Collagenosis: symptoms, diagnosis, treatment. Causes of collagenosis development, its diagnosis and treatment Collagenosis symptoms and treatment

Collagenosis is a systemic pathology characterized by the destruction of connective tissue and a progressive course. The development of the disease is based on an immunopathological process that disrupts collagen metabolism. It may be congenital or acquired. Pathology is observed at any age; in children, such diseases are also common and are especially severe.

The concept of “collagenosis” unites a group of diseases with the same functional changes in the structure of connective tissue. They differ in the degree of severity of symptoms and the involvement of various organs and tissues in the process.

Diagnosing collagenoses is difficult, as they are characterized by a variety of clinical manifestations. Therefore, treatment of pathology must be individual. Only with timely initiation and correctly chosen therapy is it possible to achieve stable remission. And without treatment, collagenosis often leads to death.

Development mechanism

This pathology is a systemic process, since connective tissue is, to one degree or another, part of all organs. Blood vessels, cartilage tissue, and skin are formed from it. It is also located between the organs. Connective tissue makes up more than half of the total human body weight. Therefore, its pathological change affects the state of the whole organism.

Mainly with collagenosis, damage to the skin, blood vessels and joints is observed. This happens because it is the tendons, bones, cartilage tissue and dermis that contain collagen protein in large quantities. It ensures the elasticity of fabrics and their strength. With collagenosis, the structure of this protein and its functions are disrupted. Hyaluronic acid, which makes collagen so strong and elastic, is especially affected. As a result of this, it begins to break down, which leads to tissue damage and deterioration of organ functions.

This happens gradually under the influence of pathological changes in the immune system. Due to provoking factors, pathogenic immune complexes are formed in the body. They trigger an autoimmune process, which is characterized by the development of allergies and the production of antibodies to one’s own tissues, especially to the cellular structures of collagen. It is accompanied by the destruction of connective tissue structures.

With collagenosis, collagen begins to break down, which provides the strength and elasticity of connective tissue.

Classification

Collagenoses are a whole group of diseases that are also called diffuse connective tissue diseases. They manifest themselves in different ways, but all are characterized by a disruption of the collagen structure. These include diseases such as periarteritis nodosa, scleroderma, rheumatoid arthritis, systemic lupus erythematosus and others. All these diseases can be divided into two groups according to the characteristics of the origin of degenerative connective tissue damage.

The first includes congenital pathologies, the causes of which are hereditary disorders in the structure of collagen, as well as in metabolic processes. Such diseases are quite rare and sometimes difficult to detect. These include mucopolysaccharidosis, Marfan syndrome, elastic pseudoxanthoma, Stickler syndrome, osteogenesis imperfecta and others. Sometimes they are so severe that they lead to disability of the patient almost from birth.

All acquired forms of collagenosis differ so much in their course that the international classification divides them into two groups. Diseases with a severe course and general damage to connective tissue throughout the body include dermatomyositis, systemic lupus erythematosus, scleroderma, Sharpe's syndrome, periarteritis nodosa. All other diseases, for example, rheumatism or septic endocarditis, are called minor collagenoses, which are characterized by a latent course, slow progression and the absence of severe complications.

Sometimes collagenosis appears immediately after birth due to hereditary genetic pathologies

In addition, several types of pathology can be distinguished according to the location of the lesion:

• joint diseases - rheumatoid and psoriatic arthritis, spondylitis, osteoarthritis;
• damage to the walls of blood vessels or systemic vasculitis - microscopic polyangiitis, periarteritis nodosa;
• diffuse changes in connective tissue throughout the body, for example, systemic lupus erythematosus.

Causes

Congenital collagenosis develops due to hereditary genetic pathologies. As a result, collagen synthesis, its structure, and metabolic processes are disrupted during intrauterine development. Therefore, diseases are often observed in children, one of whose parents suffers from this pathology.

The causes of acquired collagenoses are less studied. The most common theory is about the autoimmune nature of these diseases. But there are several factors that can provoke changes and give rise to the development of pathology:

• disruptions in the functioning of the endocrine glands, hormonal imbalances, especially during pregnancy, menopause or puberty;
• infectious diseases, especially measles, rubella, herpes, cytomegalovirus, streptococcus or staphylococcus;
• prolonged exposure to stress, strong feelings;
• vaccination in children;
• lack of substances involved in collagen synthesis or improper functioning of enzymes;
• dysfunction of the immune system;
• allergic diseases;
• serious injuries.

Sometimes the trigger for the onset of the disease can be external factors. For example, excessive exposure to solar radiation exacerbates degenerative processes with a congenital predisposition to them.

Most collagenoses are accompanied by serious damage to the skin and joints

Symptoms

A large number of diseases have been combined into one group due to the fact that they have common symptoms. This is a slow progression with alternating periods of exacerbations and remissions. With systemic involvement, the disease is often fatal.

