Collagenoses - causes, symptoms, diagnosis, prevention, treatment in Moscow

Collagenoses - what is it?

Collagenosis is an immunopathological process characterized by disorganization of connective tissue.
In rheumatology, the group of collagenoses includes:

• rheumatism;
• rheumatoid arthritis;
• systemic scleroderma;
• systemic lupus erythematosus;
• dermatomyositis;
• periarteritis nodosa;
• Wegener's granulomatosis, etc.

All these diseases have one pathomorphological sign - fibrinoid changes in collagen. Also, during their development, a disturbance of immune homeostasis is observed.

University

Team

Currently, the head and ideological inspirer of research work in the scientific group is the head of the 2nd Department of Internal Medicine, Doctor of Medical Sciences, Professor Nikolai Fedorovich Soroka.
Nikolai Fedorovich was born in the village of Osinniki, Kamenets district, Brest region. In 1972 he graduated with honors from the Minsk State Medical Institute. In 1979 he defended his PhD thesis, which was devoted to the problem of the pathogenesis of diffuse connective tissue diseases. In 1992, in Moscow, he defended his doctoral dissertation on the topic “Clinical and biochemical aspects of rheumatoid arthritis and ways to optimize treatment.” In 1993, he was awarded the academic degree of Doctor of Medical Sciences. From 1994 to the present – ​​Head of the 2nd Department of Internal Diseases of the Belarusian State Medical University. In 1995 he was approved for the academic rank of professor.

Soroka Nikolai Fedorovich is the author of more than 450 scientific works, including 7 monographs, 14 manuals, 5 reference books and popular science books. He is the author of 9 patents for inventions. From 2005 to May 2010 – editor-in-chief of the Health Care magazine. Member of 2 councils for the defense of doctoral dissertations in the specialty of rheumatology and cardiology, chairman of the commission for the publication of educational medical literature under the Ministry of Education, member of the editorial board of the magazines “Medical News”, “Recipe”, chairman of the board of the Republican Scientific Society of Cardiologists. From 1994 to 2009, he served as the chief rheumatologist of the Ministry of Health, and was a member of the Presidium of the Higher Attestation Commission of Belarus (1995-2001). In 2000 awarded the highest qualification category of general practitioner. Soroka Nikolay Fedorovich is an honorary member of the Polish Academy of Medicine, the Association of Rheumatologists of Russia and Ukraine, the International Academy of Science and Higher Education, awarded the Albert Schweitzer Gold Medal, Excellence in Health Care (2001) and Excellence in Printing (2009) of the Republic of Belarus. The Academy of Medical Sciences of Ukraine awarded the gold medal of Academician N.D. Strazhesko (2009).

In the past, a student of Professor Grigory Pavlovich Matveykov, Nikolai Fedorovich Soroka leads research projects aimed at studying the underlying mechanisms of the occurrence and progression of systemic connective tissue diseases and the development of new methods of their therapy.

Staff of the scientific group:

• Talako Tatyana Mikhailovna, researcher
• Ryabtseva Tatyana Vladimirovna, junior researcher
• Butorina Irina Ivanovna, junior researcher
• Aleshina Lyudmila Ilnichna, laboratory assistant, 1st category
• Buloichik Valentina Vasilievna, laboratory assistant, 1st category
• Gaponova Valentina Evgenievna, preparator

Classification of collagenoses

Connective tissue diseases - collagenosis - are:

• Purchased . Represented by scleroderma, dermatomyositis, rheumatoid polyarthritis, periarteritis nodosa, rheumatism, Sjogren's syndrome, diffuse eosinophilic fasciitis, systemic vasculitis.
• Congenital (hereditary). This group includes mucopolysaccharidoses, elastic pseudoxanthoma, Stickler, Ehlers-Danlos and Marfan syndromes, osteogenesis imperfecta.

Causes of collagenosis

Hereditary collagenoses of blood vessels and connective tissues are the result of congenital disorders of collagen structure or metabolic processes. The etiology of acquired forms of pathology is less clear to scientists. They consider it from the perspective of multifactorial immunopathology, which is caused by the simultaneous impact on the human body of infectious, endocrine and genetic factors, and environmental influences.

According to scientific data, there is a close connection between certain connective tissue diseases and the carriage of certain HLA antigens. Thus, scleroderma is associated with carriage of A1, B8, DR3 and DR5 antigens, lupus erythematosus - with DR3 antigen, Sjögren's syndrome - with HLA-B8 and DR3.

