Connective tissue is a fairly rare pathology. The clinical picture of this disease is characterized by a combination of signs of various collagen diseases. This pathology is otherwise called Sharpe's syndrome. Most often, this symptom complex is observed during puberty and in middle-aged patients. In its advanced form, the pathology can lead to serious and life-threatening consequences. In this article we will look in detail at the symptoms and treatment of mixed disease. connective tissue.

What is it

In the past, this pathology was very difficult to diagnose. After all, the signs of Sharpe's syndrome resemble manifestations of various rheumatic ailments. Only relatively recently has this disease been described as a distinct autoimmune disorder.

In mixed connective tissue disease (MCTD), the patient exhibits individual signs of various rheumatic pathologies:

• dermatomyositis;
• scleroderma;
• rheumatoid arthritis;
• polymyositis.

The patient does not necessarily have complete clinical picture all of the above diseases. Typically, several symptoms characteristic of various autoimmune pathologies are observed.

ICD code

According to ICD-10, mixed connective tissue disease is classified as separate group pathologies coded M35 (“Other connective tissue diseases”). Full code NWST - M35.1. This group includes cross rheumatic syndromes. The word “cross” means that with this pathology there are signs of various connective tissue diseases (collagenoses).

Reasons

At present, the exact causes of Sharp's syndrome are not clear. Mixed connective tissue disease is autoimmune in nature. This means that a person’s immune system, for unknown reasons, begins to attack its own healthy cells.

What can cause such a malfunction? protective forces body? Doctors suggest that it may affect the functioning of the immune system. long-term use some medicines. Play a major role in the occurrence of autoimmune reactions hormonal disorders and age-related restructuring of the endocrine system. For this reason, CTD is often observed in adolescents and women during menopause.

Negative emotional background can also affect work immune system. The psychosomatics of mixed connective tissue disease is associated with serious stress. This pathology is more often observed in people prone to depression, as well as in patients with neuroses and psychoses.

Usually observed in people with a hereditary predisposition to rheumatic diseases. Impact unfavorable factors is only a trigger for the occurrence of autoimmune lesions.

Symptoms

Mixed connective tissue disease occurs in chronic form and without treatment gradually progresses. This pathology is systemic, it affects not only the skin and joints, but the entire body.

Very often initial sign The disease becomes a violation of blood circulation in the fingers and toes. This resembles the manifestations of Raynaud's syndrome. Due to vascular spasm, a person’s fingers and toes become pale and cold. Then the skin on the hands and feet takes on a bluish tint. Coldness of the extremities is accompanied by pronounced pain syndrome. Such vascular spasms may occur several years before other signs of the disease develop.

Most patients experience joint pain. The fingers swell greatly and movements become painful. Muscle weakness is noted. Due to pain and swelling, it becomes difficult for the patient to bend his fingers and hold various objects in his hands. It's like initial manifestations rheumatoid arthritis or However, bone deformation very rarely occurs. In the future in pathological process Other articular joints are also involved, most often the knees and elbows.

Subsequently, the person develops red and white spots on the skin, especially in the area of ​​the hands and face. Condensed areas of muscles can be felt, as if the skin thickens, and in rare cases, ulcers appear on the epidermis.

The patient's health gradually worsens. Joint pain and skin rashes accompanied by the following symptoms:

• general weakness;
• a feeling of stiffness in the joints after a night's sleep;
• hypersensitivity to ultraviolet;
• drying of the oral mucosa and difficulty swallowing;
• hair loss;
• causeless weight loss with normal nutrition;
• increased temperature;
• enlarged lymph nodes.

In advanced cases, the pathological process spreads to the kidneys and lungs. Glomerulonephritis occurs and the protein content in the urine increases. Patients complain of chest pain and difficulty breathing.

Possible complications

Mixed connective tissue disease is quite dangerous pathology. If the pathological process affects internal organs, then the following complications may occur with poor treatment:

• renal failure;
• stroke;
• inflammation of the esophageal mucosa;
• perforation of the intestinal wall;
• myocardial infarction.

Such complications occur when the course of the disease is unfavorable and in the absence of proper therapy.

Diagnostics

CTD is treated by a rheumatologist. Symptoms of mixed connective tissue disease are extremely varied and resemble the manifestations of many other pathologies. Because of this, difficulties often arise in making a diagnosis.

