Collagen Vascular Diseases: Overview

Collagen vascular diseases are a group of autoimmune diseases that affect connective tissue.

What Are Collagen Vascular Diseases?

Collagen vascular diseases, also called connective tissue diseases (CTDs), are autoimmune diseases that include a wide array of disorders in which the body's natural immune or self-protection system fails to recognize its own tissues and attacks itself. Some of these diseases limit their damage to a single organ, while others create problems throughout the body.

What Causes Collagen Vascular Diseases?

In a healthy immune system, antigens (foreign substances such as viruses and bacteria) are recognized as different (or nonself) from normal body tissues (or self). When an antigen enters the bloodstream, it triggers the production of antibodies, substances that attack the alien (nonself) substance. Lymphocytes (LIM-fo-sites) and leukocytes (LOO-ko-sites) are the specialized white blood cells responsible for creating these antibodies that attack the antigens (nonself).

Lymphocytes include two major subtypes (T cells and B cells), which have the unique ability to recognize the invading substance and alert the immune system to destroy it. The process is highly specialized: Each lymphocyte recognizes a specific antigen and produces an antibody against only that antigen.

* rays of sunlight may be a contributing cause. Collagen vascular diseases are not contagious; people cannot catch these diseases from one another.

Rheumatoid arthritis

Rheumatoid arthritis is a chronic inflammatory * disease that causes stiffness in the joints (places where bones, tendons, muscles, and ligaments meet) and can lead to pain, limitations in mobility, and disfigurement. It is an ancient disease; archeologists have found skeletons thousands of years old that have bone changes indicating that the person had this condition.

In rheumatoid arthritis, the autoimmune process begins in connective tissue and the cushiony membranes that surround joints and the ends of bones. Collagen (KOL-a-jen) is the tough glue-like protein that gives joints their support and flexibility; it represents 30 percent of the body's protein. Rheumatoid arthritis is thought to begin when T cells mistake the body's own collagen cells for foreign antigens and alert B cells to produce antibodies to fight the invader. The leukocytes rush in and produce cytokines (SY-to-kines), small proteins that are essential in healing the body but that can also cause serious damage in large amounts. The inflammation and joint damage that result can lead to joint deformities and can spread throughout the body to other sites of connective tissue.

Systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is one of the major types of the chronic (long-lasting) disease known as lupus. Lupus, which was first described in 1828, causes inflammation of connective tissue, the material that holds in place the various structures of the body. While the cause or causes of lupus are uncertain, the disease is considered to be an autoimmune disorder. SLE affects the skin but also involves other tissues and organs.

Symptoms may include a butterfly-shaped rash over the bridge of the nose and cheeks; hair loss; skin lesions that may spread and cause damage to the mucous membranes and other tissues; arthritis; weakness; extreme fatigue; fever; sensitivity to sunlight; and loss of weight. Some people with SLE have problems with kidney function and uremia (yoo-REE-me-a), or the buildup of toxic substances in the blood due to kidney failure. These complications can be fatal. SLE can affect the nervous system and cause psychological problems, seizures, or other symptoms. The lungs, heart, liver, and blood cells may also be involved. In the blood, certain antibodies can interfere with the normal function of the blood vessels and can bring on a stroke * or heart attack. In pregnant women, the presence of these antibodies can cause a miscarriage * .

Frequently, people who have SLE also have another collagen vascular disease: scleroderma. A chronic autoimmune disease, scleroderma causes skin to thicken, and tough fibrous tissue to form in the internal organs of the digestive tract, kidneys, heart, and lungs.

Raynaud's disease

People who have collagen vascular diseases may also be diagnosed with Raynaud's disease, although not everyone who has Raynaud's disease has a collagen vascular disease. Raynaud's disease is a disorder in which the vessels that supply blood to the fingers and toes respond to cold or other stimuli by going into spasm (contracting), which reduces the supply of blood and causes the digits to turn white, feel numb, tingle, or burn. In severe cases, the restriction of the arteries may cause the fingers to thicken, which can lead to ulcerations (sores that do not heal) at the fingertips as well as changes in the fingernails. Sometimes, gangrene (tissue death) can occur.

How Do People Know They Have a Collagen Vascular Disease?