As for infectious agents that provoke the formation of collagenoses, there are still heated debates about them to this day.

Doctors do not rule out that the development of connective tissue pathology can be caused by:

• infectious and allergic diseases;
• intrauterine infections;
• parainfluenza viruses;
• measles, mumps, rubella, cytomegalovirus, herpes simplex;
• streptococci, staphylococci;
• Coxsackie and Epstein-Barr viruses.

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 :

• 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.

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Symptoms of collagenosis

In the development of all collagenoses, despite the diversity of their clinical and morphological forms, common features can be traced. Firstly, all diseases from this group have an undulating and long course. Stages of exacerbation are always followed by a period of remission. At the same time, pathological changes are steadily progressing.

Patients experience:

• fever (chills, profuse sweats);
• allergy symptoms (which is why photos of collagenoses show patients with dermatological rashes);
• muscular-articular syndrome (myositis, polyarthritis, myalgia, synovitis);
• weakness;
• damage to the mucous membranes (petechiae, erythematous rash, aphthous stomatitis, subcutaneous nodules);
• signs of heart disease (pericarditis, myocarditis, cardiosclerosis, ischemia, arterial hypertension, angina pectoris).

Collagenosis of the lungs leads to pleurisy, pneumonia, and pneumosclerosis. Renal syndrome in the described disease is characterized by renal amyloidosis, proteinuria, hematuria, and chronic renal failure.

Collagenosis can also provoke disturbances in the functioning of the gastrointestinal tract, resulting in:

• internal bleeding;
• dyspepsia;
• attacks of abdominal pain;
• appendicitis;
• cholecystitis, etc.

Deterioration of the patient's condition and exacerbation of symptoms can be caused by infectious diseases, general hypothermia, injuries, and hyperinsolation.

If you notice similar symptoms, consult a doctor immediately. It is easier to prevent a disease than to deal with the consequences.

Collagenoses

Etiology of collagenoses

To this day, the study of the reasons leading to the development and occurrence of collagenosis . These diseases are classified as multifactorial diseases. This may be genetic inheritance, infection, including streptococcal, exposure to drugs, administration of vaccines and serums, viremia (mainly Coxsackie A virus or adenoviruses), increased insolation, X-ray irradiation, hypothermia, physical and mental fatigue, allergic processes in the body. Endocrine factors are also of undoubted importance, given that most collagenoses have a gender predisposition: systemic lupus and scleroderma are more common in women than in men; at the same time, periarteritis nodosa occurs more often in men. It should also be noted that age-related features of the development of collagenosis are often associated with the characteristics of endocrine-hormonal regulation. Thus, the onset of systemic lupus is often associated with the onset of the menstrual cycle, pregnancy or childbirth; scleroderma often develops during menopause.

Collagenosis can develop as a secondary disease against the background of another pathology (for example, dermatomyositis against the background of a malignant formation).

Pathogenesis of collagenoses

It is known that, for example , viruses can directly affect the genetic system, causing various kinds of mutations. Inflammatory and immune disorders in collagenosis cause the development of an autoimmune response, in which one’s own immune cells lead to the destruction of healthy tissue. In collagen diseases, characteristic changes are observed in the hyaluronic acid system, which is part of the main substance of connective tissue, leading to collagen necrosis. As a result of immune, vascular and inflammatory disorders in collagenosis , pathological disorganization of connective tissue is observed.

Classification of collagenoses

Collagen diseases include rheumatism, rheumatoid polyarthritis, systemic lupus erythematosus, systemic scleroderma and periarteritis nodosa, and systemic connective tissue dysplasias include dermatomyositis.

Disorders caused by collagenosis

Common to all collagen diseases is the involvement in the pathological process of the entire system of connective tissue and blood vessels of the body. Thus, systemic lupus is characterized by the detection of hematoxylin bodies. In the kidneys, changes in the walls of the glomerular capillaries are observed. Periarterial fibrosis develops in the vessels of the spleen (symptom of onion peel).

Periarteritis nodosa affects muscular-type vessels. As a result, cicatricial changes, aneurysms, hemorrhages, and heart attacks can develop.

