Systemic scleroderma

The reasons contributing to the development of scleroderma include stressful situations, overcooling of the body, severe and chronic infectious diseases, radiation, and chemicals.

An important role in the development of the disease is played by the dysfunction of fibroblasts and other cells that release collagen. The main importance is the reaction of the autoimmune system to collagen. Presumably various disorders of the endocrine system, metabolism and others, together with the negative impact of external factors, create favorable conditions for the development of pathologies in the connective tissue system of both the skin and vessels, and digestive organs, liver, lungs, etc.

Symptoms of Scleroderma

Focal form is manifested in the form of a small number of rounded lesions. In the course of the development of scleroderma, they go through three stages: spots first appear, then they turn into plaques, atrophies occur. At the beginning of scleroderma, it appears weakly: one or several mauve-pink spots, round or oval, appear. Further, a condensation forms in the center of the spot, which with the course of the disease turns into a dense plaque with a smooth shiny surface of white or yellowish color.

Types of scleroderma

The most common types and more studied are considered systemic and limited scleroderma.

Systemic scleroderma affects many human organs and tissues.

There is a type of systemic scleroderma called diffuse. This rare form is found in only five percent of patients. This form is dangerous because the negative changes occur in the internal organs, so its outcome is sad.

Limited scleroderma usually affects the skin and the layers nearby, although other parts of the body do not suffer.

Plaque scleroderma is a type of localized scleroderma. A certain amount of stains forms. Very often spots appear exactly where the skin was damaged. Very rarely, however, it happens that this scleroderma passes by itself.

Linear scleroderma usually affects babies. The site of the disease is the forehead, with the course of the disease it moves towards the hair. In the case of scleroderma on the legs, trophic ulcers may occur. It happens that in the family this disease affects several people. Rarely ring-shaped form of the disease.

Generalized form of scleroderma affects large areas of the body. It is very common in children, although adults are also not insured for this disease.

The disease of white spots usually occurs in women. Children are rarely affected.

With subcutaneous morpheus, the skin becomes thick and coarse. When you donate blood for analysis, the picture changes.

Complications of scleroderma

Scleroderma affects almost all human organs and systems. Therefore, the disease is considered curable.

Initially, skin suffers from scleroderma, of course. On this basis, and diagnose this disease. The standard picture is swollen and reddened skin on the head and hands. In addition, blood circulation is often disturbed in these areas, there are boils, the shape of the nail changes, and hair falls out. Facial features change, wrinkles appear. The skin loses its qualities. It happens that the disease captures not only the head and hands, but also other parts of the body. In this case, the disease is called diffuse scleroderma, but if only certain parts of the body are affected, then this is limited scleroderma.

It happens that the disease affects the muscular system. From this muscle fibers are deformed, some fragments die off, the volume of muscle tissue becomes smaller, and the ligaments, in contrast, become larger.

If the disease gives a complication to the human skeleton, the fingers on all the limbs are deformed. This is also one of the signs of scleroderma.

About 50% of patients with scleroderma also suffer from the organs of the digestive tract. If an ultrasound scan of these organs is performed and their changes are detected, this will also be a sign of scleroderma.

In addition, almost two thirds of patients suffer from problems with the work of the heart and lungs.

Expert Editor: Pavel Alexandrovich Mochalov | D.M.N. general practitioner

Education: Moscow Medical Institute. I. M. Sechenov, specialty - “Medicine” in 1991, in 1993 “Occupational diseases”, in 1996 “Therapy”.

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General information

Systemic scleroderma - collagenosis with polysyndromic manifestations, which is based on progressive fibrosclerosis of the skin, musculoskeletal system, visceral organs and blood vessels. Among the diseases of connective tissue, it ranks second in the frequency of occurrence after systemic lupus erythematosus. Pathology was described in detail in the 17th century, however, the generally accepted term “scleroderma” was introduced only in the middle of the 19th century, and a detailed study of the disease in the framework of rheumatology began in the 40s and 50s. last century. The prevalence of systemic scleroderma ranges from 6 to 20 cases per 1 million people. The female population falls ill 3-6 times more often than the male one; the main share of patients is between the ages of 30-60 years. Systemic scleroderma has a slowly progressive course, which eventually leads to disability.

