome fatal?

Amyloidosis complicates untreated Muckle–Wells syndrome in 25% of sufferers, beginning in adult life as proteinuria, progressing to kidney failure and death within 10 years. Peripheral neuropathy due to amyloidosis may develop later than the kidney impairment. Puberty may be delayed, especially with severe disease.

Can autoinflammatory disease be cured?

There are no cures for autoimmune diseases, but symptoms can be managed. Everyone’s immune system, genetics and environment are different. That means that your treatment must be unique.