Scleroderma is characterized by widespread and progressive damage to connective tissue and blood vessels, resulting in early sclerosis of the skin and subcutaneous tissue. The following lesions of internal organs are observed: cystic pneumosclerosis (“honeycomb lung”), progressive cardiosclerosis and specific kidney damage.

Dermatomyositis is most characterized by lesions of the skin, muscles, including the myocardium, as well as arterioles, mainly in the skin and muscles.

A significantly common disease is systemic lupus erythematosus. Significant changes are observed mainly in the internal organs.

Manifestations of collagenosis

The most characteristic clinical manifestations of collagenosis are the following: - long-term progressive course, in the absence of timely treatment, leading to an unfavorable outcome; - persistent fever or low-grade fever associated with the absorption of connective tissue destruction products; - signs of an allergic process, manifested mainly by the development or exacerbation of the disease after certain effects on the body of various factors (increased insolation); - pronounced weight loss and trophic skin manifestations; - polymorphism of the clinical picture (damage to various internal organs, blood vessels, nervous system, skin, muscles, bones);

- periodic appearance of protein in the urine;

— asthenia (fatigue, decreased performance);

- damage to the mucous membranes - aphthous stomatitis, thrush and hemorrhages;

— progressive articular and bone syndromes with persistent joint deformation;

- damage to the central and peripheral nervous system, etc.

Principles of treatment of collagenosis

The basic principles of treatment of collagenosis often come down to the use of corticosteroids. Sometimes antimalarial drugs, immunosuppressants, and drugs that help reduce the side effects of corticosteroids are used. But all these medicines are not able to quickly relieve a person from the disease. Treatment is very long and strictly individual. Initially, large doses of corticosteroids are prescribed and gradually reduced, taking small doses of hormones for months and sometimes years. In spring and autumn, a course of multivitamins is recommended. As soon as stable remission of the disease is achieved, corticosteroids are discontinued, but within 2-3 years it is necessary to undergo a medical examination. It is necessary to eliminate foci of chronic infection in the body: treat bad teeth, chronic tonsillitis, sore throat, pneumonia, inflammation of the genital area.

The diet should be complete, with a sufficient amount of vitamins, mainly group B and C, eat complete protein foods - meat, fish, eggs, cheese, cottage cheese, as well as other dairy products. Limit excess insolation using photoprotection means and hypothermia. Moderate physical activity and hardening are required.

Restorative treatment for collagenosis

Indications for physical rehabilitation and spa treatment for systemic connective tissue diseases: • predominantly peripheral manifestations of the disease; • chronic or subacute course with the activity of the pathological process not exceeding stage I; • functional insufficiency of the musculoskeletal system is not higher than II degree. Contraindications to physio-functional and sanatorium-resort treatment for systemic connective tissue diseases: • general contraindications that exclude the referral of patients to resorts and local sanatoriums (acute inflammatory processes, benign and malignant neoplasms, diseases of the blood and hematopoietic organs, bleeding and a tendency to it, any tuberculosis localization, circulatory failure of functional class II and III-IV, high arterial hypertension, severe forms of thyrotoxicosis, myxedema, diabetes, kidney disease and dysfunction, all forms of jaundice, liver cirrhosis, mental illness); • predominantly visceral forms of systemic connective tissue diseases; • pronounced functional disorders of the musculoskeletal system with loss of the ability for self-care and independent movement; • treatment with large doses of corticosteroids (more than 15 mg of prednisolone per day) or taking cytostatics.

Physiotherapy for collagenosis comes down to immunosuppressive therapy (aerocryotherapy, medicinal electrophoresis, nitrogen baths); anti-inflammatory (DMV therapy on the adrenal gland area, ultraphonophoresis of hydrocortisone) and fibromodulating methods (electro- and ultraphonophoresis of enzyme preparations); vasodilator methods (high-frequency magnetic therapy).

Diagnosis of collagenosis

During the examination of the patient, the doctor pays attention to the classic clinical and laboratory signs of collagenosis - determination of nonspecific markers of inflammation in a blood test (C-reactive protein, high fibrinogen, α2-globulin, ESR, seromucoid, etc.).

The identification of immunological markers is of great diagnostic importance:

• antibodies to nuclear antigens;
• rheumatoid and antinuclear factors;
• complement level;
• antistreptolysin-O;
• antibodies to double- and single-stranded DNA.

Quite often, to make a correct diagnosis, the doctor resorts to a biopsy of the kidney, synovium of the joints, skin, and muscle tissue.