Signs and symptoms

Signs and symptoms of CVDs differ depending on the illness, but they often include joint pain, fever, rash, frequent infections, fatigue, mouth ulcers, dry mouth, dry eyes, hair loss, difficulty swallowing, swollen glands, or fingers and toes that get overly cold when exposed to cooler temperatures. In addition to SLE, rheumatoid arthritis, and scleroderma, collagen vascular diseases include:

Diagnosis Treatment

Treatment is usually focused on minimizing symptoms and preventing complications of the disease.

Some people with connective tissue disorders may experience remissions * as symptoms disappear for periods of time and subsequent flareups or exacerbations * when symptoms return. Treatment depends on the specific organs affected and the severity of the disease. Doctors may prescribe corticosteroid medications or other drugs to suppress the immune system. Analgesic (pain-relieving) medications may also be prescribed to ease discomfort.

Physical therapy may be prescribed to help improve or maintain mobility and to reduce joint and muscle pain. Occupational therapy may be prescribed to help the person improve or maintain the ability to perform normal activities of daily living such as using a telephone, driving a car, and performing work activities. Physical and occupational therapy may teach the person to use assistive devices in order to remain as independent as possible. In advanced cases, immunosuppressant drugs may help lessen the immune system's overreaction.

Can Collagen Vascular Diseases Be Prevented?

Collagen vascular diseases could not be prevented as of 2016 because their causes were not yet fully understood. However, people who have a collagen vascular disorder can make use of strategies to decrease symptom severity, risks of complications, and incidence of exacerbations. Often people are aware of specific triggers that cause problems in managing their own disease. Triggers may vary from one person to another. Avoiding triggers and reducing physical and emotional stressors seem to reduce symptoms for some people.

See also Arthritis • Autoimmune Disorders: Overview • Immune Deficiencies • Immune System and Other Body Defenses: Overview • Lupus • Raynaud's Disease • Scleroderma • Sjögren's Syndrome

Assistive Devices and Safety Measures

Resources

Books and Articles

Hertl, Michael, editor. Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management. 3rd ed. New York: Springer, 2011.

Websites

American Autoimmune Related Diseases Association. “List of Diseases.” http://www.aarda.org/autoimmune-information/list-of-diseases (accessed April 4, 2016).

MedlinePlus. “Collagen Vascular Disease.” U.S. National Library of Medicine, National Institutes of Health. https://www.nlm.nih.gov/medlineplus/ency/article/001223.htm (accessed April 4, 2016).

Organizations

American Autoimmune Related Diseases Association. 22100 Gratiot Ave., Eastpointe, MI 48021. Telephone: 586-776-3900. Website: http://www.aarda.org (accessed April 4, 2016).

Lupus Foundation of America. 2000 L Street NW, Suite 410, Washington, DC 20036. Toll-free: 800-558-0121. Website: http://www.lupus.org (accessed April 5, 2016).

Scleroderma Foundation. 300 Rosewood Dr., Suite 105, Danvers, MA 01923. Toll-free: 800-722-HOPE (800-722-4673). Website: http://www.scleroderma.org (accessed April 4, 2016).

Sjögren's Syndrome Foundation. 6707 Democracy Blvd., Suite 325, Bethesda, MD 20817. Toll-free: 800-475-6473. Website: http://sjogrens.com (accessed April 5, 2016).

* ultraviolet light is a wavelength of electromagnetic radiation beyond visible light; on the spectrum of light, it falls between the violet end of visible light and x-rays.

* inflammation Is the body's reaction to irritation, infection, or injury that often involves swelling, pain, redness, and warmth.

* stroke is a brain-damaging event usually caused by interference with blood flow to the brain. A stroke may occur when a blood vessel supplying the brain becomes clogged or bursts, depriving brain tissue of oxygen. As a result, nerve cells in the affected area of the brain, and the specific body parts they control, do not function properly.

* miscarriage (MIS-kare-ij) is the end of a pregnancy through the death of the embryo or fetus before birth.

* remission is an easing of a disease or its symptoms for a prolonged period.

* exacerbation is a worsening of a disease or the signs and symptoms of the disease.

Disclaimer:   This information is not a tool for self-diagnosis or a substitute for professional care.

(MLA 8th Edition)