Connective tissue diseases diagnostics. Diffuse connective tissue diseases

Varieties connective tissue are found in many organs and systems of our body. They are involved in the formation of the stroma of organs, skin, bone and cartilage tissue, blood and vessel walls. That is why in its pathologies it is customary to distinguish localized ones, when in pathological process one type of this tissue is involved, and systemic (diffuse) diseases in which several types of connective tissue are affected.

Anatomy and function of connective tissue

In order to fully understand the severity of such diseases, one must understand what connective tissue is. This physiological system comprises:

• intercellular matrix: elastic, reticular and collagen fibers;
• cellular elements (fibroblasts): osteoblasts, chondroblasts, synoviocytes, lymphocytes, macrophages.

Despite its supporting role, connective tissue plays important in the functioning of organs and systems. She performs protective function organs from damage and keeps organs in normal position which allows them to function correctly. Connective tissue covers all organs and makes up all the fluids in our body.

What diseases are classified as systemic connective tissue diseases?

Systemic connective tissue diseases are pathologies of an allergic nature in which autoimmune damage to the connective tissue occurs different systems. They manifest themselves in variety clinical picture and are characterized by a polycyclic flow.

Systemic connective tissue diseases include the following pathologies:

• nodular periarthritis;

Modern qualifications also include the following pathologies in the group of these diseases:

• systemic vasculitis.

Each of systemic diseases Connective tissue is characterized by both general and specific signs and causes.

Causes

The development of systemic connective tissue disease is provoked hereditary cause, but this reason alone is not enough to trigger the disease. The disease begins to make itself felt under the influence of one or more etiological factors. They could be:

• ionizing radiation;
• drug intolerance;
• temperature effects;
• infectious diseases that affect the immune system;
• changes hormonal levels during pregnancy or;
• intolerance to certain medications;
• increased insolation.

All of the above factors can cause changes in the immune system that trigger. They are accompanied by the production of antibodies that attack connective tissue structures (fibroblasts and intercellular structures).

General signsAll connective tissue pathologies have common signs:

1. Features of the structure of the sixth chromosome that cause genetic predisposition.
2. The onset of the disease manifests itself with mild symptoms and is not perceived as a connective tissue pathology.
3. Some symptoms of the diseases are identical.
4. The disorders span multiple body systems.
5. Diagnosis of diseases is carried out according to similar schemes.
6. Changes with similar features are detected in tissues.
7. Indicators of inflammation in laboratory tests are similar.
8. One principle for the treatment of various systemic connective tissue diseases.

Treatment

When systemic connective tissue diseases appear, the rheumatologist determines laboratory tests the degree of their activity determines tactics further treatment. In milder cases, the patient is prescribed small dosages of corticosteroid drugs and. With an aggressive course of the disease, specialists have to prescribe higher doses of corticosteroids to patients and, if therapy is ineffective, supplement the treatment regimen with cytostatics.

When systemic connective tissue diseases occur in severe form, plasmapheresis techniques are used to remove and suppress immune complexes. In parallel with these methods of therapy, patients are prescribed a course of irradiation of the lymph nodes, which helps to stop the production of antibodies.

Particular close medical supervision is required for the management of patients with a history of reactions hypersensitivity to some medicines and food products, and .
When changes in blood composition are detected, relatives of those patients who are already being treated for systemic connective tissue pathologies are also included in the risk group.

An important component of the treatment of such pathologies is the patient’s positive attitude during therapy and the desire to get rid of the disease. Significant help can be provided by family members and friends of the sick person, who will support him and allow him to feel the fullness of his life.

Which doctor should I contact?

Diffuse connective tissue diseases are treated by a rheumatologist. If necessary, a consultation with other specialists, primarily a neurologist, is scheduled. A dermatologist, cardiologist, gastroenterologist and other doctors can provide assistance in treatment, since diffuse diseases connective tissue can affect any organ of the human body.

Friends, today we will talk to you about mixed connective tissue disease. Have you heard of this?

Synonyms: cross syndrome, overlap syndrome, Sharp syndrome.

What is this? Mixed connective tissue disease (MCTD) is a kind of syndrome in which there are signs various diseases connective tissue (analogy with a ball of thread). It can be various manifestations, dermatomyositis, as well as often accompanying (“dry syndrome”).

There has always been a lot of talk and theories around the FTA. The natural question is: what is this - an independent disease or some kind of atypical form already known illness connective tissue (for example, lupus, scleroderma, etc.).

Currently, the FTA is classified as independent diseases connective tissue, although sometimes a disease that debuts as a CTD “leaks” subsequently into typical diseases connective tissue. CTD should not be confused with undifferentiated connective tissue disease.

Prevalence not known exactly, presumably no more than 2-3% of the total mass of all connective tissue diseases. Mostly young women are affected (peak incidence 20-30 years).

Cause. A possible genetic role has been suggested due to the presence of familial cases of CTD.

Clinical picture.