There are no exact ideas about the causes of systemic scleroderma. Accumulated observations allow only to express individual etiological hypotheses. The facts of family history of systemic scleroderma, as well as the presence of other scleroderma diseases, collagenoses (SLE, rheumatoid arthritis, Sjogren's syndrome), microangiopathies, cardiopathies and nephropathies of unknown origin in favor of genetic determinism. An association of scleroderma with certain antigens and alleles of the HLA-system, determining the immune response, which also indicates the presence of a genetic trace in the genesis of pathology.

Along with hereditary theory, the role of infection, primarily cytomegalovirus, is widely discussed. Some patients associate the debut of the disease with a postponed flu or streptococcal sore throat. A number of observations indicate the triggering role of chemical agents: quartz and coal dust, solvents, drugs (in particular, bleomycin and other cytostatics). The involvement of vibration exposure, stress, cooling and frostbite, injuries in the launch of immunopathological changes in systemic scleroderma has been proven. The background for the development of systemic sclerosis can serve as a hormonal adjustment due to puberty, childbirth, abortion, menopause. In some patients, the onset of the disease is preceded by surgery (tooth extraction, tonsillectomy, etc.) and vaccination. Thus, on the basis of the available data, we can conclude that the multifactorial genesis of systemic scleroderma, combining the complex interaction of endo- and exogenous factors with hereditary predisposition.

The pathogenetic mechanisms of systemic scleroderma are better studied in etiology. The key role in them is played by disturbances of cellular and humoral immunity, leading to an increase in the number of CD4 + and B lymphocytes, and hypersensitivity reaction, which leads to the formation of a wide range of autoantibodies (antinuclear, anti-centromeric, anti-Scl-70, antineutrophilic, anti-endothelial, cytoplasmic, antibodies to connective tissue, etc.) and circulating immune complexes. Such immune activation promotes hyperactivity of fibroblasts and damage to the vascular endothelium. The specificity of the disease is determined by generalized sclerosis of organs and tissues (skin, osteo-articular and muscular system, gastrointestinal tract, heart, lungs, kidneys) and the development of obliterating microangiopathy. This mechanism allows us to attribute systemic scleroderma to autoimmune diseases.

Classification

Today, the term "scleroderma" refers to a group of diseases with similar pathogenetic mechanisms that occur with local or generalized fibrosis of the connective tissue. From the standpoint of the international clinical classification, the following forms of scleroderma diseases are distinguished: systemic scleroderma, focal scleroderma, edematous scleroderma (scleredema Bushke), eosinophilic fasciitis, induced scleroderma, multifocal fibrosis, pseudoscleroderma.

Systemic scleroderma (diffuse or generalized scleroderma, progressive systemic sclerosis) can occur in several clinical forms:

  • Prescleroderma It has no dermatological manifestations and is accompanied only by Raynaud's phenomenon.
  • For diffuse scleroderma pathognomonic rapid development, damage to the skin, blood vessels, muscular-articular apparatus and internal organs during the first year of the disease.
  • Limited form proceeds with slow-developing fibrous changes, predominant lesion of the skin and late involvement of internal organs.
  • With scleroderma without scleroderma only visceral and vascular syndromes without typical skin manifestations are noted.
  • Cross shape may be manifested by a combination of systemic scleroderma with dermatomyositis, polymyositis, SLE, RA, vasculitis.