There are also general symptoms of collagenosis, which are characteristic of all such pathologies:

• nonspecific fever with hyperhidrosis and chills;
• damage to the skin and mucous membranes, the appearance of rashes or ulcers;
• joint pain, myalgia, impaired motor function;
• severe weakness, worsening mood;
• hydroxyprolinuria - an increase in the level of hydroxyproline in the urine due to changes in the structure of collagen;
• There may be disruptions in the functioning of the digestive, cardiovascular system, lungs or kidneys.

Other manifestations of collagenosis vary depending on the disease. Joint pain or skin rashes may be more pronounced, and disturbances in the functioning of internal organs may be observed.

Scleroderma

One of the most characteristic systemic pathologies of collagen structure is scleroderma. This disease is characterized by thickening and hardening of connective tissue. When it affects only the skin, it does not pose a threat to the patient’s life, although the skin becomes rough, abscesses appear, and hair may fall out. But sometimes systemic scleroderma develops, which affects the muscles, gastrointestinal tract and other organs. The outcome of the disease in this case is often unfavorable.

Serious damage to the skin is observed with scleroderma - it becomes coarser and thickens

Systemic lupus erythematosus

Systemic lupus erythematosus is a severe autoimmune disease that mainly affects young women. Characteristic symptoms of the pathology are redness of the cheeks, nose and décolleté, as well as rashes in the form of red rings throughout the body. Skin damage increases with stress and temperature fluctuations. The patient is also concerned about pain in muscles and joints, inflammation of the mucous membranes, and renal failure. Arthritis of the hands and feet, aphthous stomatitis develop, and vision deteriorates.

Rheumatoid arthritis

This disease is characterized by damage to the knees, as well as small joints of the hands and feet. In this case, the synovial membrane is destroyed, due to which the joint is deformed and therefore cannot perform its functions. The patient experiences severe pain, especially at night, and morning stiffness. Weakness, insomnia gradually develops, and weight changes are observed. This disease is one of the most common among collagenoses.

Periarteritis nodosa

This collagenosis belongs to systemic vasculitis. It primarily affects the arteries, but soft tissues and internal organs can also be destroyed. This disease is characterized by necrosis of small and medium-sized vessels with the formation of microaneurysms. Because of this, the kidneys and heart are seriously affected. External manifestations of the disease include a characteristic rash and the appearance of subcutaneous nodules.

Collagenoses can affect blood vessels, leading to their fragility

Hereditary diseases

Congenital systemic pathologies are quite rare, but are mainly characterized by a severe course. Ehlers-Danlos syndrome has symptoms characteristic of collagenosis. With this disease, the skin and joints become overly elastic. Due to the fragility of the blood vessels, frequent bruising, abnormal growth of scar tissue, and increased bleeding of the gums are observed.

Epidermolysis bullosa is accompanied by a very severe course. With this disease, the patient's skin is so fragile that any touch leads to damage. The respiratory tract, digestive tract, and internal organs are also affected. Serious disability in most cases is also caused by osteogenesis imperfecta, in which disruption of the collagen structure causes increased bone fragility.

Diagnostics

Due to the nonspecificity of the manifestations of these pathologies, their diagnosis can be difficult. It is not always possible to determine the disease by external symptoms alone, so a full examination is necessary. The main method of diagnosis is laboratory blood tests and tissue biopsy. Nonspecific markers of inflammation, increased ESR and C-reactive protein levels are detected in the blood. These signs indicate the presence of connective tissue damage.

For more accurate diagnosis and determination of its location, radiography, CT, MRI, and ultrasound are used. But the most informative is histological analysis of tissues. A rheumatologist deals with the differential diagnosis of various types of collagenosis. Sometimes you also need to consult a pulmonologist, cardiologist, dermatologist or other specialists.

For a favorable outcome of the disease, it is very important to make the correct diagnosis in time.

Treatment

Patients with collagenosis often require lifelong therapy. In most cases, such diseases are incurable, but it is possible to slow down the pathological process and significantly improve the patient’s condition. Treatment is prescribed individually according to the type and severity of the disease. Treatment methods also differ in the acute period and during remission.

In case of exacerbation, symptomatic drug therapy is indicated. These are most often non-steroidal anti-inflammatory drugs, for example, Brufen. With high activity of the inflammatory process, rapid progression of symptoms, prescribed glucocorticoids. The most commonly used is Prednisolone in a daily dose of 15 mg or Dexamethasone. And if the patient experiences side effects from such treatment, it is permissible to use cytostatics: Cyclophosphamide or Azathioprine. Also in the hospital, when stopping acute attacks of collagenosis, detoxification methods are used, for example, plasmaphoresis or hemosorption.

Correctly chosen treatment can significantly increase periods of remission. During this time, treatment is not interrupted. Rehabilitation therapy is indicated for patients: electrophoresis, phonophoresis, ultrasound, magnetic therapy, physical therapy. Balneotherapy is effective for increasing the period of remission: radon, carbon dioxide or hydrogen sulfide baths. To prevent exacerbations, it is necessary to follow the correct lifestyle and diet. Hypothermia, sun exposure, infectious diseases, injuries and stress must be avoided. Necessary promptly treat inflammatory diseases, eliminate chronic foci of infection in the body. It is very useful for strengthening immunity and general health for patients. Spa treatment.