Manifestations of the disease are very diverse and dynamic. At the onset of the disease, symptoms often predominate systemic scleroderma, such as: Raynaud's syndrome, swelling of the hands or fingers, intermittent joint pain, fever, lymphadenopathy, less often - typical for lupus skin rashes. Subsequently, signs of damage appear internal organs, such as hypotension of the esophagus and difficulty swallowing food, damage to the lungs, heart, nervous system, kidneys, muscles, etc.

Most frequent symptoms NFTZ (descending):

• Arthritis or joint pain
  
• Raynaud's syndrome
  
• Hypotension of the esophagus
  
• Lung damage
  
• Swelling of the hands
  
• Myositis
  
• Lymphadenopathy
  
• Skin lesions similar to SSD
  
• Defeat serous membranes(pleura, pericardium)
  
• Kidney damage
  
• Damage to the nervous system
  
• Sjögren's syndrome

Joints : unstable and migrating polyarthritis, migrating joint pain. Any joints (large, small) can be affected; the process is much more benign than with rheumatoid arthritis, for example.

Raynaud's syndrome- one of the earliest and most persistent manifestations.

Swelling of fingers and cyst th - soft, pillow-shaped swelling of the hands. Often observed in conjunction with Raynaud's syndrome.

Muscles: from mild and migrating muscle pain to severe damage as with dermatomyositis.

Esophagus: mild heartburn, difficulty swallowing.

Serous membranes and: pericarditis, pleurisy.

Lungs: shortness of breath, increased pressure in the pulmonary artery.

Leather: lesions are very diverse and variable: pigmentation, discoid lupus, typical “butterfly”, diffuse hair loss, skin lesions around the eyes (Gottron’s symptom), etc.

Kidneys: moderate proteinuria, hematuria (appearance of protein and red blood cells in the urine), severe nephritis rarely develops.

Nervous system: polyneuropathy, meningitis, migraine.

Diagnostics.

Pay great attention to laboratory diagnostics NWTA. May be: anemia, leukopenia, less often - thrombocytopenia, increase in ESR, rheumatoid factor, circulating immune complexes (CIC), AST, CPK, LDH.

Specific laboratory marker CTDs are antibodies to nuclear ribonucleoprotein (RNP), found in 80-100% of cases. When ANF is detected, a speckled type of glow is noted (granular, reticular).

Diagnosis is made based on symptoms and the presence of RNP.

Treatment.

The main therapy is hormones different doses depending on activity and clinical manifestations. The duration of therapy ranges from several months to several years. Cytostatics, NSAIDs, and symptomatic treatment can also be used.

Despite the fact that the FTA is an “explosive mixture” of such serious illnesses, such as SSc, SLE, dermatomyositis, etc., the prognosis is usually better than in patients with typical connective tissue diseases.

What are systemic connective tissue diseases? The list of diseases is extensive, the symptoms are numerous, and often contradictory or mild. To understand this, you will have to start from afar. Because this is a very broad group of diseases and syndromes, one way or another affecting almost all organs. Perhaps we should start by understanding what connective tissue is.

What is connective tissue

Connective tissue is not directly responsible for the functioning of the organ, but is part of any organ, making up from 60 to 90% of them. This unique substance is present in the body in four forms:

• hard (bone tissue);
• gel-like (cartilage tissue);
• fibrous (ligaments);
• liquid (blood, lymph, cerebrospinal fluid, synovial fluid- fluid that fills the joints, intercellular fluid– plasma, etc.);

From the above it is clear that connective tissue is very important for the body - wherever you poke, you will definitely find one type or another of connective tissue. Therefore, the list of diseases and pathologies of connective tissue can stretch to many points.

What are systemic diseases

Systemic diseases affect all tissues of an organ or even several organs. Systemic diseases, according to some experts, are the price to pay for a civilized lifestyle. Our ancestors, and not so distant ones, for example, possible witnesses to the events of two thousand years ago in Jerusalem, did not know about the existence of systemic diseases and did not suffer from them.

A systemic, or complex type of disease is associated with the functioning of the immune system, or rather, with a failure in its normal functioning. Now we come to autoimmune diseases.

What are autoimmune diseases

Those who watched the series "House M.D." probably noticed that during the discussion of the diagnosis by House's team, when everyone possible illnesses were listed, the phrase always came up: “it’s probably something autoimmune.” What kind of scourge is this? modern man, which did not bother the Roman legionnaires at all and from which, according to WHO, more than 350 million people suffer today?

Let's remember how immunity works. The bone marrow produces a special type of cell - lymphocytes. The generated lymphocytes enter the bloodstream and then to the places of their maturation - the thymus gland and lymph nodes (because Bone marrow produces only a semi-finished product - immature lymphocytes). Those cells that matured in thymus gland(thymus), are called T-lymphocytes, and those located in lymph nodes– B-lymphocytes. The purpose of both is to create antibodies to fight various infections and foreign tissues. It is thanks to this mechanism that we, having had mumps or measles in childhood, no longer get sick from these infectious diseases.