Systemic scleroderma can occur in a chronic, subacute and acute form. In chronic course over many years, Raynaud's syndrome serves as the only indication of the disease, other typical lesions develop gradually and for a long time. In the subacute variant of systemic scleroderma, skin-articular (scleroderma, polyarthritis, polymyositis) and visceral syndrome (cardiopulmonary) with minor vasomotor disturbances predominate. The acute form of pathology is characterized by rapid (within 12 months) formation of systemic fibrosis and microvascular disorders. There are three degrees of activity of systemic scleroderma: I - minimal, typical for the chronic variant, II - moderate, usually occurring in the subacute process, III - maximum, accompanying the course of acute and sometimes subacute forms.

Symptoms of systemic scleroderma

The clinical specificity of systemic scleroderma is polymorphic and polysyndromic manifestations. Options for the development of the disease can vary from mild forms with a relatively favorable prognosis to rapidly progressive diffuse lesions with an early fatal outcome. In the debut of systemic scleroderma, even before the appearance of specific lesions, there is a loss of weight, weakness, low-grade fever.

The earliest sign of the disease is Raynaud's syndrome, characteristic of 99% of patients and occurring with transient vasospasm paroxysms. Under the influence of stress or cooling, the fingers sharply turn pale, then the skin becomes bluish-purple. Vascular spasm may be accompanied by a feeling of coldness and numbness of the hands. After the resolution of vasoconstriction, the stage of reactive hyperemia begins: the skin becomes bright pink, there is a feeling of aching and pain in the fingers. Raynaud's phenomenon in scleroderma can be systemic, that is, it can spread to the vessels of the skin of the face, tongue, kidneys, heart, and other organs.

Skin syndrome present in most patients with systemic scleroderma. In its evolution, it passes through 3 phases: inflammatory edema, induration and skin atrophy. The initial stage is characterized by the appearance of a dense swelling of the skin of the hands and feet, accompanied by itching. Further, sclerodactyly (thickening of the skin of the fingers) develops, trophic ulcers are formed, nails are deformed. The frontal and nasolabial folds are smoothed, as a result of which the face acquires a mask-like expression. Due to the atrophy of the sebaceous and sweat glands, the skin becomes dry and rough, devoid of hair. Often found telangiectasia, depigmentation or hyperpigmentation of the skin, subcutaneous calcinates.

Musculo-articular syndrome also often accompanied by systemic scleroderma. Typical swelling and stiffness of the joints, arthralgia - this symptom complex is called sclerodermic polyarthritis. As a result of skin tightening, flexion contractures of the joints are formed, and tenosynovitis develop. Osteolysis of the nail phalanges is possible, leading to shortening of the fingers. Muscle damage in systemic scleroderma proceeds according to the type of polymyositis or non-inflammatory myopathy.

Visceral lesions can affect the digestive tract (90% of cases), lungs (70%), heart (10%), kidneys (5%). On the part of the digestive system is marked dysphagia, heartburn, nausea and vomiting. It develops reflux esophagitis, aggravated by the formation of ulcers and strictures of the esophagus. Against this background, patients with systemic scleroderma have an increased risk of forming Barrett's esophagus and adenocarcinoma. With the defeat of the small intestine diarrhea, flatulence, weight loss occurs, with the involvement of the large intestine - constipation and intestinal obstruction.

Damage to the lungs in systemic scleroderma can be expressed in the form of pulmonary fibrosis and pulmonary hypertension. Both syndromes show unproductive cough, progressive expiratory dyspnea, and respiratory failure. Lung damage is the leading cause of death in patients with systemic scleroderma, therefore, is regarded as a prognostic adverse factor. With cardiac involvement, arrhythmias, pericarditis (adhesive or exudative), endocarditis, heart failure can develop.

Renal syndrome with systemic scleroderma often occurs in the form of latent nephropathy with moderate functional impairment. However, a number of patients in the first five years from the debut of the disease develop a formidable, potentially lethal complication - acute scleroderma kidney, which proceeds with hyperreninemia, malignant arterial hypertension, thrombocytopenia and hemolytic anemia, rapidly increasing renal insufficiency. Among other syndromes of systemic scleroderma, polyneuropathy, Sjogren's syndrome, autoimmune thyroiditis, primary biliary cirrhosis of the liver, etc. are found.