Among the functional methods during the examination, the following can be used:

• X-ray of joints and bones (narrowing of the lumens of the joint spaces, osteoporosis is detected). In systemic lupus erythematosus, aseptic necrosis of the articular surfaces is diagnosed; in rheumatoid arthritis, their usuration is diagnosed.
• Ultrasound of the kidneys, abdominal organs, pleural cavity.
• Magnetic resonance imaging (MRI).
• CT scan ().
• Echocardiography (EchoCG).

A rheumatologist is involved in the differential diagnosis of collagenosis. In some situations, the patient is referred for consultation to a pulmonologist, cardiologist, dermatologist, or immunologist.

Diagnosis and treatment of nervous system lesions in rheumatic diseases

Rheumatic diseases belong to a group of diseases that are characterized by the development of autoimmune processes against antigens of almost all organs and tissues of the body, which is combined with the formation of autoantibodies with organ-nonspecific properties.

Autoimmune processes carry out information exchange between the neuroendocrine and immune systems, with autoantibodies to hormones, mediators and their receptors playing a major role. The synthesis of neuropeptides in immunocompetent cells has been demonstrated, and the possibility of synthesis of lymphokines and monokines has been proven in the cells of the neuroendocrine system.

Data have been obtained on the neurogenic regulation of immune functions and their disorders, at the same time, immunocompetent cells and their mediators can influence the function of the central nervous system (CNS) according to the principle of neuroimmunomodulation. It has been shown that the entire central and peripheral nervous system has the property of neurosecretion. The influence of the immune and nervous systems on each other is realized through the receptor structures of cells, the interaction of which creates “receptor-receptor” connections and thus organizes the molecular mechanism for the joint work of both systems.

Cell functioning and signaling information are provided by mediators and neurotransmitters in both systems; information is exchanged between the nervous and immune systems using cytokines, steroids and neuropeptides [1, 2].

Thus, the commonality and interrelation of the nervous and immune systems, the similarity between their structures and functions, and the development of a new direction in modern immunology—neuroimmunology—have been proven [3, 4]. A wide range of neurological syndromes in autoimmune systemic diseases allows us to consider them as model systems for studying the pathogenetic role of immune mechanisms of damage to the central and peripheral nervous system [5].

Potential targets for autoimmune aggression may be various antigens of nervous tissue, including myelin, including that associated with glycoprotein, and its main protein, gangliosides, nuclear protein of neuronal cells, and others [6]. Thus, target antigens in neurolupus are represented by neuronal tissue antigens, ribosomal P protein, rDNA, small nuclear ribonucleoprotein, as well as anionic phospholipids in antiphospholipid syndrome, which causes a wide range of neurological symptoms in this pathology [7, 8].

According to various authors, the frequency of damage to the nervous system in rheumatic diseases (RD) ranges from 40% to 70% and higher, if mental syndromes and headaches are taken into account. Neurological syndromes are included in the classification criteria for systemic vasculitis published by the American College of Rheumatology in 1990, in the diagnostic and activity criteria for systemic lupus erythematosus (SLE), as well as in a number of other diagnostic criteria, in particular polyarteritis nodosa in children. Neurological disorders in RD require differential diagnosis and the appointment of adequate treatment jointly by a rheumatologist and a neurologist.

In SLE, the diagnostic criteria for neurological lesions include seizures or psychosis. Damage to the central nervous system is caused mainly by vascular pathology, which includes vasculopathy, thrombosis, true vasculitis, heart attacks and hemorrhages [7]. Antineuronal antibodies are detected in the cerebrospinal fluid, an increase in protein levels and an increase in cellular composition are determined. Different types of convulsive seizures have been described: large, small, type of temporal lobe epilepsy, as well as hyperkinesis. In CNS lupus, there is a migraine-type headache that is resistant to analgesics but responds to treatment with glucocorticosteroids. Cranial nerve palsies are usually accompanied by ophthalmoplegia, cerebellar and pyramidal symptoms, and nystagmus. There are visual disturbances and transient cerebrovascular accidents. Acute transverse myelitis is rare and has a poor prognosis. Psychiatric syndromes are diverse and are characterized by affective, organic brain or schizophrenia-like manifestations [9, 10].