Treatment of collagenosis is often lifelong, its goal is to increase the period of remission and prevent complications

Complications

Large collagenoses are the most severe. They are often complicated by serious damage to the liver, kidneys, and heart. As a result, the patient's death may occur. Most often this occurs due to cardiovascular failure. Cardiosclerosis, pericarditis, myocarditis, and angina pectoris may appear. Sometimes myocardial infarction develops. Respiratory failure also increases rapidly due to progressive pleurisy pneumonia or pulmonary fibrosis. In most cases, collagenosis severely affects the kidneys. In addition to chronic renal failure, amyloidosis and hematuria develop.

Collagenosis also causes complications in the gastrointestinal tract. Necrosis of the walls of the abdominal cavity may develop, accompanied by severe pain and bleeding. Complications also include visual impairment, and in the most severe cases, cerebral infarction develops.

These systemic diseases are quite severe, and without treatment they often lead to the death of the patient. Therefore, if there is a predisposition to metabolic disorders or immune pathologies, it is necessary to avoid provoking factors, to prevent the development of infection in the body, hypothermia or prolonged exposure to the sun. Treatment should be started as early as possible and not interrupted, following all the doctor’s recommendations. Then you can achieve long-term remission and improve the patient’s standard of living.

Collagenosis is an immunopathological condition in which the development of degenerative disorders is observed, which most often affect connective tissue, but the possibility of involvement of other segments in the pathology cannot be excluded.

Such a disease can develop primary and secondary, which is why the provoking factors in each specific case will differ. In addition, the main place in the development of such a disease is also given to the penetration of pathological agents into the human body.

Despite the existence of a large number of ailments that are included in the group of collagenoses, they all have a number of common clinical manifestations. These include fever and chills, increased sweating and unexplained weakness, as well as skin rashes of various forms.

The process of establishing the correct diagnosis is aimed at conducting a wide range of laboratory and instrumental examinations, which must be supplemented by a thorough physical examination.

In the vast majority of situations, in order to treat the disease, they turn to conservative methods based on oral medication.

Etiology

As mentioned above, collagenoses can be congenital or acquired.

In the first case, genetically determined pathologies that develop at the stage of intrauterine development of the fetus serve as a predisposing factor, against the background of which there is a disruption in the process of collagen synthesis, as well as the structure of this substance and its metabolism. It is for this reason that diseases included in this category very often develop in those children whose parents suffer from a similar illness.

The sources of acquired collagenoses have not been fully studied, but infection of the human body by pathogenic microorganisms comes to the fore. In this case, the causative agent may be:

• viruses;
• provocateurs of such pathologies as and;

A relationship has also been established between collagenosis and hormonal imbalances. Thus, the reasons can be identified:

• period of bearing a child;
• the course of menstruation;
• labor activity;
• menopause;
• abortive termination of pregnancy;
• disruption of the functioning of the organs of the endocrine system, namely the thyroid gland and ovaries, adrenal glands and pituitary gland.

If there is a genetic predisposition, the following may serve as a trigger:

• prolonged effects on the body of excessively low or high temperatures;
• prolonged stress;
• wide range of injuries;
• uncontrolled use of certain medications;
• vaccination;
• various allergic reactions;
• decreased resistance of the immune system.

Sometimes it is impossible to determine the factors that contributed to the development of such a disease - in such cases, a person is diagnosed with undifferentiated collagenosis.

Classification

In accordance with the peculiarities of the origin of the degenerative process, collagenoses are:

• congenital;
• acquired.

• Stickler syndrome;
• epidermolysis bullosa;
• osteogenesis imperfecta;
• Ehlers-Danlos disease;
• elastic pseudoxanthoma.

As for acquired forms of collagenosis, they differ in their course:

• with severe course;
• with a hidden course, slowly progressive development and the absence of life-threatening consequences.

The first group of pathologies includes:

• Sharp's syndrome;
• systemic lupus erythematosus.

The following types of collagenosis are characterized by a milder course:

• spondylitis;
• septic endocarditis;
• or .

Such diseases are combined into a category called minor collagenoses.

In addition, there is a classification depending on the location of the lesions:

• diffuse changes in connective tissue – most often diagnosed;
• joints;
• synovial membrane;
• vessel walls.

Also, specialists in the field of rheumatology distinguish mixed and transitional collagenoses.

Symptoms

The presence of a large number of ailments in such a group of diseases is due to the fact that they have many common clinical signs and characteristic features, for example, slow progression, as well as alternating phases of exacerbation and remission.

Common symptoms that develop in children and adults include:

• nonspecific fever;
• increased sweating;
• chills;
• inflammatory or ulcerative lesions of the skin and mucous membranes;
• joint and muscle pain;
• motor dysfunction;
• progressive weakness;
• frequent mood changes;
• sleep disturbance;
• decreased or complete lack of appetite;
• disruption of the functioning of the digestive and cardiovascular systems, as well as organs such as the kidneys and lungs.