Diagnostics

The American College of Rheumatology has developed criteria on the basis of which systemic scleroderma can be diagnosed. Among them, there is a large criterion (proximal scleroderma - hardening of the skin of the hands, face and torso) and small (sclerodactyly, digital scars, bilateral pneumofibrosis). If two small or one large symptom is detected, the clinical diagnosis can be considered confirmed. Differential diagnostic activities are carried out both within the group of scleroderma diseases and among other systemic diseases: Sjogren's syndrome, polymyositis, dermatomyositis, thromboangiitis obliterans, and many others. others

General clinical analyzes are uninformative, and the changes detected in them are not specific. On the blood side, hypochromic anemia, leukocytopenia or leukocytosis, a moderate increase in ESR is observed. In the general analysis of urine, proteinuria, leukocyturia, microhematuria can be detected.Biochemical indicators indicate signs of inflammation (increased seromucoid and fibrinogen, CRP, RF). The most important are the results of immunological examination. In systemic scleroderma, scleroderma autoantibodies markers are found in the blood: antibodies to Scl-70 and anti-centromeric antibodies.

Among the instrumental methods for the early diagnosis of systemic scleroderma, the capillaroscopy of the nail bed is the most valuable, which allows to identify the initial signs of the disease. To assess the condition of the bone system, radiography of the hands is performed. In order to detect interstitial pulmonary fibrosis, it is advisable to perform radiography and CT of the lungs. To study the digestive tract prescribed x-rays of the esophagus, radiography of the passage of barium through the intestines. Electrocardiography and echocardiography are necessary for the detection of cardiogenic lesions and pulmonary hypertension. Electromyography allows you to confirm myopathic changes. For histological verification of systemic scleroderma, a biopsy of the skin, muscles, kidneys, lungs, and pericardium is performed.

Treatment of systemic scleroderma

Persons suffering from systemic scleroderma should avoid stress factors, vibration, hypothermia, insolation, contact with household and industrial chemical agents, stop smoking and drinking caffeine, taking vasoconstrictor agents. Pharmacotherapy, its dosage and duration depend on the clinical form, activity and rate of disease progression, the severity of visceral lesions.

Pathogenetic therapy of systemic scleroderma is performed using vascular, antifibrosis and immunosuppressive drugs. To prevent episodes of vascular spasm and prevent ischemic complications, vasodilators (nifedipine, verapamil, diltiazem, cinnarizine, etc.), antiplatelet agents (acetylsalicylic acid, pentoxifylline) and anticoagulants (heparin, warfarin) are prescribed. D-penicillamine is used to suppress the development of systemic fibrosis. Anti-inflammatory therapy for systemic scleroderma includes taking NSAIDs (ibuprofen, diclofenac, nimesulide) and glucocorticoids. The drugs in this group help to reduce the signs of inflammation (myositis, arthritis, tendosynovitis) and immunological activity. To slow the progression of systemic fibrosis, methotrexate, cyclosporine, pulse therapy with cyclophosphamide can be used.

Symptomatic therapy for systemic scleroderma is aimed at reducing digestive disorders, heart failure, pulmonary hypertension. With the development of scleroderma renal crisis, captopril is prescribed, enalapril, in some cases hemodialysis may be required. Surgical treatment - thoracic sympathectomy - is indicated for the complicated form of Raynaud's syndrome.

The prognosis for systemic scleroderma is generally unfavorable. The lowest five-year survival rate (30–70%) is associated with a diffuse form. Predictors of a poor prognosis are pulmonary and renal syndromes, the debut of the disease in patients over 45 years of age. The limited form and the chronic course of the disease have a more favorable prognosis and better survival, with pregnancy planning and successful delivery. Patients with systemic scleroderma are subject to dispensary registration and observation every 3–6 months.

Limited scleroderma

Limited scleroderma is characterized by the appearance on the skin of pronounced, dense spots, hard to the touch, yellow-wax or porcelain color. Changes are limited only to the skin - the disease does not affect the internal organs.