Patients are prescribed a serological blood test for antibodies to nuclear ribonucleoprotein. If the indicators of this study exceed the permissible level and the patients have arthralgia and Raynaud's syndrome, then the diagnosis is considered confirmed.

Additionally prescribed next research:

• clinical and biochemical tests blood and urine;
• urine test according to Nechiporenko;
• analysis for rheumatoid factor and specific immunoglobulins.

If necessary, an ultrasound of the kidneys is prescribed, as well as a chest X-ray and an echocardiogram.

Treatment methods

Treatment of mixed connective tissue disease is aimed primarily at suppressing the autoimmune reaction. Patients are prescribed the following medications:

1. Corticosteroid hormones: Dexamethasone, Metipred, Prednisolone. These drugs reduce autoimmune reactions and inflammation in the joints.
2. Cytostatics: "Azathioprine", "Imuran", "Plaquenil". Takei medications also suppress the immune system.
3. Nonsteroidal drugs anti-inflammatory action: "Diclofenac", "Voltaren". They are prescribed for severe pain and swelling of the joints.
4. Calcium antagonists: Verapamil, Diltiazem, Nifedipine. These drugs are prescribed to prevent damage cardiovascular system.
5. Inhibitors proton pump: "Omeprazole." Patients with Sharp syndrome have to take medications for a long time, and sometimes for life. This can negatively affect the gastrointestinal tract. The drug "Omeprazole" helps protect the gastric mucosa from the aggressive effects of drugs.

This complex treatment prevents exacerbations of the disease and allows you to achieve stable remission.

It is important to remember that drugs for the treatment of CTD significantly reduce immunity. Therefore, patients need to protect themselves from contact with infectious patients and hypothermia.

Forecast

Does Sharpe syndrome affect life expectancy? The prognosis of this disease is considered conditionally favorable. Dangerous lesions of internal organs with CTD develop less frequently than with other autoimmune pathologies. Fatal outcome It is noted only in advanced forms of the disease and the presence of complications from the heart and kidneys.

However, it should be remembered that this disease is chronic and cannot be completely cured. Patients are often prescribed lifelong medication. If the patient adheres to the recommended treatment regimen, the prognosis of the disease is favorable. Timely therapy helps maintain a normal quality of life for the patient.

Prevention

Specific prevention This disease has not been developed, since the exact causes of autoimmune pathologies have not been established. Rheumatologists advise adhering to the following recommendations:

1. Should be avoided uncontrolled intake medicines. Long-term treatment with medications can only be carried out under the supervision of a doctor.
2. If there is a hereditary predisposition to autoimmune pathologies, unnecessary exposure should be avoided sunlight and go through regularly preventive examination see a rheumatologist.
3. It is very important to avoid stress as much as possible. Emotionally labile people must be taken sedatives and see a psychotherapist.
4. If you experience pain in the joints of the limbs and spasms of peripheral vessels, you should consult a doctor and undergo an examination.

These measures will help reduce the likelihood of autoimmune rheumatic pathologies.

13. AUTOIMMUNE CONNECTIVE TISSUE DISEASES - a group of acquired diseases with predominant damage to the fibrillar structures of connective tissue. In the past, this group of diseases was called collagen diseases, or collagenoses. Classificationally, they belong to the same group, since they reveal similar pathogenetic and clinical-anatomical criteria associated with immunological and inflammatory changes connective tissue. All these diseases share common clinical and pathophysiological parameters, and differential diagnosis between them is often difficult. In a number of cases, a pathological process is identified that includes symptoms of several nosological units, and therefore a new taxonomic form has been identified and documented - mixed autoimmune connective tissue disease. Common clinical and anatomical manifestations of this group of diseases are polyserositis, pancarditis (or one of its components), vasculitis, myositis, nephritis and skin changes(Table 8.1). Laboratory findings include autoimmune hemolytic anemia, thrombocytopenia, excess or deficiency of immunoglobulins, various autoantibodies (the diagnostic value of which is presented below), complement changes, false-positive syphilitic reaction, etc.

14. SOME IMMUNE CONNECTIVE TISSUE DISEASES.

Rheumatoid arthritis(M06.9). The symptoms necessary to establish a diagnosis are a constitutional syndrome, a gradual onset with a predominant involvement of small joints, centripetal and symmetrical progression, severe deformities (are a common manifestation). Rheumatoid factor is positive in the vast majority of cases.