Antiphospholipid syndrome has also been described as part of SLE. This syndrome includes: recurrent arterial or venous thrombosis, recurrent miscarriage and thrombocytopenia and additional signs: livedo reticularis, neurological manifestations: chorea, epilepsy, migraine-like headache, cerebrovascular accidents and dementia due to multiple infarctions, chronic leg ulcers, Coombs-positive hemolytic anemia, valvular heart lesions and serological markers - antiphospholipid antibodies, which include anticardiolipin antibodies IgG and IgM and lupus anticoagulant [11].

In systemic scleroderma (SSc), the neurological syndrome is represented mainly by polyneuritic manifestations associated with vascular changes and fibrotic processes in the connective tissue. Polyarteritis nodosa is characterized by multiple mononeuritis, Wegener's granulomatosis is characterized by asymmetric polyneuropathy, and nonspecific aortoarteritis is characterized by discirculatory encephalopathy and cerebrovascular accidents.

Our own data included an examination of 229 patients with various forms of RD, among whom 110 patients suffered from systemic connective tissue diseases: 88 patients with SLE, 22 with SSc and 119 patients with systemic vasculitis: thromboangiitis obliterans (OT) - 21, polyarteritis nodosa (PU) - 27, nonspecific aortoarteritis - (NAA) - 32, hemorrhagic vasculitis (HV) - 15 and Wegener's granulomatosis (GrV) - 2, other forms - 22. A detailed neurological examination, ultrasound transcranial Dopplerography of cerebral vessels, rheoencephalography, computer (CT) and magnetic resonance imaging were performed. resonance imaging (MRI) of the brain, electroencephalography, study of immune status.

In most patients, the disease began with cutaneous (50.6%), joint-muscular (35.4%) and vascular (27.1%) syndrome. Organ lesions at the onset were recorded with a frequency of 7%, arterial hypertension syndrome - in 5.2%, fever - in 7.0%, hematological disorders - 7.9%. Neurological disorders at the onset of the disease were noted in 12.2% and were manifested by mono- and polyneuropathy and encephalomyelopolyradiculoneuritis syndrome (EMPRN). Damage to the peripheral nervous system at the onset of the disease was especially characteristic of UP and was observed in 30% of patients. The main onset syndromes from the central nervous system were cephalgic (10.5%) and vestibular (6.3%), more often they were observed in NAA. Involvement of the central nervous system occurred in 96 (41.9%) patients, being most pronounced in SLE, NAA, and UP.

Cerebrovascular pathology was dominant in the clinical picture of the disease in 34.7% of patients, and sometimes various symptoms of central nervous system damage developed long before the appearance of the polysyndromic picture of the disease. The main clinical manifestations of cerebrovascular pathology included: cephalgic (82%), asthenic (76%), vestibular-atactic (80%), pyramidal (74%) syndromes, autonomic-vascular failure syndrome (69%), dyssomnic (79%) and basal-meningeal (37%), hypopothalamic dysfunction (34.7%).

The described neurological symptoms were often combined with symptoms of vascular insufficiency of the brain, which were combined by the syndrome of discirculatory encephalopathy of 1 (11%), 2 (26.4%) or 3 (8%) degrees. Transient cerebrovascular accidents occurred in 7.8% of patients.

Hypothalamic dysfunction in patients with RD was manifested by polymorphic neuroendocrine disorders, impaired thermoregulation, mainly of the type of paroxysmal central hyperthermia, insomnia, and pathology of the psychoemotional sphere.

A significant predominance was established in patients with pyramidal insufficiency on the left (41%). The predominance of pyramidal insufficiency on the right was recorded less frequently (23.7%). Dystonic phenomena in the form of vestibular-cerebellar alignment of the hand and dissociated muscle hypotonia in the legs were also more pronounced on the left. The data obtained indicate a predominant lesion of the pyramidal and sensory systems, as well as nonspecific structures of the right hemisphere, which is closely connected with the hypothalamic region and ensures the body’s adaptation to influencing environmental factors. The discovered functional asymmetry indicates a breakdown in the adaptive mechanisms of the nervous system and indicates the role of dysfunction of the right hemisphere-hypothalamic system.

When using MRI and/or CT methods, changes in the ventricular system were identified in the form of its expansion or deformation and/or expansion of the subarachnoid space, as well as focal lesions of various brain structures, atrophy of the brain substance and craniovertebral anomalies. Signs of external, internal or combined hydrocephalus were noted in all nosological forms. Focal changes in the brain substance included hyperdense zones, hypodense zones with or without edema, single or multiple.