Skin scleroderma is chronic, although it tends to spontaneously cure - skin changes persist up to several years, after which they atrophy (in rare cases they may be permanent).

The disease affects women and men equally often, unlike the systemic type, often occurs in children.

There are several varieties. limited scleroderma:

  • limited skin scleroderma - changes of various sizes and shapes, initially surrounded by an inflammatory boundary, after some time the border disappears, the changes begin to weaken, the changes can last for more than a dozen years,
  • recurrent limited scleroderma - changes are scattered throughout the skin, lasts longer
  • systemic scleroderma - changes include almost all skin
  • linear limited scleroderma - this form of the disease often leads to permanent contractures of the limbs
  • facial scleroderma - The changes include half of the face, giving severe and disfiguring symptoms.

Systemic scleroderma

Generalized scleroderma refers to systemic connective tissue diseases. It consists in progressive fibrosis of the skin and internal organs, which leads to their failure. Women suffer 3-4 times more often than men. The peak incidence occurs in the period between 3 and 5 decades of life.

In the clinical picture of systemic scleroderma prevail:

  • Raynaud's symptom, that is, a spasm of the arteries of the palm under the influence of cold or without any kind, which initially leads to blanching of the fingers, which after a while become bluish and numb, and then redden and “warm up”, this symptom may even be several years ahead of scleroderma.
  • Skin changesfibrosis and hardening of the skin may concern fingers (hardening of the skin on the fingers leads to fixation of their position, without the possibility of compression or extension, loss of sensation in the fingers contributes to frequent skin damage, which leads to difficult healing ulcers on the pads, in the late stages of the disease narrowing and disappearance of the nails occur) faces (the skin on the face is smoothed and hardens, which deprives her facial expressions, the face takes the form of a fixed mask), and shells (which leads to the formation of "hard armor" on the abdomen and chest).
  • Symptom "salt and pepper"consisting in the appearance of discolored spots on the skin that accompanies or precedes the hardening of the skin.
  • Skin calcificationmainly fingers, often calcium deposits make their way through the skin to the outside.
  • Pain syndromes: joint pain, swelling of the joints or morning stiffness in the joints, restriction of their mobility.
  • Atrophy of the papillae of the tongueleading to loss of sensation and taste perception.
  • Gingivitisleading to loss of teeth.
  • Fibrosis of the esophaguswhich leads to impaired swallowing.
  • Pulmonary fibrosis, manifested by a feeling of shortness of breath, chest pain, prolonged dry cough, leading to the development of dangerous pulmonary hypertension.
  • Myocardial fibrosis, which leads to conduction disorders and cardiac arrhythmias.

Systemic scleroderma has a difficult and slow process, often leading to a significant disruption of the vital activity of the body, many complications (especially from the gastrointestinal tract and lungs), as well as disability.

Scleroderma treatment

Modern medicine, unfortunately, does not have effective treatments for scleroderma. However, patient survival is increased by using treatment that reduces the effects of organ damage.

For this purpose, for example, cyclophosphamide is used in lung lesions or methotrexate in the case of joints. Nonsteroidal anti-inflammatory drugs are also recommended, which can reduce pain symptoms and slow down inflammatory changes in the joints.

In addition, penicillamine or gamma-interferon is used, which inhibits fibroblasts and reduces collagen production.

From the general recommendations: patients should provide an adequate level of skin hydration, protect hands and feet from the effects of cold, it is strictly forbidden to smoke cigarettes.

Tips for the sick

Patients with scleroderma may take several steps to alleviate the symptoms:

1. Stay active. Exercise keeps the body flexible and agile, improves blood circulation, reduces stiffness.

2. Do not smoke. Nicotine increases spasm of blood vessels, which is very bad for blood circulation. If you smoke and can not quit yourself - consult a doctor, because it is very important.