Extra-articular manifestations include the presence of subcutaneous nodules, polyserositis, lymphadenopathy, splenomegaly, and vasculitis. X-ray revealed juxtaarticular osteoporosis, erosions articular surfaces and narrowing of joint spaces.

The pathogenesis of rheumatoid arthritis is associated with chronic systemic inflammation, mainly affecting synovial membranes. It occurs in 1-2% of the population, 3 times more often in women. In most cases, the disease manifests itself between the ages of 20 and 40. Sensitivity to rheumatoid arthritis has a genetic predisposition, since most patients exhibit human leukocyte antigen class 2.

The main macroscopic manifestation of rheumatoid arthritis is chronic synovitis with the development of pannus and then, as it progresses, the formation of fibrous ankylosis.

Systemic manifestations of rheumatoid arthritis are diverse and include damage to the heart, lungs, skin and blood vessels. Macroscopic changes with secondary involvement of organs are nonspecific and the diagnosis is established based on clinical laboratory and histological research methods. In the heart, granulomatous inflammation and fibrinous pericarditis are determined, in the lungs - nonspecific diffuse interstitial fibrosis, interstitial pneumonitis, chronic pleurisy and diffuse granulomatosis. The process can occur with varying intensity and lead to the development of decompensated pulmonary heart. Skin manifestations are represented by rheumatoid nodes - dense subcutaneous foci of a round shape.

Certain forms of rheumatoid arthritis: Felga's syndrome (RF + in combination with leukopenia and splenomegaly) and SSHLP disease - rheumatoid arthritis with fever and minor articular manifestations.

Systemic lupus erythematosus(M32). The symptoms necessary to make a diagnosis are the appearance of skin rash in areas of sun exposure, involvement of joints and multisystem manifestations, suppression of bone marrow hematopoiesis with a decrease in the level of all cellular components of the blood (leukopenia, erythropenia, thrombocytopenia), detection of antinuclear antibodies, high titer of antibodies to natural double DNA.

Mostly young women are affected (85% of all cases). In 90% of cases, systemic lupus erythematosus develops between menarche and menopause. For clinical course Spontaneous remissions and relapses are characteristic. The intensity of the disease varies significantly.

Hormonal, racial and genetic factors play a role in the pathogenesis of the disease. Violation of immunological tolerance is expressed in the formation of three types of autoantibodies - antinuclear, anticytoplasmic and antimembrane. The mechanisms of the formation of immune complexes and the direct destructive action of antibodies are described in detail in the relevant immunology manuals. In the United States, the white population is sick 4 times more often than African Americans. The disease shows 70% concordance in twins, and vertical transmission is more typical for females: if the mother has systemic lupus erythematosus, the probability of developing the disease in sons is 1:250, in daughters - 1:40.

Genetic mechanisms are associated with high concentration in patients with certain types of human leukocyte antigens - DR2 and DR3. It is necessary to differentiate between systemic lupus erythematosus and drug-induced lupus. The probability of occurrence of the latter varies significantly depending on the drug. Thus, it is highest during treatment with isoniazid, hydralazine, chlorpromazine, methyldopa, procainamide, and quinidine. There are four differential diagnostic features that help differentiate drug-induced lupus:

1) the frequency is the same in men and women;

2) nephritis and pathology of the central nervous system absent;

3) hypocomplementemia and antibodies to natural DNA are not detected;

4) symptoms disappear when you stop using the medicine.

Defeat gastrointestinal tract, especially the esophagus, is observed in the vast majority of cases of systemic sclerosis (synonymous with scleroderma) and is represented by diffuse atrophy of the mucosa and replacement collagenosis of the submucosal layer. In advanced cases, the lower esophagus is represented by a rigid tube, which naturally leads to multiple complications associated with reflux (metaplasia, development of Barrett's esophagus, high probability adenocarcinoma and aspiration pneumonia). Similar changes in the small intestine lead to the development of malabsorption syndrome.

Changes in muscular system boil down to inflammatory myositis, which does not reach the same intensity as with dermatomyositis/polymyositis and occurs only in 10% of cases.

In the joints, nonspecific non-purulent chronic synovitis is determined with the subsequent development of fibrosis and ankylosis. Naturally, the intensity of articular lesions is lower than in rheumatoid arthritis, but they can be significant, especially when secondary osteoarthritis is associated.

