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What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

Что такое склеродермия и как ее лечить?</p><p> Symptoms and diagnosis of the disease “/></p></div><p>Scleroderma is a systemic disease. The disease can affect not only the skin, but also any organ that has developed connective tissue.</p><p><strong>The replacement of healthy cells with fibrosis is the result of a disruption of the blood supply in certain areas.</strong>.</p><p>In the article we will explain what it is: scleroderma and how to treat the disease itself and its manifestations.</p><h2>Changes in histology at the cellular level.</h2><p>The true causes of the disease remain a mystery. Only one fact has been proven: in women, scleroderma occurs about six times more than in men.</p><p><strong>In children, the disease manifests as a result of the following factors:</strong></p><ul><li>Genetic mutations</li><li>Immune disorders</li><li>The consequences of diseases carried by the mother.</li></ul><p>The histological changes in scleroderma are excessive deposits of proteins in the organs and skin. This is especially true of a protein like collagen.</p><p> This is the main essence of the pathological changes.</p><p>The vessels begin to take the form of fibrosis of the small arteries of the skin, heart, lungs, stomach, intestines and kidneys. The vascular walls become thicker and the light narrows until it is completely filled.</p><p> As a result, the patient develops ischemia of the affected areas.</p><p>And as a result of ischemia, there is a lack of nutrients and oxygen. <strong>Partially the capillaries expand, partially disappear completely.</p><p></strong> An increase in capillary tortuosity occurs and telangiectasias appear.</p><p><strong>Changes in histology, as a result of systemic scleroderma, also consist of:</strong></p><ul><li>Immune disorders</li><li>Violations of the functionality of fibroblastic cells.</li><li>Chromosomal anomalies</li></ul><p>On the role of immune disorders, it should be said that they are generally related specifically to cellular immunity. <strong>Patients manifest excessive activity of T helper cells, monocytes, mast cells.</p><p></strong> All elements are ways to activate cellular fibroblasts. Scleroderma causes alterations in humoral immunity.</p><p>When the functionality of the fibroblast cells is altered, the production of collagen protein is accelerated. Collagen impregnates the entire skin.</p><p> At the same time, the protein is welded to other tissues that are well below the skin.</p><p>Chromosomal abnormalities occur in the vast majority of patients with scleroderma. <strong>The number of these patients reaches 90%.</strong></p><p><strong>They form:</strong></p><ul><li>Breaks links in the chromosomal chain.</li><li>Ring-shaped chromosomes.</li></ul><h2>Pathology in the internal organs and in the outbreak of education.</h2><p>The specialists who study scleroderma argue that the disease can cause unique and multiple changes.</p><p><strong>These changes refer to:</strong></p><ul><li><div style=What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

Skin

  • Digestive System (GIT)
  • Pulmonary system
  • Joints
  • Muscle
  • Hearts
  • Of the liver.
  • The skin, or rather its epidermis, thins.. At the same time or sooner, T lymphocytes, monocytes and plasma cells accumulate in the skin.

    Then, under the epidermis, a mass of collagen protein is formed.

    The result is an internal growth of the upper layers of the skin in the fiber and subsequent welding with deeper tissues. Everything that lies between the layers atrophies.

    Gastrointestinal tract loses thickness of the skin.. This process begins approximately from the middle third of the esophagus, but may start even lower.

    The motor function of the affected areas is gradually lost, they begin to expand. The muscular layer thins.

    Fibrosis of the lungs originates in the wall of the bronchi.. It extends even more to the alveoli of lung tissue.

    Tissue compaction causes emphysema, loss of ventilation, interruption of the natural exchange of carbon dioxide with oxygen.

    As a result of pulmonary fibrosis, the walls may break. Small cysts form.

    Scleroderma in the joints leads to infiltration of the inner lining Articular bags of plasma cells and lymphocytes. The fibrin is deposited in the thickness of the bag.

    The same process occurs in the tendons.

    Since the disease is accompanied by a violation of the nutrition of the muscles, they are affected by atrophy. Another consequence is a breach of contractility.

    Tissue compaction occurs as a result of the infiltration of collagen and lymphocytes.

    Scleroderma causes changes in the heart, and the main ones refer to the myocardium. The replacement of myocytes by scar tissue and dystrophy occurs due to hypoxia.