3. Prevent heartburn. Avoid foods that cause heartburn and gas formation. Do not eat before bedtime. Raise the upper body during sleep, so that gastric juice does not fall into the esophagus. Antacids also help to overcome heartburn.

4. Beware of the cold. Wear warm mittens and shoes to protect your limbs from the cold, as frost can aggravate vascular spasm.

Possible complications of scleroderma

The variety of complications of scleroderma is enormous. The severity of these complications can also vary from subtle problems to severe, life-threatening conditions.

Vasoconstriction in scleroderma can be so severe that insufficient blood circulation leads to tissue necrosis, mainly of the fingers and toes. As a result, amputation may be required.

Scarring of lung tissue (pulmonary fibrosis) can lead to a decrease in lung function, poor exercise tolerance, shortness of breath. Pulmonary hypertension may also develop - an increase in pressure in the arteries of the lungs.

When scleroderma affects the kidneys, the patient may develop hypertension and proteinuria - an increased protein content in the urine. This is due to impaired blood flow in the glomeruli. A more serious complication is renal scleroderma crisis - a sudden increase in blood pressure and kidney failure.

Scarring of the heart tissue increases the risk of arrhythmia and congestive heart failure, as well as causes inflammation of the pericardial bag - pericarditis.

Strong tightening of the skin around the mouth can lead to a noticeable reduction in the mouth, which makes it difficult even to clean the teeth. People with scleroderma do not produce enough saliva, which also increases the risk of caries. In addition, acid reflux destroys tooth enamel, and changes in the gums can lead to tooth loss.

Scleroderma affects all parts of the digestive system. This is manifested by difficulty in swallowing, poor digestion of food, acid reflux, persistent constipation with episodes of diarrhea.

Men with scleroderma may suffer from erectile dysfunction. Scleroderma can impair sexual function in women, resulting in impaired lubricant secretion and narrowing of the vaginal opening.

What is scleroderma?

Among autoimmune processes, scleroderma ranks second in terms of prevalence after lupus erythematosus. The disease is chronic and progressive. Skin and connective tissues undergo scarring, which leads to impaired tissue metabolism and blood circulation in small vessels. Collagen is formed in excess, sclerosed and hardened. In the systemic form of the disease, there is a serious damage to the internal organs.

Most often women suffer from scleroderma. The disease is usually diagnosed in middle age, but often skin lesions are detected in children or adolescents. There are 2 forms of scleroderma: focal and systemic. In the first case, the disease proceeds more easily.

Focal scleroderma

When focal scleroderma affects certain areas of the skin. Sometimes there are changes in the bones and muscles. Internal organs are not affected by the pathological process. No spasm of blood vessels, which is accompanied by numbness of the fingers (Raynaud's syndrome). The disease is often detected in childhood. More often this disease affects girls. Doctors give a relatively good prognosis for this type of scleroderma. Focal form of the disease is divided into several varieties:

  • Ring-shaped. Spots that look like plaques appear on the skin. They are brown.
  • Linear. White stripes of scar tissue appear on the face. They have the appearance of a line.
  • Blyashechnaya. Manifested in the form of redness of the skin due to the expansion of small blood vessels, as well as seals on the body, hands and feet. In the affected lesions, the secretion of the sebaceous glands stops, the skin pattern disappears and the hair falls out.
  • Idiopathic atrophoderma Pasini-Pierini. This rare form of the disease is observed in young women. Violet spots are formed on the back, rashes on the face are not typical for this type of scleroderma. Over time, the education on the body progresses.
  • Parry-Romberg face hemiatrophy. The disease begins in childhood or adolescence. On one half of the face, yellow or bluish spots are formed, which creates a strong cosmetic defect. The pathological process can affect not only the skin, but also the musculoskeletal tissue.

  • Scleroderma Bushke. Puffiness is formed on the skin of the face, neck and shoulders. They are tight and painful on palpation.
  • Teardrop ("white spots disease"). White spots appear on the face of a child or adult. Rashes are convex, with a pearly shade. The size of the formations is from 0.5 to 1.5 cm. Often this type of disease is combined with the patchy form of scleroderma. Photo spots can be seen in the photo.