Macroscopic changes in the kidneys are nonspecific (pallor and focal variegation, increase in organ weight) and boil down to the development of vasculitis and subsequently nephrosclerosis. Changes in the lungs in the form of interstitial fibrosis and pulmonary hypertension are detected in 50% of cases and can reach moderate intensity, but are also nonspecific.

Immunological symptoms are represented by antinuclear, anti-Sd-70 and anti-centromere antibodies. Hematological changes are characterized by mild hemolytic anemia.

Nodular tanargerinth(M30) is a systemic vasculitis characterized by transmural necrotizing inflammation of small and medium-sized muscular arteries, involving the kidneys and visceral vessels. In this case, the pulmonary vessels remain uninvolved.

Traditionally, panarteritis nodosa is an autoimmune disease. The criteria necessary for diagnosis are polyangiitis of small and medium vessels or only medium vessels in the classic version of nodular panarteritis. Symptoms are nonspecific and include constitutional syndrome, mononeuritis, anemia and high ESR. A pathogenetic connection with hepatitis B or C is possible. Despite the absence of pulmonary vascular damage, frequent morphological symptoms are pulmonary hemorrhages and glomerulonephritis. Sensitive, but nonspecific sign is the presence of anticytoplasmic antibodies with a perinuclear distribution.

Macroscopic changes are multiorgan, but extremely non-specific, and therefore the diagnosis is made only on the basis of clinical, laboratory and histological criteria. AnP tineutrophil cytoplasmic antibodies are positive in 75-85% of patients, other immunological tests are negative. The clinic determined mild hemolytic anemia.

Dermagomyositis/polymyositis (idiopathic inflammatory myopathies) (IDM). Symptoms necessary for diagnosis are proximal muscle weakness, characteristic skin manifestations, high level creatine kinase and other muscle enzymes, specific histological picture and immunological disorders. Dermatomyositis/polymyositis are systemic diseases with unknown etiology.

Clinical guidelines clearly state the diagnostic criteria for systemic lupus erythematosus, and the diagnosis is considered reliable if 4 out of 11 existing criteria are identified.

In addition, when drug-induced lupus AnP tihistone antinuclear antibodies are detected, which are quite characteristic of this pathology. Special group immunological markers in systemic lupus erythematosus are associated with the formation of lupus anticoagulants and antiphospholipid antibodies. Their clinical and pathophysiological significance comes down mainly to a violation of the coagulation system (see section “Hypercoagulation syndromes”). In this case, the first group of antibodies predisposes to arterial thrombosis (less often venous), leading to the development of infarctions in the corresponding areas of the blood supply. Antiphospholipid antibodies are associated with a false-positive test for syphilis and are quite characteristic of recurrent venous and arterial thrombosis, recurrent miscarriages, thrombocytopenic syndrome with bleeding, and non-infectious endocarditis.

Macroscopic manifestations of systemic lupus erythematosus are polymorphic and nonspecific. Most often (in 85-100% of cases) the skin is involved ( skin rash and erythema) and joints (non-erosive synovitis with minor deformation), somewhat less often the kidneys (60-70%) (see Chapter 6 " Clinical pathology kidneys and urinary tract"), heart (see Chapter 2 "Clinical pathology of the cardiovascular system"), lungs (pleurisy, moderate interstitial fibrosis, pulmonary edema, hemorrhagic pulmonary syndrome).

Despite the polymorphism morphological changes the diagnosis is established only on the basis of clinical, laboratory and histological criteria.

Scleroderma(systemic sclerosis) (M34). Necessary criteria for diagnosis are: skin changes (thickening, telangiectasia, combination of pigmentation and vitiligo); Raynaud's phenomenon; multisystem manifestations (gastrointestinal tract, lungs, heart, kidneys); positive test for antinuclear antibodies.

Systemic sclerosis is chronic disease with characteristic involvement of the skin and internal organs. The etiology of the process is unknown; in the pathogenesis, the main importance is given to autoimmune processes and external influence silicates. Clinical manifestations manifest at the age of 30-50 years, women are affected 3 times more often than men. Clinically, systemic sclerosis manifests itself in two forms: limited (80%) and diffuse (20%).

Macroscopic changes can be detected in almost any organ and system, but the most characteristic is the involvement of the skin, gastrointestinal tract, musculoskeletal system and kidneys.

