CREST syndrome is a systemic scleroderma of a limited form. This variety causes the body to produce excessive amounts 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 pathology of chronic connective tissue that affects the skin and a number of internal organs of a person. The basis of this disease is a defect in the signs of the connective tissue skeleton, in connection with which sclerotic changes are manifested, expressed as 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 rheumatologic autoimmune disease. The term “autoimmune” means that this ailment occurs due to impaired functioning of the immune system, which, due to various circumstances, attacks the tissues and cells of one’s own body. As a result, an inflammatory reaction occurs, leading to tightening 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 location, it is not possible to precisely 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 is significantly complicated with the advent of scleroderma. This is primarily due to a decrease in physical activity and pain, which can be felt in some cases. Due to digestive problems, patients have to adhere to a special diet and eat small meals, but often. Degenerative-sclerotic skin changes make patients constantly monitor the value of its hydration and be especially careful during sports activities or during 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 thoughts of a disease that is currently chronic and incurable. Since such an ailment can cause significant external changes, the image of a person and self-esteem suffer, as a result of which various kinds of personal and social conflicts arise.
Extremely important is the psychological support of patients with scleroderma from households, relatives and friends. It will save the necessary quality of life.
What causes scleroderma
Scleroderma is a chronic acquired pathology of connective tissue, and its exact cause has not yet been elucidated. But thanks to the improvement of medicine, laboratory diagnostics and molecular biology, it is now possible to identify and study the main pathological mechanisms that take part in the development of the disease. Now there are a number of theories that describe factors that can provoke scleroderma, and especially its effects.
It is generally accepted that factors such as can participate in the appearance of scleroderma and CREST syndrome:
- inflammatory;
- genetic;
- infectious;
- autoimmune;
- the environment;
- some medications.
Systemic scleroderma
Scleroderma of a systemic nature is an autoimmune pathology of connective tissue, which leads to the stimulation of the immune system, resulting in the formation of hard tissue and skin containing too much collagen. This form of scleroderma can cause depletion of the patient's body. Among its symptoms are the following:
- muscle atrophy;
- an increase in intestinal volume;
- pulmonary fibrosis;
- heart enlargement;
- renal failure;
- bouts of coughing and suffocation;
- circulatory failure.
CREST syndrome is a form of systemic scleroderma, which is limited and mild, manifests itself mainly on the skin.
Symptomatology
The name of the syndrome is an abbreviation formed from the first letters of the English names of the symptoms characteristic of the disease:
- C - calcification is calcification that affects soft tissues.
- R - Raynaud's phenomenon.
- E - esophagitis (esophageal dysmotility), that is, defects in the motility of the esophagus.
- S - sclerodactyly (sclerodactyly) - thickening of the skin on the fingers.
- T - telangiectasia (telangiectasia) - the expansion of small blood vessels.
Symptoms of the CROSS syndrome are rather unpleasant.
The deposition of calcium salts in soft tissues can be determined by x-ray. There is calcification on the face, fingers, skin of the elbows and knees, the trunk. If under the influence of calcification the skin breaks, then 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, in more rare cases, 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 fingers or their pads. When the attack is completed, the fingers turn red and become inflamed, in addition, there is a sensation of heat in the hands. This can also lead to the occurrence of ischemia, scars, ulcers and gangrene.
CREST 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 problems with swallowing, and severe heartburn and inflammation of the esophagus can also occur.
Due to the coarsening 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 reason for which is the expansion of small blood vessels. These changes are local in nature and are for the most part a cosmetic problem.
CREST syndrome is characterized by a slow course, while its prognosis is better than with general scleroderma of the systemic type, affecting, in addition to the skin, tissues, blood vessels, internal organs, bones and muscles.
Pathology therapy
What can help a patient with this diagnosis?
Appointment of timely therapy significantly increases the patient's chances of successful completion and the absence of disability in the future. When the first symptoms of the disease are detected, you should immediately consult 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 techniques and varieties that are used depending on the nature of the development of the disease and its form. The treatment of CREST syndrome with scleroderma is symptomatic, most often anti-inflammatory and restorative agents are used, due to which the patient is gradually returned with lost motor activity.
Medicines for a speedy recovery
If infections are detected 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 bone destruction, in some cases surgical intervention is required (if the accumulations of calcium are large). A pulmonary disease is also a danger. If there are difficulties with breathing, small doses of immunosuppressants and corticosteroids, as well as pulmonary vasodilators are prescribed for the patient (observation in the clinic). If the specialist carries out the correct treatment, then patients can recover and live for more than ten children. With progressive pulmonary fibrosis, the prognosis is harder. We examined what is CROSS syndrome.