    Fibrous areas cause pathological changes in the conduction of nerve impulses. Disturbed formation of excitation foci.

    The main manifestations of these changes:

    • Frequent arrhythmias
    • Atrioventricular block.

    The necrosis can appear in the kidneys, as a result of changes in the structure of the capillary glomeruli..

    Other manifestations include a reduction in urine leakage and minor heart attacks. It is necessary to clarify that changes in renal tissue are observed in approximately 50% of patients.

    What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

    Constriction of the hand vessels.

    Scleroderma code in ICD-10

    Scleroderma manifests clinically in various forms.

    Therefore, the international classification of ICD-10 diseases in the registration process in one way or another divides the disease into groups:

    • Class L – skin related diseases
    • Class M – Diseases related to connective tissue pathologies.

    This practice has made it possible to include known forms of the disease in a group whose coding is denoted as L94 and M34. The name of the group is “Systemic sclerosis”, “Other localized changes in the connective tissue”.

    The following classification has been adopted:

    • Focal scleroderma (limited)
    • Scleroderma on plate
    • Scleroderma tear
    • What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

      Ring-shaped scleroderma

    • Linear scleroderma
    • Buske’s disease
    • Generalized scleroderma
    • Eosinophilic fasciitis
    • Induced scleroderma.

    You should briefly describe each form of the disease. Limited scleroderma affects only the areas of the skin.

    The changes do not affect the internal organs.

    The shape of the plaque is a change in the structure of the skin tissue. Part of the skin is rounded. The lesion is quite large.

    Also characteristic are the bluish rings around the plots.

    If you see a tear shape, you may see small, slightly round spots. Its color is whitish with a pearly nuance.

    The surface of the site sinks a little inside. Scleroderma drops are found in the upper part of the body, closer to the head.

    The ring-shaped form is a specific ring-shaped rash. They appear on the skin of the legs.

    Very often, one of the symptoms of this form is the disease of the elephant.

    Linear scleroderma is somewhat different from other forms. Its manifestation is a strip of skin on the forehead, manifested as a result of a pathology.

    Buske’s disease is also known as the edematous form of scleroderma. Other names are adult sclerema, benign aponeurotic sclerema.

    Such scleroderma is a rapidly progressive edema. It will be dense to the touch.

    There is swelling in the face and neck. Possible dissemination will occur on the shoulders and forearms.

    The pathology does not develop in the internal organs. A distinctive feature of Bushke is the connection of the disease with acute streptococcal infections.

    What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

    Generalized scleroderma is also known as progressive sclerosis, diffuse or systemic scleroderma.

    This is one of the most severe forms of scleroderma.

    In this case, the injury affects all the connective tissue. Here there is a gradual capture of the pathological processes of the skin, and then the organs as well.

    Eosinophilic fasciitis involves skin manifestations, which are very similar to most forms of scleroderma.

    However, the difference between fasciitis is the accumulation of eosinophils in the tissue. In the course of the veins formed a specific “orange peel”.

    The furrows of the veins begin to fall.

    Fasciitis also has contractures of joint flexion.

    The form of scleroderma, known as induced, becomes the result of a reaction to low quality silicone breast prostheses for the bleomycin and pentazine preparations. Another condition for appearance – a long contact of silicone or vinyl chloride.

    Scleroderma sometimes occurs in combination with the following types of connective tissue diseases:

    • Dermatomyositis
    • Systemic lupus erythematosus
    • Rheumatoid arthritis.

    In this case, special syndromes are distinguished in the ICD-10 classification. It is extremely important to separate scleroderma from a series of diseases and forms of diseases that also lead to fibrosis.

    Therefore, in case of a disease, the precise diagnosis is extremely important, in which mandatory punctures of the affected skin tissue and plaques are used.

    At the same time, punctures of the tissues of the internal organs are made.

    Sometimes abnormalities develop with scleroderma. One of them is dermatosclerosis: diffuse sclerosis of the skin.

    The skin tightens and gradually loses its elasticity.

    Causes of scleroderma

    The etiology of scleroderma is not fully understood. The true causes of the disease are separated.

    Only a series of factors that cause scleroderma were studied and scientifically substantiated.

