CIDPUSA.org Autoimmune diseases

MARFRAN SYNDROME

Marfan syndrome is a disorder that affects the body's connective tissue . Connective tissue is found everywhere in the body. Think of it as a type of "glue" that helps support all of your organs, blood vessels, bones, joints, and muscles.

In people with Marfan syndrome, this "glue" is weaker than normal. This causes changes in many systems of the body, but especially the heart, eyes, and bones.

People with Marfan syndrome often share similar traits. They tend to be tall and thin with very long arms, legs, fingers, and toes. They often develop curves in their spines (scoliosis) or their chest bones. They may also be nearsighted and have other problems with their eyes. But the most serious thing about Marfan syndrome is what can happen with the heart.

Over time, weak connective tissue can cause the aorta, the large artery that carries blood away from the heart to the rest of the body, to stretch and dilate , or widen. If not treated, the aorta can suddenly tear, causing blood to leak out of the heart. This condition, called a dissection (, is very serious and can cause death.

The good news is that, even though Marfan syndrome has been considered as autoimmune , doctors can treat just about all of its symptoms and if diagnosed early stop the disease progression. Thanks to new research and treatments, people with Marfan syndrome who are diagnosed early and get good medical care can live long, happy lives.

Definition of Marfan syndrome:

Marfan syndrome is a disorder of connective tissue, the tissue that strengthens the body's structures. Disorders of connective tissue affect the skeletal system, cardiovascular system, eyes, and skin. Marfan Traits

Marfan syndrome often affects the long bones of the body. This can lead to signs, or traits

• A tall, thin build.
• Long arms, legs, fingers, and toes and flexible joints.
• A spine that curves to one side. This condition is called scoliosis.
• A chest that sinks in or sticks out. pr pectus excavatum and pectus carinatum,
• Teeth that are too crowded.
• Flat feet.

Stretch marks on the skin also are a common trait in people who have Marfan syndrome. Stretch marks usually appear on the lower back, buttocks, shoulders, breasts, thighs, and abdomen.

Not everyone who has these traits has Marfan syndrome. Some of these traits also are signs of other connective tissue disorders.