In cases of scleroderma development, the above signs will be supplemented by:

• thickening and hardening of connective tissue;
• roughening of the skin;
• increased hair loss;
• the appearance of abscesses.

The characteristic signs of systemic lupus erythematosus are considered to be:

• redness of the skin on the face and décolleté;
• the appearance of rashes throughout the body, resembling red rings in appearance;
• temperature fluctuations;
• decreased visual acuity;
• bones.

With rheumatoid arthritis, patients most often complain of:

• damage to the joints of the upper and lower extremities;
• deformation of the affected segment;
• strong, prone to intensification at night;
• complete lack of sleep;
• weight loss.

All of the above diseases and their manifestations are among the most frequently diagnosed among collagenoses.

Diagnostics

An experienced rheumatologist will be able to make the correct diagnosis at the stage of the initial examination of the patient, however, laboratory and instrumental examinations are necessary to determine the type of collagenosis.

First of all, the clinician must personally perform several manipulations, namely:

• study the medical history of not only the patient, but also his close relatives - to find the most likely etiological factor for a particular person;
• collect and analyze life history to establish the influence of other unfavorable sources;
• conduct a thorough physical examination, with mandatory examination of the condition of the skin and mucous membranes;
• interview the patient in detail to draw up a complete clinical picture and the severity of the symptoms of collagenosis.

Among the laboratory tests it is worth highlighting:

• general and biochemical blood test;
• enzyme immunoassay and DNA blood tests;
• serological tests.

The most informative are the following instrumental procedures:

• ultrasonography and radiography of the affected segment;
• ECG and EHLCG;
• CT and MRI;
• biopsy of the skin, muscles and joints, as well as kidney tissue.

Not only a rheumatologist, but also specialists from other fields of medicine take part in the diagnostic process, in particular:

• dermatologist;
• gastroenterologist;
• pulmonologist;
• cardiologist;
• immunologist;
• endocrinologist;
• pediatrician.

Additional examinations are necessary to differentiate different forms of collagenosis.

Treatment

Given that a large number of diseases are characterized by a slowly progressive and relapsing course, their therapy should be gradual, long-term, and often lifelong.

In the vast majority of situations, the following medications are used to treat diseases:

• steroid glucocorticoids;
• non-steroidal anti-inflammatory substances;
• Cyostatics;
• gold preparations;
• aminoquinoline derivatives.

The daily rate and duration of use are determined individually for each patient, this is due to the fact that the doctor takes into account:

• type of illness;
• severity and severity of the course;
• age category of the patient;
• individual characteristics of patients.

During an exacerbation of symptoms, it is advisable to use the following extracorporeal methods of hemocorrection:

• plasmapheresis;
• cascade plasma filtration;
• hemosorption.

When a particular form of collagenosis is in remission, treatment may include:

• completing a physical therapy course;
• physiotherapeutic procedures - in this case represented by medicinal electrophoresis and magnetotherapy, ultrasound and ultraphonophoresis, therapeutic baths, which can be radon, carbon dioxide and hydrogen sulfide;
• maintaining a gentle diet;
• spa therapy.

Possible complications

Depending on the type of collagenosis, in the absence of therapy, there is a high probability of developing the following complications:

• , or cardiovascular failure;
• or ;
• or pneumosclerosis;
• or ;
• or ;
• hemorrhages in the gastrointestinal tract or.

Prevention and prognosis

The development of congenital collagenoses cannot be avoided, however, to prevent the development of secondary degenerative processes, it is necessary to follow the following preventive recommendations:

• avoiding hypothermia and overheating of the body;
• complete elimination of foci of any chronic infections;
• strengthening the immune system on an ongoing basis;
• adequate use of medications with strict adherence to the recommendations of the attending physician;
• minimizing impact;
• avoiding contact with allergens;
• Regular visits to all clinicians for preventive examinations.

The prognosis of collagenosis is completely dictated by the variant of its course and the adequacy of the implementation of therapeutic techniques. Early and continuous treatment allows to achieve stable remission and improve the patient’s quality of life. A complete lack of therapy is fraught with the development of the above complications, which often result in death. The most dangerous are the so-called major collagenoses.

– a group of diseases united by similar functional and morphological changes in connective tissue (mainly collagen-containing fibers). A characteristic manifestation of collagenosis is a progressive course, involving various internal organs, blood vessels, skin, and musculoskeletal systems in the pathological process. Diagnosis of collagenosis is based on multiple organ lesions, identification of positive laboratory markers, and biopsy data of connective tissue (skin or synovial membranes of joints). Most often, corticosteroids, immunosuppressants, NSAIDs, aminoquinoline derivatives, etc. are used to treat collagenosis.

Collagenoses (collagen diseases) are immunopathological processes characterized by systemic disorganization of connective tissue, multisystem damage, a progressive course and polymorphic clinical manifestations. In rheumatology, collagenoses include rheumatoid arthritis, rheumatism, systemic lupus erythematosus, systemic scleroderma, periarteritis nodosa, dermatomyositis, Wegener's granulomatosis, etc. These diseases are combined into a single group based on a common pathomorphological feature (fibrinoid changes in collagen) and a pathogenetic mechanism (violations immune homeostasis).