    These factors include:

    • Genetic predisposition
    • Genetic mutations
    • What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

      Viral infections (cytomegalovirus is especially dangerous)

    • Carbon or carbon powder
    • Contact or work with organic solvents.
    • Polyvinyl chloride
    • Chemotherapy in the treatment of cancer.
    • Vibration effects
    • Freezing
    • Malaria
    • Injuries
    • Endocrine disruption
    • Blood transfusion
    • Some drugs

    Specialists who study the blood composition of patients with scleroderma, found in the blood a high content of enzymes that damage the walls of blood vessels from the inside.

    Clotting factor VIII was also detected in a large volume. The diabetic form of scleroderma also has characteristics, which will be discussed later.

    The clinical picture of scleroderma.

    The symptoms of scleroderma will develop according to the nature of the disease. As an example, a plaque form can be given, when the changes relate only to the skin.

    Or vice versa: when systemic scleroderma occurs, the skin lesions are accompanied by injuries to muscles and joints, as well as internal organs.

    Raynaud’s syndrome – the earliest manifestation. This syndrome is caused by vascular spasms in the arms and legs.

    Temporal narrowing of the arteries, as one of the clinical manifestations, and some other early signs may appear in a cold or stressful state.

    In the case of narrowing of the arteries, a spasm occurs, which is expressed in the pallor of the hands, and then in the cyanosis. When the spasm passes, the bluish areas turn pink.

    In adults, there are several types of changes in skin sensitivity. Patients begin to freeze, even when they are hot. Such attacks are accompanied by painful sensations at the tips of the fingers.

    Such phenomena can occur on the lips, on the tip of the nose or on the ears.

    The process of compaction and thickening of the skin initially affects the fingers. This is a common reaction for most patients.

    Then the compaction process extends to other parts of the body.

    There is a typical facial expression characteristic of patients with scleroderma:

    • Nasolabial septum smoothed
    • Softens frontal wrinkles
    • Radial wrinkles around the lips.

    For the face it is characterized by the absence of facial expressions, that is, the face becomes a mask.

    What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

    To understand that swelling in the skin is a sign of scleroderma, you can push it.

    On the skin there are no traces or depressions.

    In 90% of cases, changes in the skin go through three stages:

    • Swelling
    • Compaction
    • Thinning
    What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

    Clinical manifestations of scleroderma.

    In running situations, ulcers form on the fingers, and these ulcers will be quite painful. There are scars, shaped like a cured bite.

    Some ulcers appear below the ankles, on the heels, elbows and knees.

    In the first of these signs, do not panic, but you should immediately consult a doctor. The fact is that similar manifestations are characteristic of a much milder disease, xeroderma.

    Joint changes are characterized by pain in the morning. The patient feels a clear stiffness in all joints.

    The nails begin to collapse and gradually thin out.

    The fingers become shorter and thicker. Therefore, the hand seems to have lost its shape.

    Muscle changes are accompanied by fatigue and weakness.

    The vast majority of people with scleroderma have digestive problems. A person can not swallow, after a meal, heartburn begins.

    Another common symptom is a rape of the stool, pain and swelling.

    Scleroderma clinic in children.

