A connective tissue disease is a disease that has the connective tissue of the body as a target pathology. Connective tissue is any type of biological tissue with an extensive extracellular matrix that supports, binds together, and protects organs. These networks form a framework, or matrix, for the body, and consist of two major structural protein molecules: collagen and elastin. There are many types of collagen proteins in each of the tissues of the body. Elastin has the ability to stretch and return to its original length - like a spring or a rubber band. Elastin is the main component of the ligaments (the tissue that connects the bone with the bone) and the skin. In patients with connective tissue disease, it is common for collagen and elastin to be injured by inflammation (ICT). Many connective tissue diseases have abnormal immune system activity with inflammation of the tissues as a result of an immune system that is directed at one's body tissues (autoimmunity).
The disease in which inflammation or weakness of collagen tends to occur is also referred to as collagen disease. Collagen collagen disease can (but not always) be associated with collagen and blood vessel disorders and which are autoimmune. See also vasculitis.
Connective tissue disease can have a strong or weak inherited risk, and can also be caused by environmental factors.
Video Connective tissue disease
profitable connective tissue disruption
- Marfan syndrome - a genetic disease that causes abnormal fibrillation.
- Ehlers-Danlos syndrome - disability in collagen synthesis (Type I or III) causes progressive collagen damage, with different types of EDS affecting various places in the body, such as joints, heart valves, organ walls, artery walls.
- Osteogenesis imperfecta - caused by poor quality collagen, or insufficient number of normal collagen (especially type I), is required for healthy, strong bone and other connective tissues. Stickler's syndrome affects collagen (especially type II and XI), and can cause typical facial appearance, eye abnormalities, hearing loss, and joint problems.
- Alport syndrome - collagen defects (type IV), found in the renal basin membrane, inner and inner ears, leading to glomerulonephritis, hearing loss, and eye disease.
- Contraction of congenital arachnodaktia - Also known as Beals syndrome. It is similar to Marfan's syndrome but with contractures of hips, knees, elbows and ankles and tangled ears.
- Loeys-Dietz Syndrome - This disorder is characterized by aneurysms in the aorta, often in children. Symptoms look like Marfan Syndrome and EDS. This is caused by a TGFBR gene mutation on chromosome 3 or 9 depending on its type.
Maps Connective tissue disease
Autoimmune auto network troubles
It is also referred to as a systemic autoimmune disease. An autoimmune CTD may have genetic and environmental causes. Genetic factors can create a tendency to develop this autoimmune disease. They are characterized as a group by the spontaneous overactivity of the immune system that results in the production of additional antibodies into the circulation. Classical collagen vascular disease has a "classic" presentation with typical findings that doctors can recognize during examination. Each also has a "classic" blood test disorder and an abnormal pattern of antibodies. However, each of these diseases can evolve slowly or rapidly from a very delicate disorder before demonstrating the classic features that aid in the diagnosis. Classical collagen vascular disease includes:
- Systemic lupus erythematosus (SLE) - Inflammation of the connective tissue, SLE can attack any organ system. It's up to nine times more common in women than men and affects black women three times more often than white women. This condition is worsened by the sun.
- Rheumatoid arthritis - Rheumatoid arthritis is a systemic disorder in which the immune cells attack and inflate the membrane around the joint. It can also affect the heart, lungs, and eyes. Of the approximately 2.1 million Americans with rheumatoid arthritis, about 1.5 million (71 percent) are women.
- Scleroderma - activation of immune cells that produce scarring in the skin, internal organs, and small blood vessels. It affects women three times more often than men overall, but increases to a rate 15 times greater for women during the year of childbirth, and appears to be more common among black women.
- The Sj̮'̦gren syndrome - also called Sj̮'̦gren disease, is a chronic, slowly developing disability to salivate and tear. May occur alone or with rheumatoid arthritis, scleroderma, or systemic lupus erythematosus. Nine out of 10 cases occur in women, most often in or around mid-life. Mixed connective-tissue disease (MCTD) is a disorder in which features of various connective tissue diseases (CTD) such as systemic lupus erythematosus (SLE); systemic sclerosis (SSc); dermatomyositis (DM); polymyositis (PM); anti-synthetase syndrome; and, occasionally, Sj̮'̦gren's syndrome can coexist and overlap. The course of the disease is chronic and usually lighter than other CTDs. In most cases, MCTD is considered a transitional stage of disease that eventually becomes SLE or Scleroderma.
- Undifferentiated connective tissue disease (UCTD) is a disease in which the body mistakenly attacks its own tissues. It is diagnosed when there is evidence of an existing autoimmune condition that does not meet the criteria for certain autoimmune diseases, such as systemic lupus erythematosus or scleroderma. Lupus latent and incomplete lupus are alternate terms that have been used to describe this condition.
- Psoriatic arthritis is also a collagen vascular disease.
Other connective tissue disruptions
- Peyronie's disease - involves the growth of abnormal collagen (Type I and III) in the penis.
- Scurvy - caused by dietary vitamin C deficiency, leading to abnormal collagen.
References
External links
- Merck's manual: musculoskeletal and connective tissue disorders
- Merck's Manual: Inherited connective tissue disorders
- The Arthritis Foundation
- Myositis Foundation
Source of the article : Wikipedia