When studying the vascular system and cerebral circulation, an increase in vascular tone, a hypertensive and discirculatory type of blood circulation according to rheoencephalography (REG) and an increase in the linear velocity of blood flow in the middle cerebral artery were reliably observed. Patients with central nervous system involvement differed in electroencephalography: they were characterized by diffuse pathological changes, the presence of alpha rhythm disorganization, dysrhythmias and paroxysmal activity.

Correlation analysis of cerebrovascular pathology and the results of instrumental studies of cerebral vessels showed that in all nosological forms, patients had impaired venous hemocirculation. Subsequently, there was a narrowing of the cerebral arteries, liquorodynamic disturbances with the formation of intracranial hypertension, and disruption of the microcirculation system in the brain. Focal brain lesions differed in the localization of the process depending on the nosological form. IN

. The main neurological manifestations of RD are presented.

39% of young SLE patients with central nervous system lesions had cerebrovascular accidents, and in half of them a stroke developed at the onset of the disease. Simultaneously with a stroke, at the onset of SLE, patients more often had a “vascular butterfly” and/or vasospastic syndrome, increased blood pressure, often diastolic. These patients had moderate or high titers of cardiolipin IgG, antibodies to native DNA and rheumatoid factor (RF) IgM, which could indicate the presence of current cerebral vasculitis. These data were confirmed by the detection of hypertonicity of resistive intracranial vessels and pathology of the microvasculature in the form of an increase in the number of functioning capillaries, their pronounced tortuosity with slowing of blood flow in the arterioles. Changes in the coagulation system were characterized by hypercoagulation syndrome. The main risk factors for the development of stroke in patients with RD have been identified: arterial hypertension, heart damage, hypercoagulation, immune inflammation of the vascular wall, asymmetry of cerebral blood flow.

Among patients with RD, cerebrovasculitis (CV) occurred in 28.3% of patients. The diagnosis of CV was made upon detection of focal neurological symptoms, changes in the fundus, decreased vision, the presence of signs of cerebrovascular accident, as well as based on the results of CT and nuclear magnetic resonance imaging (NMRI), which revealed external and internal hydrocephalus, focal changes in cortex and subcortical substance. At the same time, over time, the number of foci of any localization in the brain increased. Magnetic resonance angiography (MRA) examination revealed multiple segmental irregularities of the vascular wall, circular or eccentric stenoses and dilatation of small and medium-sized intracranial arteries with the formation of aneurysms and blood flow disturbances. The detected decrease in the intensity of the MRA signal against the background of increased activity of the rheumatic process indicated the presence of CV.

Immunological markers of CV were considered antibodies to native DNA, IgG antibodies to cardiolipin (aCL) and IgM aCL, antineutrophil cytoplasmic antibodies (ANCA), and, to a lesser extent, RF and lupus anticoagulant (LA). There were clinical and immunological correlations with neurological manifestations.

Isolated (primary) CV was characterized by the detection of symptoms of central nervous system involvement and such signs as headache, convulsions, meningeal syndrome, acute progressive encephalopathy without signs of extracranial or systemic vasculitis, mental syndromes, dementia, progressive decline in intelligence, strokes, visual impairment, nystagmus. More often, periventricular lesions were detected in the first year of the disease.

A number of patients consulted an ophthalmologist due to deterioration of vision, even amaurosis, the presence of uveitis, and ischemic neuritis. Retinal angiopathy occurred in 41% of these patients, phlebopathy - in 14%, retinovasculitis - in 6%, vasospasm - in 13%, angiosclerosis - in 18%.

Polyneuritic syndrome occurred in the vast majority of patients (96.7%) in the form of sensory, sensory-motor polyneuropathy or in combination with lesions of the central nervous system, with EPN and EMPRN syndrome. In SSc, OT and HV, forms in the form of sensitive or sensory-motor polyneuropathy predominated, and in SLE and NAA - forms with combined damage to the peripheral NS (PNS) and CNS - EPN and EMPRN syndromes. In OT and NAA, there was a clear dissociation in the severity of polyneuropathy along the body axis, and in OT, the symptoms were more distinct in the legs, and in NAA, in the arms. Overall, asymmetric polyneuropathy occurred in 19.2% of patients, reaching a maximum in UP (59.3%).