Most patients exhibit diffuse sclerotic atrophy of the skin, which begins in the distal extremities and spreads centrally. IN initial stages skin swollen and have a pasty consistency. Subsequently, the skin itself atrophies and becomes inseparable from subcutaneous tissue. The skin of the affected areas loses collagen, takes on a waxy color, becomes tense, shiny and does not fold. In the skin and subcutaneous tissue, the development of focal calcifications is possible, especially intense in limited scleroderma or CREST syndrome, including calcification of subcutaneous tissue, Raynaud's phenomenon, dysfunction of the esophagus, syndactyly and telangiectasia.

A number of authors regard dermatomyositis as polymyositis in combination with skin symptoms, others are of the opinion that it is various diseases. Dermatomyositis/polymyositis occurs in individuals of any age group, women get sick 2 times more often than men. In both diseases (forms of the disease), and especially in dermatomyositis, there is high risk emergence malignant tumors(probability about 25%). The level of creatine phosphokinase and aldolase is diagnostic and allows us to assess the effectiveness of therapy. Antinuclear antibodies are found in 80-95% of patients, they have high sensitivity, but nonspecific. Inflammatory myopathy in dermatomyositis/polymyositis is difficult to treat differential diagnosis, since it also occurs with other autoimmune diseases: systemic lupus erythematosus, systemic sclerosis, Sjogren's syndrome.

Autoimmune diseases– these are diseases associated with disruption of the functioning of the human immune system, which begins to perceive its own tissues as foreign and damage them. Such diseases are also called systemic, because, as a rule, the entire system or even the entire body is affected.

Nowadays, we often talk about new infections that pose a threat to all of humanity. This is, first of all, AIDS, as well as SARS ( atypical pneumonia), bird flu and other viral diseases. If we recall history, most dangerous viruses and the bacteria were defeated, largely due to stimulation of one’s own immune system (vaccination).

The mechanism of occurrence of these processes has not yet been identified. Experts cannot understand what is causing the negative reaction of the immune system to its own tissues. Injuries, stress, hypothermia, various infectious diseases, etc. can trigger a malfunction in the body.

Diagnosis and treatment of systemic diseases can be carried out by doctors such as a therapist, immunologist, rheumatologist and other specialists.

Examples

The most famous disease from this group is rheumatoid arthritis. However, this disease is by no means the most common autoimmune pathology. The most common autoimmune diseases thyroid gland- diffuse toxic goiter (Graves' disease) and Hashimoto's thyroiditis. By autoimmune mechanism they also develop diabetes mellitus Type I, systemic lupus erythematosus and multiple sclerosis.

Not only diseases, but also some syndromes can have an autoimmune nature. A typical example is chlamydia, a disease caused by chlamydia and sexually transmitted. With this disease, the so-called Reiter's syndrome can develop, which is characterized by damage to the eyes, joints and genitourinary organs. These manifestations are not associated with direct exposure to the microbe, but arise as a result of autoimmune reactions.

Reasons

In the process of maturation of the immune system, the main time of which falls on the period from birth to 13-15 years, lymphocytes - cells of the immune system - undergo “training” in the thymus and lymph nodes. In this case, each cell clone acquires the ability to recognize certain foreign proteins in order to fight various infections in the future.

Some lymphocytes learn to recognize the proteins of their body as foreign. Normally, such lymphocytes are tightly controlled by the immune system and probably serve to destroy defective or diseased cells of the body. However, in some people, control over these cells is lost, their activity increases and the process of destruction of normal cells begins - an autoimmune disease develops.

The causes of autoimmune diseases are not well understood, but existing information allows us to divide them into external And internal.

External causes are mainly pathogens infectious diseases or physical impact, for example, ultraviolet radiation or radiation. When a specific tissue is affected human body, they change their own molecules in such a way that the immune system perceives them as foreign. After an “attack” on the affected organ, the immune system causes chronic inflammation and, accordingly, further damage to one’s own tissues.

Another external reason is the development of cross-immunity. This happens when the infectious agent turns out to be “similar” to its own cells - as a result, the immune system simultaneously attacks both the microbe and the cells (one explanation for Reiter's syndrome in chlamydia).

Internal reasons are, first of all, gene mutations, transmitted by inheritance.