Causes of disease

The causes of this disease are not fully understood. It is assumed that the development of pathology is influenced by genetic factors. There is a genetic predisposition to autoimmune diseases. This does not mean that the disease manifests itself immediately after birth, pathology can develop at any age.

In people with a tendency to scleroderma provoke the onset of the disease can the following factors:

  • past infections
  • hormonal disorders,
  • hypothermia (especially frostbite of hands and feet),
  • molecular mimicry of microorganisms, due to which there is an increased activity of lymphocytes,
  • hormonal disorders,
  • intoxication with drugs or chemicals
  • poor ecology of the place of residence,
  • work related to exposure (for example, in chemical production).

The environment and human health are closely interrelated. Very often, the development of scleroderma provokes living in a region with a large number of industrial enterprises. This is especially true of the juvenile form of the disease, which manifests itself at an early age. Silicon dioxide, white spirit, welding gases, ketones, solvents, trichlorethylene can have a harmful effect. Not every person whose work is associated with these substances, the disease will certainly develop. But with hereditary predispositions, the risk of scleroderma due to constant contact with chemical compounds is very high.

Skin symptoms

Symptoms of a disease depend on its variety. Skin changes go through several stages of development. First, there is a dense swelling, usually on the hands and face. Then the skin thickens and atrophic processes are observed in the later stages of the pathology.

The following external signs of the disease can be distinguished:

  • Raynaud's syndrome. Numbness, burning and tingling of the fingers and toes. Such phenomena are observed with even a slight freezing. The skin of the limbs becomes pale and bluish. This symptom is associated with spasm of the vessels of the arms and legs.
  • The skin on the fingers becomes rough. Scar tissue appears. Hand skin lesions are accompanied by thickening and deformation of the fingers. Because of this, the disease is often mistaken for rheumatoid arthritis.
  • Around the mouth can be seen piteto-like folds.
  • Pigmentation of the skin.
  • The feeling of constant chilliness, the patient often and quickly freezes.
  • With a drop-shaped form, white spots on the face of a child or adult become the first sign of the disease.
  • Mimics disappear. The face becomes smooth and mask-shaped, the nasolabial fold is almost invisible.

In addition, a person loses weight heavily, quickly gets tired and complains of a headache. Rashes appear on the body. Their color can vary from brown to red. The nature of the rash depends on the form of scleroderma. Photo spots can be seen in the photo above.

Manifestations of the internal organs

Systemic scleroderma affects the entire body. The patient may complain of negative manifestations from different systems. The most commonly observed changes are in the kidneys, lungs and heart. Often there are changes in the digestive organs, which is manifested by the following symptoms:

  • heartburn,
  • flatulence,
  • diarrhea,
  • ulceration on the walls of the stomach,
  • difficulty swallowing
  • peristalsis disorders
  • deterioration of food processing.

If the pathological process has affected the kidneys, then the following symptoms may occur:

  • rare urination,
  • protein and red blood cells in urine analysis,
  • arterial hypertension,
  • renal failure
  • disorders of consciousness.

Heart failure can occur with the following symptoms:

  • dysfunction of the left ventricle,
  • myocardial induration,
  • inflammation of the serous and muscular membrane of the heart,
  • dyspnea,
  • tachycardia,
  • heartache,
  • heart failure (rare).

If the lungs are involved in the pathological process, the following manifestations are observed:

  • cough without sputum,
  • shortness of breath on physical exertion
  • inflammation of the pleura and lungs with the formation of scars and seals,
  • increased risk of lung cancer.

In addition, peripheral nerve damage may occur. There are pains in the limbs and on the face along the trigeminal nerve. The patient complains of numbness or a violation of the sensitivity of the arms and legs. In severe cases, paresis (paralysis) is possible. On the part of the endocrine glands, a decrease in thyroid function (hypothyroidism) is possible.