B-lymphocytes, in the course of their opposition to strangers, join them, forming an immune complex. After the antigen is destroyed, the immune complex disintegrates under the influence of special substances. But sometimes the slender immune system makes a mistake, and the lymphocytes are white blood cells– quite decent cells begin to be considered foreign and dangerous own body. And they begin to attack these cells with all their lymphocyte passion.

The mechanism of self-destruction is launched - the body begins to fight with itself and, admittedly, very effectively. Or, for some reason, the body does not produce enough substances that contribute to the breakdown of the immune complex of B-lymphocytes and the defeated pathogen. So they hang out - the criminal element, and the lymphocytes that neutralized it, “connected by one chain.” These pathological complexes, being everywhere in the circulatory system, can settle in tissues various organs, causing all kinds of systemic pathologies.

Depending on the genetic predisposition, adsorption of pathogenic complexes occurs in different organs. If there is a predisposition, they will settle in the joints, causing arthritis, or in the tissues of the kidneys - that’s glomerulonephritis. In the case of attractiveness of the walls of blood vessels - vasculitis, in skin– scleroderma and other dermatitis. If brain tissue is affected - Parkinson's disease, chorea, Alzheimer's disease.

The cause of an autoimmune disease can be several factors:

• own gene mutations;
• impact environment- the notorious " bad ecology", radiation, ultraviolet radiation;
• allergies;
• protracted, complex infections;
• modifications (mutations) of pathogens - their cells become similar to our own, only modified, sick, which means, according to lymphocyte orderlies, healthy ones are also subject to destruction - according to the principle of similarity.

Medicine has not yet explained the nature of its occurrence. autoimmune diseases. Perhaps one of the factors is hormonal disorders– this is why women most often suffer from autoimmune diseases childbearing age; during the period of possible hormonal imbalances– during menopause; in children - during puberty.

Vivid examples demonstrating what systemic connective tissue diseases of an autoimmune nature are are systemic lupus erythematosus, some vasculitis, scleroderma, rheumatoid arthritis and many other ailments.

Diagnostics

Often the symptoms of a systemic autoimmune disease are vague and similar to those of other diseases. Diagnosis of a systemic autoimmune disease is possible using tests: it is known which groups of antibodies indicate the presence of an autoimmune disease. But the vagueness of the symptoms makes it difficult to decide on the need for analysis, or the analyzes look at completely different indicators. If you notice the following symptoms:

• pain, swelling and numbness in the joints, especially small ones - on the fingers and toes;
• feeling of tightness in the chest;
• drowsiness;
• general muscle weakness;
• lethargy;
• cold fear;
• heat intolerance;
• unquenchable thirst;
• frequent urination;
• increased appetite;
• decreased appetite;
• weight loss;
• sudden weight gain;
• heartache;
• skin rashes;
• changing skin;
• decreased skin sensitivity;
• sweating;
• menstrual irregularities;
• increased dryness of mucous membranes;
• Any other condition that is uncharacteristic of you, recurring or ongoing, consult a specialist.

Which doctors should I contact?

There are many described signs, and some of them are contradictory (for example, fear of cold or intolerance to heat). As we have already found out, systemic connective tissue diseases can have the most different manifestations, so it is required comprehensive examination. Contact your therapist first, and if he cannot make a diagnosis, visit a dermatologist, rheumatologist, endocrinologist, hematologist.

Also visit a cardiologist and nephrologist. Depending on the type of disease, the doctor will decide on treatment. You shouldn’t get carried away with home-grown therapy like “drink vitamins or dietary supplements.” Of course, this won’t make it worse, but it won’t make it any better. By the way, one of the signs of the presence of “something specifically autoimmune” is considered to be ineffectiveness, lack of improvement, when taking vitamins, a complex of microelements, adaptogenic drugs (ginseng, sea buckthorn, etc.).

If you receive a diagnosis of “something autoimmune,” you should understand that the prescribed treatment can relieve the symptoms of a systemic disease. But it is not able to restore immunodeficiency - to return the immune system to the possibility of proper and effective functioning.

It is also important to understand: having realized early stage, until the body destroys itself, fighting emerging pseudo-infections (its own cells) and becoming overfilled with pathogenic complexes (unbroken bundles of B-lymphocytes and harmful pathogens), it is possible to slow down the development pathological conditions caused by systemic diseases.

Autoimmune diseases are diseases associated with dysfunction of the human immune system, which begins to perceive own fabrics, as alien, and damage them. Similar diseases also called systemic, because, as a rule, it affects the whole system or even the entire body.

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 others 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 negative reaction immune system on 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 known 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 are also developing diabetes 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 may 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.

Causes

In the process of maturation of the immune system, the main time of which occurs from the birth of a person 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 begins normal cells– 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, such as 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 cause 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 causes are, first of all, gene mutations that are inherited.

Some mutations can change antigenic structure a certain 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 of autoimmune (systemic) diseases involves taking anti-inflammatory drugs and drugs that suppress immune system(they are very toxic and such therapy promotes susceptibility to various kinds 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.

Gene therapy seems to be the most promising method, with the help of which it will be possible to replace 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.