Connective tissue forms the skeleton, skin, stroma of internal organs, blood vessels, fills the spaces between organs and makes up more than half the mass of the human body. The main functions of connective tissue in the body include protective, trophic, supporting, plastic, structural. Connective tissue is represented by cellular elements (fibroblasts, macrophages, lymphocytes) and the intercellular matrix (ground substance, collagen, elastic, reticular fibers). Thus, the term “collagenosis” does not fully reflect the pathological changes occurring in the body, therefore, at present, this group is usually called “diffuse connective tissue diseases.”

Classification of collagenoses

There are congenital (hereditary) and acquired collagenoses. Congenital connective tissue dysplasias are represented, in particular, by mucopolysaccharidoses, Marfan syndrome, osteogenesis imperfecta, Ehlers-Danlos syndrome, elastic pseudoxanthoma, Stickler syndrome, etc.

Acquired collagenoses, in turn, include SLE, scleroderma, periarteritis nodosa, dermatomyositis, rheumatoid polyarthritis, Sjögren's syndrome, rheumatism, systemic vasculitis, diffuse eosinophilic fasciitis, etc. Of these, the first four nosological units are considered major collagenoses, distinguished by the truly systemic nature of the lesion and the severity of the prognosis; the rest – to minor collagen diseases. It is also customary to distinguish between transitional and mixed forms of diffuse connective tissue diseases (Sharpe's syndrome).

Causes of collagenosis

Congenital collagenoses are caused by a hereditary (genetic) disorder of collagen structure or metabolism. The etiology of acquired systemic connective tissue diseases is less studied and understood. It is considered from the point of view of multifactorial immunopathology, caused by the interaction of genetic, infectious, endocrine factors and environmental influences. Numerous studies have confirmed the association between specific systemic connective tissue diseases and the carriage of certain HLA antigens, mainly histocompatibility class II antigens (HLA-D). Thus, systemic lupus erythematosus is associated with carriage of the DR3 antigen, scleroderma - A1, B8, DR3 and DR5 antigens, Sjögren's syndrome - with HLA-B8 and DR3. In families of patients with collagenosis, more often than in the population as a whole, systemic diseases are registered among first-degree relatives.

Scientific research is still underway regarding infectious agents involved in the development of collagenosis. The infectious-allergic genesis of diffuse connective tissue diseases cannot be ruled out; The possible role of intrauterine infections, staphylococci, streptococci, parainfluenza viruses, measles, rubella, mumps, herpes simplex, cytomegalovirus, Epstein-Barr, Coxsackie A, etc. is considered.

It should be noted the connection of collagenoses with changes in endocrine-hormonal regulation: with the onset of the menstrual cycle, abortion, pregnancy or childbirth, menopause. External environmental factors, as a rule, provoke an exacerbation of a latent pathology or act as triggers for the occurrence of collagenosis in the presence of a corresponding genetic predisposition. Such triggers can be stress, trauma, hypothermia, insolation, vaccination, medication, etc.

Pathogenesis and pathomorphology of collagenoses

The pathogenesis of collagenosis can be presented in the form of the following general diagram. Against the background of bacterial-viral sensitization of the body, pathogenic immune complexes are formed, which settle on the basement membrane of blood vessels, synovial and serous membranes and provoke the development of nonspecific allergic inflammation. These processes cause autoallergy and autosensitization to one’s own tissues, disruption of cellular and humoral factors of immunogenesis, hyperproduction of autoantibodies to cell nuclei, collagen, vascular endothelium, and muscles.

Perverted immune, vascular and inflammatory reactions in collagenoses are accompanied by pathological disorganization of connective tissue. Pathomorphological changes go through 4 stages: mucoid swelling, fibrinoid necrosis, cellular proliferation and sclerosis.

Collagenoses are accompanied by various pathological changes, but all diseases are united by diffuse involvement of the body’s connective tissue in the pathological process, which can occur in various combinations. Thus, with periarteritis nodosa, vessels of the muscular type are predominantly affected, which leads to scarring and desolation of the latter, therefore, in the clinical course, vascular aneurysms, hemorrhages, bleeding, and heart attacks are often observed. For scleroderma, the development of widespread sclerosis (lesions of the skin and subcutaneous tissue, pneumosclerosis, cardiosclerosis, nephrosclerosis) is typical. With dermatomyositis, lesions of the skin and muscles, as well as the arterioles located in them, predominate. Systemic lupus erythematosus is characterized by a polysyndromic course with the development of dermatosis, polyarthritis, Raynaud's syndrome, pleurisy, nephritis, endocarditis, meningoencephalitis, pneumonitis, neuritis, plexitis, etc.