Some mutations can change the antigenic structure of a particular organ or tissue, preventing lymphocytes from recognizing them as “their own” - such autoimmune diseases are called organ-specific. Then the disease itself will be inherited (the same organs will be affected in different generations).

Other mutations can disrupt the balance of the immune system by disrupting the control of self-aggressive lymphocytes. Then a person, when exposed to stimulating factors, can develop an organ-nonspecific autoimmune disease that affects many systems and organs.

Treatment. Promising methods

Treatment for autoimmune (systemic) diseases involves taking anti-inflammatory drugs and drugs that suppress the immune system (they are very toxic and such therapy contributes to susceptibility to various types of infections).

Existing medications do not act on the cause of the disease, or even on the affected organ, but on the entire body. Scientists are striving to develop fundamentally new methods that will act locally.

The search for new drugs against autoimmune diseases follows three main paths.

The most promising method seems to be gene therapy, with the help of which it will be possible to replace a defective gene. However, before practical application gene therapy is still a long way off, and mutations corresponding to a specific disease have not been found in all cases.

If the cause turns out to be a loss of body control over the cells of the immune system, then some researchers suggest simply replacing them with new ones, first carrying out strict immunosuppressive therapy. This technique has already been tested and showed satisfactory results in the treatment of systemic lupus erythematosus and multiple sclerosis, however, it is still unknown how long this effect lasts, and whether suppression of the “old” immunity is safe for the body.

Perhaps, before others, methods will become available that do not eliminate the cause of the disease, but specifically remove its manifestations. These are, first of all, antibody-based drugs. They are able to block the immune system from attacking their own tissues.

Another way is to prescribe substances that take part in the fine regulation of the immune process. That is, we're talking about not about substances that suppress the immune system as a whole, but about analogues of natural regulators that act only on certain types cells.

Nowadays, a common reason for visiting a doctor is joint pain - rheumatism, Reiter's syndrome, arthritis. There are many reasons for the increase in incidence, including environmental disturbances, irrational therapy, and late diagnosis. Systemic connective tissue diseases, or diffuse connective tissue diseases, are a group of diseases characterized by a systemic type of inflammation various organs and systems, combined with the development of autoimmune and immune complex processes, as well as excessive fibrosis formation.

The group of systemic connective tissue diseases includes:

Modern rheumatology names the following causes of diseases: genetic, hormonal, environmental, viral and bacterial. For successful and effective therapy it is necessary to make a correct diagnosis. To do this, you should contact a rheumatologist, and the sooner the better. Today, doctors are armed with an effective SOIS-ELISA test system, which allows them to carry out high-quality diagnostics. Since very often the cause of joint pain is an infectious process caused by various microorganisms, its timely detection and treatment will not allow the development of an autoimmune process. After diagnosis, it is necessary to receive immunocorrective therapy while preserving and maintaining the functions of internal organs.

It has been proven that with systemic connective tissue diseases, profound disturbances of immune homeostasis occur, expressed in the development of autoimmune processes, that is, reactions of the immune system accompanied by the appearance of antibodies or sensitized lymphocytes directed against the antigens of one’s own body (autoantigens).

Treatment of systemic joint diseases

Among the methods of treating joint diseases are:
- medicinal;
- blockade;
- physiotherapeutic;
- therapeutic exercises;
- method manual therapy;
- .

Medicines that are prescribed to a patient for arthrosis and arthritis have, for the most part, an effect that is aimed only at relieving the pain symptom and inflammatory reaction. These are analgesics (including narcotics), non-steroidal anti-inflammatory drugs, corticosteroids, psychotropic drugs, and muscle relaxants. Ointments and rubs are often used for external use.
With the blockade method, the anesthetic device is injected directly into the source of pain - into trigger points in the joints, as well as into the places of the nerve plexuses.

As a result of physiotherapy, warming procedures reduce morning stiffness, ultrasound produces micro-massage of the affected tissues, electrical stimulation improves nutrition of the joint.
The joints affected by the disease need movement, so under the guidance of a doctor you need to choose an exercise program physical therapy and determine their intensity.

In recent years, manual therapy has become popular in the treatment of joint diseases. It allows for a transition from forceful methods to soft, gentle ones, which are ideal for working with pathologically altered periarticular tissues. Manual therapy techniques involve reflex mechanisms, the impact of which improves metabolism in the affected elements of the joint and slows down degenerative processes in them. On the one hand, these techniques relieve pain (reduce unpleasant symptom diseases), on the other hand, promote regeneration and trigger restoration processes in the diseased organ.

