CROSS-syndrome is systemic scleroderma of a limited form. This variety causes the body to produce an excessive amount of collagen in the skin. The disease can affect almost every part of the body. Its symptoms most often appear gradually and worsen over time. Scleroderma is a chronic connective tissue pathology that affects the skin and a number of internal organs. The basis of this disease is a defect in the signs of the connective tissue framework, in connection with which sclerotic changes appear, expressed in the form of the appearance of coarse fibrous non-functional fibers, that is, scar tissue.
How does CREST syndrome manifest itself in scleroderma?
Features of the disease
Scleroderma is a rheumatological autoimmune disease. The concept of "autoimmune" means that this disease occurs due to impaired functioning of the immune system, which, due to the influence of various circumstances, attacks the tissues and cells of its own body. As a result, an inflammatory reaction occurs, leading totightening and thinning of blood vessels, skin and some internal organs, such as the esophagus, stomach, kidneys, lungs, heart, intestines. Although the lesion may have a different localization, it is not possible to accurately distinguish between the forms of scleroderma. Moreover, a number of famous researchers believe that both forms of the disease are the result of the same pathological process.
The life of patients becomes much more complicated with the advent of scleroderma. This is primarily due to a decrease in physical activity and pain, which can make itself felt in some cases. Due to digestive problems, patients have to follow a special diet and eat small portions, but often. Degenerative-sclerotic skin changes make patients constantly monitor the amount of its moisture and be especially careful during sports or any physical activity. The causes of CREST syndrome are not fully understood.
Psychological discomfort
In addition, many patients with scleroderma feel psychological discomfort caused by thinking about the disease, which is currently chronic and incurable. Since such an ailment can cause significant external changes, a person’s image and self-esteem suffer, as a result of which various personal and social conflicts arise.
Psychological support for patients with scleroderma from family members, relatives and friends is extremely important. She will allowmaintain the quality of life you need.
What causes scleroderma
Scleroderma is a chronic acquired pathology of the connective tissue, and its exact cause has not yet been elucidated. But thanks to the improvement of medicine, laboratory diagnostics and molecular biology, it has now become possible to determine and study the main pathological mechanisms that are involved in the development of the disease. Now there are a number of theories describing the factors that can provoke scleroderma, and the features of its impact.
It is generally accepted that factors such as:
- inflammatory;
- genetic;
- infectious;
- autoimmune;
- environment;
- some medications.
Systemic scleroderma
Systemic scleroderma is an autoimmune connective tissue disorder that stimulates the immune system, resulting in tough tissue and skin that contain too much collagen. This form of scleroderma can cause depletion of the patient's body. Her symptoms include the following:
- muscular atrophy;
- increase in intestinal volume;
- pulmonary fibrosis;
- heart enlargement;
- kidney failure;
- coughing and choking attacks;
- insufficiencycirculation.
CROSS-syndrome is a form of systemic scleroderma that is limited and mild, manifesting mainly on the skin.
Symptomatics
The name of the syndrome is an abbreviation formed from the first letters of the English names of symptoms characteristic of this disease:
- C – calcification is calcification affecting soft tissues.
- R - Raynaud's phenomenon.
- E - esophageal dysmotility, that is, defects in esophageal motility.
- S - sclerodactyly (sclerodactyly) - thickening of the skin on the fingers.
- T - telangiectasia (telangiectasia) - dilation of small blood vessels.
The symptoms of CREST syndrome are quite unpleasant.
The deposition of calcium s alts in soft tissues can be determined by x-ray. There is calcification on the face, fingers, skin of the elbows and knees, torso. If the skin bursts under the influence of calcification, painful ulcers form.
Raynaud's disease
What is this mysterious disease? Raynaud's disease is an unexpected arterial spasm, most often of the fingers and, more rarely, of the legs, which occurs under the influence of strong emotions or cold. It manifests itself in the form of cooling and blanching, and then blue of the fingers or their pads. When the attack is over, the fingers turn red and become inflamed, in addition, there is a sensation of heat in the hands. This may also lead toischemia, scarring, ulcers and gangrene.
CROSS-syndrome also includes defects in the motility of the esophagus, which appear due to the loss of normal mobility of the smooth muscles of the esophagus. The patient has trouble swallowing and may develop severe heartburn and inflammation of the esophagus.
Due to the roughness of the skin on the fingertips, it is difficult to bend and straighten the fingers. Small red spots appear on the face and hands, the cause of which is the expansion of small blood vessels. These changes are local and mostly cosmetic.
CROSS-syndrome is characterized by a slow course, while its prognosis is better than with general systemic scleroderma, affecting, in addition to the skin, also tissues, blood vessels, internal organs, bones and muscles.
Pathology Therapy
How can you help a patient with such a diagnosis?
Timely prescribed therapy greatly increases the patient's chances for a successful outcome and the absence of disability in the future. When the first symptoms of the disease are detected, you should immediately contact a specialist. Doctors also recommend periodically checking people whose relatives also suffered from a similar ailment, since there is a hereditary predisposition. There is no clear therapeutic system, since there are a large number of methods and varieties that are used depending on the nature of the development of the disease and its form. TreatmentCROSS-syndrome in scleroderma is symptomatic, most often anti-inflammatory and restorative agents are used, thanks to which the patient gradually returns the lost motor activity.
Medicines for a speedy recovery
If infections are found in the body, then antibiotics are used. With the rapid development of Raynaud's syndrome, it is necessary to use calcium channel blockers that prevent the destruction of bone tissue, in some cases surgery is required (if calcium accumulations are large). Danger is also a pulmonary variety of the disease. If there are difficulties with breathing, the patient is prescribed small doses of immunosuppressants and corticosteroids, as well as pulmonary vasodilators (observation in the dispensary). If a specialist carries out the correct treatment, then patients can recover and live for more than ten years. With progressive pulmonary fibrosis, the prognosis is more difficult. We looked at what CREST syndrome is.