Symptoms of collagenosis

Despite the variety of clinical and morphological forms of collagenosis, common features can be traced in their development. All diseases have a long, wave-like course with alternating exacerbations and remissions, and the steady progression of pathological changes. Characterized by persistent fever of the wrong type with chills and profuse sweats, signs of allergies, and unexplained increasing weakness. Common to all collagenoses are systemic vasculitis, muscular-articular syndrome, including myalgia, arthralgia, polyarthritis, myositis, synovitis. Often there is damage to the skin and mucous membranes - erythematous rash, petechiae, subcutaneous nodules, aphthous stomatitis, etc.

Heart damage due to collagenosis can be accompanied by the development of myocarditis, pericarditis, myocardial dystrophy, cardiosclerosis, arterial hypertension, ischemia, and angina pectoris. On the part of the respiratory system, pneumonitis, pleurisy, pulmonary infarction, and pneumosclerosis are noted. Renal syndrome includes hematuria, proteinuria, renal amyloidosis, chronic renal failure. Disorders of the gastrointestinal tract can be represented by dyspepsia, gastrointestinal bleeding, attacks of abdominal pain simulating cholecystitis, appendicitis, etc.

The polymorphism of the clinical picture is explained by the organ specificity of the lesion in various forms of collagenosis. Exacerbations of diffuse connective tissue diseases are usually associated with infections, hypothermia, hyperinsolation, and injuries.

Diagnosis of collagenosis

The basis for the assumption of one form or another of collagenosis is the presence of classical clinical and laboratory signs. Typically, the appearance in the blood of nonspecific markers of inflammation: C-reactive protein, increased α2-globulins, fibrinogen, seromucoid, ESR, etc. Of great diagnostic importance is the determination of immunological markers characteristic of each disease: CEC, antinuclear and rheumatoid factors, antibodies to one and double-stranded DNA, antistreptolysin-0, antibodies to nuclear antigens, complement levels, etc. Often, to make a pathomorphological diagnosis, it is necessary to resort to a biopsy of the skin, muscles, synovial membrane of joints, and kidneys.

Some help in diagnosing collagenosis can be provided by x-ray examination of bones and joints, which reveals general (osteoporosis, narrowing of joint spaces), as well as specific x-ray signs (usuration of articular surfaces in rheumatoid arthritis, aseptic necrosis of articular surfaces in SLE, osteolysis of the distal phalanges in scleroderma and so on.). To identify the nature and extent of damage to internal organs, ultrasound diagnostic methods (EchoCG, ultrasound of the pleural cavity, ultrasound of the kidneys, ultrasound of the abdominal organs), MRI, and CT are used. Differential diagnosis of various forms of collagenosis is carried out by a rheumatologist; if necessary, the patient is consulted by other specialists: cardiologist, pulmonologist, immunologist, dermatologist, etc.

Treatment and prognosis of collagenosis

The course of most collagen diseases is progressive and recurrent, which requires gradual, long-term, often lifelong treatment. Most often, the following groups of drugs are used for the treatment of various forms of collagenosis: steroid (glucocorticoids) and non-steroidal anti-inflammatory drugs, cytostatics, aminoquinoline derivatives, gold preparations. The dosage and duration of courses are determined strictly individually, taking into account the type of disease, severity and severity, age and individual characteristics of the patient.

During periods of exacerbations, the use of extracorporeal methods of hemocorrection (plasmapheresis, cascade filtration of plasma, hemosorption) is effective. During remission of collagenosis, physical rehabilitation may be recommended: exercise therapy, medicinal electrophoresis, ultrasound, ultraphonophoresis, magnetic therapy, UHF therapy, radon, carbon dioxide, hydrogen sulfide therapeutic baths, sanatorium treatment.

All types of collagenosis are characterized by a chronic progressive course with multisystem damage. Prescribing corticosteroid or immunosuppressive therapy helps reduce the severity of clinical symptoms and lead to more or less long-term remission. The so-called major collagenoses have the most rapid development and severe course. The death of patients can occur as a result of renal, cardiovascular, respiratory failure, or the addition of an intercurrent infection. To prevent exacerbations of collagenosis, it is important to eliminate foci of chronic infection, undergo a medical examination, and avoid excessive insolation, hypothermia and other provoking factors.

Collagenosis is a group of diseases characterized by damage to connective tissue, including fibers containing collagen. Collagenosis refers to a group of pathologies involving protein-rich tissues that maintain the proper functioning of organs and body parts. The result of collagenosis is disruption of the functioning of muscles, joints and other organs, as well as deterioration of the condition of the skin.

Symptoms of collagenosis can be:

• weakness;
• painful sensations in muscles and joints;
• low-grade fever – an increase in body temperature over a long period of time within the range of 37 – 37.5°C;
• skin rashes;
• excessive sweating.

There are more than 200 connective tissue pathologies, the symptoms and causes of which are closely interrelated. Let's try to understand some of the most common types of collagenosis.