The pathology of the NS in RD often determines the prognosis, the clinical picture of the disease and the quality of life of patients, and also requires the mandatory combined use of basic anti-inflammatory therapy, angio- and neuroprotectors. The group of neuroprotectors includes Actovegin and Instenon. Drugs that improve cerebral circulation are used - Vinpocetine, Cavinton, metabolic agents with antihypoxic effects - Nootropil, Piracetam, Cerebrolysin, and, if indicated, sedatives and anticonvulsants, antidepressants.

For RD, therapy includes glucocorticosteroids, immunosuppressants, immunoglobulin, plasmapheresis, immunomodulators, antiplatelet agents, non-steroidal anti-inflammatory drugs and symptomatic agents.

Treatment consists of several stages: rapid suppression of the immune response at the onset of the disease and during its exacerbations (induction of remission); long-term maintenance therapy with immunosuppressants, in doses sufficient to achieve clinical and laboratory remission of the disease; determining the degree of damage to organs or body systems and their correction, carrying out subsequent rehabilitation measures.

The first stage includes the effective suppression of immune inflammation in the early stages of the disease and involves the use of glucocorticosteroids, cytostatic immunosuppressants such as Cyclophosphamide and antimetabolite action such as Methotrexate, the cytokine suppressive drug Cyclosporin A, intravenous immunoglobulin, the appointment of repeated courses of pulse therapy with methylprednisolone and Cyclophosphamide, in combination with extracorporeal methods treatment.

For acute cerebral disorders with high activity of SLE, a pulse therapy regimen is used with the administration of Metipred 1 g intravenously once a day for 3 days and with the addition of 800 mg of Cyclophosphamide on the 2nd day. In the chronic course of SLE, the daily dose of prednisolone was 15–20 mg, followed by a gradual reduction; Cyclophosphamide is used intramuscularly at a dose of 400 mg per week up to 1600–2000 mg per course, then 200 mg per week for a year or more. The drugs mycophenolate mofetil and leflunomide are being tested.

In case of pathology of the organ of vision, non-steroidal anti-inflammatory drugs are prescribed in the form of diclofenac injections, and then oral drugs of this group, disaggregants; if there are signs of inflammatory activity, moderate doses of glucocorticosteroids are added, and in case of a sharp decrease in vision and pronounced signs of activity, pulse therapy is used.

The most effective and less toxic regimens for the use of immunosuppressive drugs, routes of their administration are being determined, and drugs that improve microcirculation and/or affect the rheological properties of blood (Heparin, Fraxiparin, Trental, Ralofect, Tiklid) are included in the complex treatment of patients.

In some cases, drugs such as Reaferon are prescribed, and in the presence of infected ulcers, necrosis of the skin or limbs, antibiotics are used. Due to the variety of nosological forms, the choice of drugs at the onset of the disease is influenced by the prevalence of the pathological process and the presence of intercurrent infection. The use of angioprotectors and syndromic therapy is indicated.

Considering the high proportion of neurological pathology, patients with RD should undergo a comprehensive clinical and instrumental neurological examination at an early stage of the pathological process. Diagnosis of RD and complex therapy with glucocorticosteroids and immunosuppressants contribute to the correction of CNS and PNS disorders.

Literature

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N. P. Shilkina, Doctor of Medical Sciences, Professor N. N. Spirin , Doctor of Medical Sciences, Professor I. V. Dryazhenkova YGMA, National Health at the Yaroslavl station " JSC "Russian Railways", Yaroslavl

Treatment of collagenosis

Treatment of collagenosis is always long-term (often lifelong).

The following medications are most often prescribed to patients:

• non-steroidal anti-inflammatory drugs;
• glucocorticoids;
• cytostatics;
• gold preparations;
• aminoquinoline derivatives.

The duration of medications and their doses are selected by the attending physician individually, taking into account the severity of the symptoms of the disease, the age and well-being of the patient, and the presence of other health problems.

During periods of exacerbation of collagenosis, patients can use extracorporeal methods of hemocorrection (hemosorption, cascade filtration of plasma, plasmapheresis). During remission, exercise therapy and physiotherapeutic procedures (ultrasound, electrophoresis of drugs, UHF therapy, magnetic therapy, carbon dioxide, radon and hydrogen sulfide baths) are indicated. Every year, people diagnosed with collagenosis are recommended to undergo spa treatment.