Prognosis of the disease

Patients are often confused when they hear the diagnosis of scleroderma. What is it and how to treat this ailment? Such a question worries the sick. We can say that today this disease is not cured completely. To stop the increased formation of collagen is impossible. Modern therapy can only slow down the development of pathology.

The prognosis for focal scleroderma is more favorable than for systemic. On average, the survival rate for this disease is about 68% for 5 years. The effectiveness of treatment depends on the following factors:

  • Gender of the patient. The disease in men has a more unfavorable prognosis.
  • The age of the patient. Scleroderma is less amenable to treatment in people older than 45 years.
  • The degree of damage to the internal organs. If in the first 3 years of the disease the lungs and kidneys were involved in the pathological process, the prognosis of the course of the disease worsens.
  • A decrease in hemoglobin in the blood, an increase in ESR and the presence of protein in the urine indicates a severe form of the disease.
  • An adverse factor is the early onset of the disease before the age of 20 years.

Is scleroderma dangerous? Without treatment, restraining the development of the disease, this pathology can lead to very serious consequences. An autoimmune disease can cause life-threatening complications, such as renal and heart failure, pulmonary hypertension, and finger necrosis due to impaired blood circulation.

Alternative medicine

Often patients are interested in: can scleroderma be treated with folk remedies? Of course, it is impossible to completely heal from such a complex disease only with home remedies alone. The use of folk remedies is permissible only after consultation with the doctor, as an addition to the main therapy. The following recipes are used:

  • Aloe juice is applied in the form of compresses to the lesions. You can use ichthyol ointment.
  • Baked onions are crushed and mixed with one part of honey and two parts of kefir. This mixture is used for compresses.
  • Make a homemade ointment of melted pork fat and dry grass wormwood or celandine.
  • To restore the connective tissue consumed inside the decoction of horsetail, lungwort, knotweed.
  • In case of complications of the heart, decoction of adonis and cyanosis will help, and in case of kidney damage, compounds with bearberry and lingonberry leaf are useful.

New developments in the treatment of scleroderma

Medical scientists have long been studying the nature of scleroderma: what it is and how to treat this complex and serious illness. After all, traditional methods of therapy do not lead to the complete elimination of the disease. New methods of treatment are being sought, and drugs are being developed that can more effectively influence pathology.

A new genetically engineered drug "Totsilizumab" ("Actemra") has been created. This drug is an antibody against interleukin-6 receptors. It is this anti-inflammatory cytokine and is a provoking factor for the occurrence of scleroderma and rheumatoid arthritis. Clinical studies have shown the effectiveness of the drug.

A new complex method of therapy with the enzyme "Lidaza" in combination with the vitamin means "Pantogam", "Dexpanthenol" and "Pikamilon" is proposed. This method is effective in the focal form of the disease. Biologically active substances help to improve the metabolism in the affected tissues.

Currently, studies are underway on drugs that could block the signaling pathway for the development of scleroderma. Inhibitor molecules that can influence the pathogenesis of the disease are studied. This could help prevent fibrosis - the proliferation of connective tissue. New in the treatment of scleroderma is also associated with the use of stem cells. They are able to replace diseased cells. This leads to a decrease in fibrosis and the formation of new blood vessels instead of those that have been damaged by pathology.

Disease prevention

Due to the uncertainty of the exact causes of the disease, specific prevention of scleroderma has not been developed. People with a genetic propensity for autoimmune pathologies can be reduced by the following rules:

  • It is necessary to treat infectious diseases in a timely manner.
  • Avoid exposure to cold and harmful chemical factors.
  • Lead an active lifestyle to maintain in the tone of the musculoskeletal system and connective tissue.

Patients who have been diagnosed with scleroderma, you must get on the dispensary registration and regularly visit a rheumatologist. It is necessary to take the prescribed medication continuously, in many cases life-sustaining maintenance therapy is required.

Watch the video: Rheumatology - systemic sclerosis (December 2019).

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