2. Hereditary connective tissue diseases

Some connective tissue diseases result from changes in certain genes. Here are the most common ones:

Ehlers-Danlos syndrome

This syndrome is a group of hereditary diseases characterized by hyper-soft joints, increased skin elasticity, damaged blood vessels, abnormal growth of scar tissue and bruising. The symptoms of this syndrome can vary from mild to severe. Depending on the specific type of Ehlers-Danlos, signs of this disease may include the following:

• bleeding gums,
• weak blood vessels
• slow wound healing,
• curved spine,
• flat feet,
• problems with the lungs, heart valves and digestive organs.

Congenital epidermolysis bullosa

People with this disease have such fragile skin that almost any contact with it is accompanied by the appearance of thin-walled blisters with serous contents. Such bubbles can arise from an impact, a person falling to the ground, or even from clothing rubbing on certain areas of the skin.

Depending on the specific type of epidermolysis bullosa congenita, the airway, digestive tract, bladder, muscles, or other organs may be adversely affected. As a rule, epidermolysis bullosa appears immediately at the birth of a child, since the act of childbirth itself is to some extent the first mechanical injury.

Marfan syndrome

The clinical picture of this disease is characterized by damage to most vital organs and systems: the cardiovascular and central nervous systems, the musculoskeletal system, the organs of vision and breathing. People with Marfan syndrome have tall stature, as well as excessively long bones and thin “spider-like” fingers and toes.

Other problems that accompany Marfan syndrome include vision problems due to abnormal placement of the lens of the eye and enlargement of the aorta. Marfan syndrome is caused by mutations in a gene that regulates the structure of the fibrillin protein.

Osteogenesis imperfecta

This pathology is a congenital disorder characterized by brittle bones and low muscle mass. There are several types of this disease. Specific symptoms of osteogenesis imperfecta depend on the specific type of disease and may include the following signs:

• gray or blue tint of the sclera - the white of the eyes;
• thin skin;
• hearing loss;
• slight curvature of the spine;
• breathing problems.

The cause of this pathology is a mutation in the COL1A1 and COL1A2 genes, responsible for type 1 collagen, as well as a change in protein quality.

3. Autoimmune diseases

Autoimmune diseases are a set of diseases in which, under the influence of one’s own immune system, deformation and destruction of tissues and organs of the human body occurs. Researchers believe that this disorder occurs in genetically susceptible people. Their protective immune system produces antibodies that attack their own tissues. The following diseases can be classified as this type of pathology.

Dermatomyositis and polymyositis

These diseases are based on inflammatory processes that occur against the background of the specific action of the human immune system. Dermatomyositis is characterized by inflammation of the skin, and polymyositis is characterized by inflammation of the muscles. The symptoms of both diseases are characterized by:

• fatigue,
• muscle weakness,
• dyspnea,
• difficulty swallowing,
• weight loss,
• fever.

Rheumatoid arthritis

In this disease, the immune system attacks the synovium, the membrane lining the joint cavity. As a result of such exposure, it becomes inflamed, pain and swelling appear, and a feeling of stiffness throughout the body. Other symptoms of rheumatoid arthritis include:

• loss of appetite;
• fatigue;
• fever;
• anemia.

Scleroderma

This term refers to a group of diseases from the group of collagenoses, which are characterized by thickening of the skin, build-up of scar tissue and damage to internal organs. These disorders fall into two main categories: systemic and focal scleroderma.

Sjögren's syndrome

Sjogren's syndrome is a chronic systemic disease in which the lacrimal and salivary glands, as well as the glands of the mucous membranes, are attacked by the immune system. The result of this pathological process is dysfunction of these glands with a subsequent decrease in the amount of secretion produced. The main symptoms of Sjögren's syndrome are dry eyes and mouth, as well as constant fatigue and joint pain.

Systemic lupus erythematosus

Systemic lupus erythematosus, affecting capillaries and connective tissue, has a negative effect on the entire body as a whole. The symptoms of lupus erythematosus are characterized by the following signs:

• sensitivity to sunlight;
• rashes on the cheeks and bridge of the nose;
• hair loss;
• kidney dysfunction;
• problems with concentration and memory;
• anemia.

Vasculitis

This term is characteristic of more than 20 different conditions characterized by inflammation of the walls of blood vessels. As a result, vasculitis can impair blood circulation to organs and other tissues of the body.

Mixed connective tissue disease – mixed collagenosis.

With such collagenosis, people exhibit characteristics of several diseases simultaneously: lupus erythematosus, dermatomyositis, rheumatoid arthritis, etc. The multifaceted manifestations of this pathology in patients manifest themselves in different ways: some complain of mild symptoms, while others may experience serious complications, including infections, strokes, kidney failure and other dangerous phenomena.

Treatment of collagenosis depends on many different factors: the type of disease and its symptoms, the severity of the disease, as well as the individual characteristics of the patient’s body. Most often, measures to treat collagenosis help, if not cure the disease completely, then at least control the unpleasant symptoms of collagenosis.

A connective tissue disease is collagenosis. This complex pathological process involves not only human skin, but also deep internal membranes - the musculoskeletal system, some internal organs located in close proximity to connective tissue lesions. Also, in patients diagnosed with collagenosis, lesions of blood vessels and nerve endings are often found.