    Что такое склеродермия и как ее лечить?</p><p> Symptoms and diagnosis of the disease “/></p></div><p>The symptomatology of scleroderma in children proceeds in exactly the same way as adults. The shape of the plaque is accompanied by the appearance of yellow-pink spots on the legs and arms.</p><p>The same is observed later in the whole body. <strong>Interestingly, the spots will be bright and very dense at the same time.</strong></p><p>The remaining forms in 90% of cases appear in the same way as in older patients: Raynaud’s syndrome, subsequent damage to the joints, skin and internal organs.</p><p>However, unlike adults, children are less likely to suffer a systemic form of the disease.</p><h2>Diagnosis of scleroderma</h2><p>The difficulty of diagnosis lies in the fact that doctors often accept changes in muscles and joints, along with cardiac arrhythmias, which are very similar to rheumatoid arthritis.</p><p><strong>A seal of the skin of the hands can be sclerodactyly, one of the options for scleroderma.</strong> The correct differential diagnosis is only possible after the radiography and the appropriate immunological tests.</p><p>Many people wonder what doctor is treating scleroderma. The reason for this is that the disease, judging by the origin of the name, may seem a dermatological problem.</p><p> But she is dedicated to rheumatologists.</p><p>The rheumatologists of the USA UU They developed two criteria standards: “big” and “small” criteria. If one of the “large” criteria or two of the “small” criteria is met, it is very likely that the scleroderma will be diagnosed.</p><p><strong>“Large” criteria:</strong></p><ul><li>Thickening of the skin of the fingers, rising and moving towards the body.</li><li>Changes in the skin of the chest, face, neck and abdomen, typical of the disease.</li></ul><p><strong>“Small”:</strong></p><ul><li>Changes in the skin of the fingers.</li><li>Scars on the fingertips and several phalanges.</li><li>Bilateral compaction of lung tissue, and several other criteria.</li></ul><p>Signs are considered changes in kidney tests. In the differential diagnosis, specialists pay special attention to the presence of autoantibodies in the blood.</p><p> Iron and vitamin B12 deficiency are important.</p><p>Along with the radiography, instrumental techniques are applied. Capillaroscopy applied to the nail of the hand is the most informative.</p><p> The rest of the research is called specifically for scleroderma.</p><h2>Scleroderma therapy</h2><p>The treatment of the disease is a constant observation in the clinic. <strong>There is no standard practice of therapy, so it is required for each patient individually.</p><p></strong> We will try to tell everything about how the disease is treated.</p><p><strong>The treatment is necessary to:</strong></p><p style=
    • What is scleroderma and how to treat it? Symptoms and diagnosis of the disease.

      Elimination of vascular changes.

    • The destruction of fibrosis in the skin and internal organs.
    • Supporting the functionality of internal organs.
    • Correction of immunity.

    Day of routine and nutrition.

    Treatment involves adherence to a particular regimen throughout life. Otherwise, the scleroderma will progress. Patients can not sunbathe, experience hypothermia or vibration.

    It is important to completely give up bad habits and caffeine.

    Dairy products, spicy, smoked foods can cause allergies. While vegetables, fruits, brown rice and algae are the healthiest food for the patient and their immunity.

    The obligatory vitamins for scleroderma are A, C and E, which have a positive effect on immunity. The B vitamins increase the resistance to infections.

    The use of all products containing vitamins is possible at home and does not require periodic visits to special establishments.

    Conservative treatment of scleroderma.

    Conservative or pharmacological treatment consists of taking medications, the dosage of which is prescribed only by the attending physician. It will also control the frequency of intake and how the drugs combine with each other.

    Appointed:

    • Vasodilator medications (verapamil, flunarizine, lacidipine, and other calcium channel blockers)
    • Adrenoreceptor blockers A (nicergoline, terazosin)
    • Ginkgo biloba extract medicines (Tanakan)
    • Synthetic Prostaglandin (Alprostadil)
    • Prostacyclins (iloprost)
    • Antiplatelet
    • Anticoagulants

    All groups of medications are necessary to relieve symptoms. Thus, vasodilator drugs relieve spasms of blood vessels, antiplatelet agents and anticoagulants prevent the formation of blood clots.

    Also named Artamin and Kuprenil, which act through the immune system and limit fibrosis. In addition, D-penicillamine, a drug that reduces collagen synthesis to zero, will inhibit fibrosis.

    For joints that periodically experience inflammation, NSAIDs (diclofenac, ketoprofen) are required along with hormone therapy. If swallowing is altered, the patient will have to drink prokinetic – Meklozin.

    If the intestines or lungs are affected, then special therapy is needed, including sanatorium treatment. To eliminate the manifestations of scleroderma on the face, you can apply cosmetics: Cleoderma face products and many others.

    Traditional methods of treatment

    Folk remedies therapy is not recommended by experts. The reason for this is the lack of knowledge of the pathology, as well as its mechanism.

    In addition, the prognosis of the course of the disease is not always obvious.

    Even an experienced specialist can not predict the positive or negative effect of “popular” procedures. If the patient decides to try one of the methods, he should consult a doctor.

    Prognosis of the disease

    The prognosis will depend directly on the form of the disease and the initial manifestations.

    Adverse factors are the age of the patient with a diagnosis of more than forty-five years, pulmonary fibrosis, arrhythmia and kidney damage in the first three years of the disease.

    A disease such as scleroderma has a specific etiology and an individual course. The nature of the disease is not fully understood.

    And only the correct diagnosis, made as soon as possible, and the appropriate treatment can play a positive role in the patient’s survival factor.

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