Surgical treatment is indicated only in extremely advanced cases. However, before resorting to surgery, it is worth thinking: firstly, surgery- this is always a shock for the body, and secondly, sometimes arthrosis is precisely the consequence of unsuccessful operations.

Mixed connective tissue disease (MCTD), also called Sharpe's syndrome, is an autoimmune connective tissue disease manifested by a combination of individual symptoms such systemic pathologies as SSD, SLE, DM, SS, RA. As usual, two or three symptoms of the above diseases are combined. The incidence of CTD is approximately three cases per hundred thousand of the population, affecting mainly females of mature age: for every one sick man there are ten sick women. CTD is slowly progressive. In the absence of adequate therapy, death occurs from infectious complications.

Despite the fact that the causes of the disease are not completely clear, it is believed established fact autoimmune nature of the disease. This is confirmed by the presence in the blood of patients with CTD large quantity autoantibodies to U1 ribonucleoprotein (RNP)-related polypeptide. They are considered to be markers of this disease. CTD has a hereditary determination: almost all patients have the presence of HLA antigen B27. When treatment is started on time, the course of the disease is favorable. Occasionally, CTD is complicated by the development of hypertension in the pulmonary circulation and renal failure.

Diagnosis of mixed connective tissue disease

Presents certain difficulties, since the FTA does not have specific clinical symptoms, sharing similarities with many other autoimmune diseases. General clinical laboratory data are also nonspecific. However, the FTA is characterized by:

• UAC: moderate hypochromic anemia, leukopenia, accelerated ESR.
• OAM: hematuria, proteinuria, cylindruria.
• Blood biochemistry: hyper-γ-globulinemia, appearance of RF.
• Serological study: increased ANF titer with speckled type of immunofluorescence.
• Capillaroscopy: sclerodermatous-changed nail folds, cessation of capillary circulation in the fingers.
• X-ray of the chest: infiltration of lung tissue, hydrothorax.
• EchoCG: exudative pericarditis, valve pathology.
• Pulmonary function tests: pulmonary hypertension.

An unconditional sign of CTD is the presence of anti-U1-RNP antibodies in the blood serum at a titer of 1:600 ​​or more and 4 clinical signs.

Treatment of mixed connective tissue disease

The goals of treatment are to control the symptoms of CTD, maintain the function of target organs, and prevent complications. Patients are advised to keep active image life, observe dietary restrictions. In most cases, treatment is carried out on an outpatient basis. The most commonly used drugs are NSAIDs, corticosteroid hormones, antimalarial and cytostatic drugs, calcium antagonists, prostaglandins, and proton pump inhibitors. The absence of complications with adequate supportive therapy makes the prognosis of the disease favorable.

Essential drugs

There are contraindications. Specialist consultation is required.

1. (synthetic glucocorticoid drug). Dosage regimen: in the treatment of CTD, the starting dose of prednisolone is 1 mg/kg/day. until the effect is achieved, then slowly (no more than 5 mg/week) reduce the dose to 20 mg/day. Further reduction of the dose by 2.5 mg every 2-3 weeks. up to a maintenance dose of 5-10 mg (indefinitely).
2. Imuran) is an immunosuppressive drug, a cytostatic. Dosage regimen: for CTD, it is used orally at the rate of 1 mg/kg/day. The course of treatment is long.
3. Diclofenac sodium (Diclonate P) is a non-steroidal anti-inflammatory drug with an analgesic effect. Dosage regimen: medium daily dose diclofenac in the treatment of CTD is 150 mg, after achieving therapeutic effect it is recommended to reduce it to the minimum effective (50-100 mg/day).
4. Hydroxychloroquine (,) is an antimalarial drug and immunosuppressant. Dosage regimen: for adults (including the elderly), the drug is prescribed in the minimum effective dose. The dose should not exceed 6.5 mg/kg body weight per day (calculated based on ideal, not actual body weight) and can be either 200 mg or 400 mg/day. In patients able to take 400 mg daily, the initial dose is 400 mg daily in divided doses. When obvious improvement is achieved, the dose can be reduced to 200 mg. If effectiveness decreases, the maintenance dose can be increased to 400 mg. The drug is taken in the evening after meals.