To make a correct diagnosis and prescribe the necessary treatment to the patient, it is necessary to submit a biopsy (a small piece of connective tissue) to the laboratory. This disease is treatable, but the therapy is extremely complex - hormonal.

About collagenosis

Collagen disease refers rather to damage to the immune system. it is characterized by complete damage to the connective tissue and, as a result, multiple painful clinical manifestations.

Collagenosis includes diseases such as rheumatoid arthritis, lupus erythematosus, scleroderma, as well as more complex pathological forms. As you know, human skin, as well as the skeleton, the lining of internal organs and all blood vessels consists entirely of connective tissue.

The functions of connective tissue are presented in the form of: protective, supporting, as well as plastic and structural. Any damage to connective tissue leads to damage to internal organs and human vital systems.

Forms of skin lesions

According to its form and origin, collagenosis is classified into congenital, that is, one that is transmitted to a person from mother and father, and also acquired. If we are talking about congenital collagenosis, then in this case we mean Marfan syndrome, Ehlers-Danlos syndrome, Stickler syndrome.

D for reference And!

Marfan syndrome is a hereditary disease. It affects the human skeleton and visual organs. A person has the following pathologies: dislocation and subluxation of joints, very long and thin fingers, severe heart defects, damage to blood vessels.

Ehlers-Danlos syndrome is a hereditary disease characterized by increased unnatural joint mobility, increased skin trauma, severe bleeding, and deformation of the musculoskeletal system.

Stickler syndrome is a pathology, as a result of which a person quickly loses his ability to work, his vision is impaired, a cleft palate appears, the lower jaw is deformed, and there is increased trauma.

Acquired collagenosis

Collagenosis can be an acquired disease. In this case, the patient has such signs and connective tissue lesions as systemic vasculitis, rheumatoid arthritis and polyarthritis, as well as dermatomyositis.

In particular, Sjögren's syndrome or also called dry syndrome. This name is due to the fact that a person’s mucous membranes of the eyes, oral cavity and partially of internal organs dry out completely. The danger of Sjogren's syndrome is that it is characterized by oncological complications. Treatment of pathologies such as Sjogren's syndrome should be carried out throughout life.

Causes of pathology

There are a huge number of reasons for the occurrence of collagenosis. The first is genetics or heredity. If the mother or father of the child had connective tissue pathologies, then with a probability of about 80% they are passed on from generation to generation.

Among the provoking factors of collagenosis disease are: disorders of the thyroid gland, infectious lesions, the negative effects of radiation and external exposure.

Doctors do not rule out a negative impact on the role of various intrauterine infections, the herpes virus, measles and rubella, as well as staphylococci and streptococci, which a woman may have had during pregnancy.

In women, changes in connective tissue are affected by a number of hormonal disorders in the form of abortions, multiple pregnancies, and difficult childbirth. Doctors warn that any disease in our body can worsen under the influence of stress, psychological trauma, constant tension, improper vaccination, long-term treatment with hormones or antibiotics.

Clinical picture

With collagenosis in humans, small blood vessels are initially affected, then the pathology spreads to muscle tissue. The process of collagenosis leads to extensive scarring of blood vessels, resulting in bleeding and periodic hemorrhage into the internal organs. Following such damage to the blood vessels, the patient will suffer a myocardial infarction in 80%.

Collagenosis leads to damage to the skeleton, muscles, skin; increases the risk of developing dermatological diseases, pleurisy and endocarditis, as well as severe neuritis.

Symptoms

The following symptoms of illness are typical for patients with collagenosis:

• Chills;
• Fever;
• Dermatological skin lesions;
• Weakness throughout the body;
• Sleep disturbance;
• Damage to bone mass and joints by arthritis;
• Pathological nodules under the skin.

With collagenosis, the patient experiences severe damage to the cardiovascular system in the form of: pericarditis, cardiosclerosis, pathological increase in pressure, as well as heart attacks of varying severity.

Patients have numerous complaints about disorders of the gastrointestinal tract - constipation, abdominal pain, inflammation of appendicitis, cholecystitis.

To diagnose collagenosis, it is necessary to undergo a full range of all clinical blood tests, as well as resort to a biopsy of muscles, connective tissue, kidneys and joints. The procedure for diagnosing collagenosis will take several weeks.

Treatment of the disease

Basically, the treatment of collagenosis is lifelong therapy. The course of treatment and dosage of medications is determined by the doctor. Therapy is carried out with cytostatics, hormonal agents in the form of steroids and glucocorticoids, and antibiotics that relieve the inflammatory process. During an exacerbation, patients are prescribed procedures in the form of plasmapheresis, ultrasound, as well as therapeutic baths with hydrogen sulfide.

Regarding the prognosis for recovery, they are minimal. With a large area of ​​damage to the patient’s connective tissue, there is a risk of death. To avoid it, patients need to undergo examination of the whole body every six months, not expose their body to stress, hypothermia and not trigger existing inflammatory processes.