Collagenases – group of the diseases united by the same functional and morphological changes from connecting fabric (mainly, kollagensoderzhashchy fibers). Characteristic manifestation of kollagenoz is the progressing current, involvement in pathological process of various internals, vessels, skin, musculoskeletal systems. Diagnostics of kollagenoz is based on a defeat poliorgannost, identification of the positive laboratory markers given to a biopsy of connecting fabric (skin or sinovialny covers of joints). Most often corticosteroids, immunodepressants, NPVS, aminokhinolinovy derivatives, etc. are applied to treatment of kollagenoz.
Collagenases (collagenic diseases) – the immunopathological processes which are characterized by system disorganization of connecting fabric, polysystem defeat, the progressing current and polymorphic clinical manifestations. In rheumatology it is accepted to carry rheumatoid arthritis, rheumatism to number of kollagenoz, system red to a wolf cub, a system sklerodermiya, nodular periarteriit, dermatomiozit, Wegener, etc. These diseases are united in uniform group on the basis of the general patomorfologichesky sign (fibrinoidny change of collagen) and the pathogenetic mechanism (violation of an immune homeostasis).
Connecting fabric forms a skeleton, skin, to Strom of internals, blood vessels, fills intervals between bodies and makes more than a half of mass of a human body. In an organism treat the main functions of connecting fabric protective, trophic, basic, plastic, structural. Connecting fabric is presented by cellular elements (fibroblasta, macrophages, lymphocytes) and an intercellular matriks (the main substance, collagenic, elastichesky, retikulyarny fibers). Thus, the term "collagenases" not fully reflects the pathological changes happening in an organism therefore now it is accepted to call the given group "diffusion diseases of connecting fabric".
Classification of kollagenoz
Distinguish the congenital (hereditary) and acquired collagenases. Congenital soyedinitelnotkanny are presented to a dysplasia, in particular, by mukopolisakharidoza, Marfan's syndrome, imperfect osteogenesis, Elersa-Danlos's syndrome, elastichesky psevdoksantomy, Stikler's syndrome, etc.
The acquired collagenases, in turn, include hard currency, a sklerodermiya, nodular periarteriit, dermatomiozit, rheumatoid polyarthritis, Shegren's syndrome, rheumatism, system vaskulit, diffusion eozinofilny fastsiit, etc. From them the first four nosological units rank as the big collagenases differing in truly system nature of defeat and weight of the forecast; the others – to small collagenic diseases. It is also accepted to allocate the transitional and mixed forms of diffusion diseases of connecting fabric (Sharp's syndrome).
Reasons of kollagenoz
Congenital collagenases are caused by inherited (genetic) disorder of structure of collagen or a metabolism. The etiology of the diseases acquired system connecting fabric is less studied and clear. It is considered from the point of view of the multifactorial immunopathology caused by interaction of genetic, infectious, endocrine factors and influences of the external environment. Numerous researches confirm communication between concrete system diseases of connecting fabric and a carriage of the anti-genes of a gistosovmestimost of the II class (HLA-D) defined HLA , mainly. So, system red a wolf cub the sklerodermiya - A1, B8, DR3 and DR5 , Shegren's syndrome - with HLA-B8 and DR3 is associated with carriage DR3 . In families of patients with collagenases more often than in population in general, system diseases are registered among relatives of the first degree of relationship.
Concerning the infectious agents involved in development of kollagenoz, scientific research is still conducted. Infectious and allergic genesis of diffusion diseases of connecting fabric is not excluded; the possible role of pre-natal infections, stafilokokk, streptococci, viruses of paraflu, measles, rubella, parotitis, simple herpes, a cytomegalovirus, Epstein-Barre, Koksaki And, etc. is considered.
It should be noted connection of kollagenoz with changes of endocrine and hormonal regulation: from the beginning of a menstrual cycle, abortions, pregnancy or childbirth, a climax. Vneshnesredovy factors, as a rule, provoke an aggravation is hidden the proceeding pathology or act as triggers of emergence of kollagenoz with the corresponding genetic predisposition. The stress, a trauma, overcooling, insolation, vaccination, drug intake and so forth can be such triggers.
Pathogenesis and patomorfologiya of kollagenoz
The pathogenesis of kollagenoz can be presented in the form of the following general scheme. Against the background of a bacterial and virus sensitization of an organism pathogenic immune complexes which settle on a basal membrane of vessels, sinovialny and serous covers are formed and provoke development of a nonspecific allergic inflammation. These processes cause an autoallergiya and an autosensibilization to own fabrics, violation of cellular and humoral factors of immunogenesis, autoantitet hyperproduction to kernels of cages, collagen, an endoteliya of vessels, to muscles.
The perverted immune, vascular and inflammatory reactions at collagenases are followed by pathological disorganization of connecting fabric. Patomorfologichesky changes take place 4 stages: mukoidny swelling, fibrinoidny necrosis, cellular proliferation and sclerosis.
Collagenases are followed by various pathoanatomical changes, however all diseases are united by diffusion involvement in pathological process of connecting body tissue which can meet in various combinations. So at a nodular periarteriit vessels of muscular type mainly are surprised that leads to scarring and a zapustevaniye of the last therefore in a clinical current aneurisms of vessels, hemorrhages, bleedings, heart attacks are quite often noted. Development of a widespread sclerosis (damage of skin and hypodermic cellulose, a pneumosclerosis, a cardiosclerosis, nephrosclerosis) is typical for a sklerodermiya. At a dermatomiozita prevails damages of skin and muscles, and also located in them . System red a wolf cub it is characterized by a polysyndromic current with development of a dermatosis, polyarthritis, Reynaud's syndrome, pleurisy, nephrite, endocarditis, encephalomeningitis, pneumonitis, neuritis, pleksit and so forth.
Symptoms of kollagenoz
Despite variety of kliniko-morphological forms of kollagenoz, in their development common features are traced. All diseases have a long wavy current with alternation of aggravations and remissions, steady progressing of pathological changes. Persistent fever of the wrong type from oznoba and profuzny sweats, symptoms of an allergy, the inexplicable increasing weakness is characteristic. The general for all kollagenoz are system vaskulit, musculoarticulate the syndrome including mialgiya, artralgiya, polyarthritises, miozita, sinovita. Damage of skin and mucous membranes - eritematozny rash, petekhiya, hypodermic small knots, aftozny stomatitis, etc. is often noted.
Damage of heart at collagenases can be followed by development of myocarditis, a perikardit, miokardiodistrofiya, cardiosclerosis, arterial hypertension, ischemia, stenocardia. From respiratory organs pneumonites, pleurisy, a lung heart attack, a pneumosclerosis are noted. The kidney syndrome includes a gematuriya, a proteinuria, kidneys, a chronic renal failure. Violations of activity of a GIT can be presented by the dyspepsia, gastrointestinal bleedings, attacks of abdominal pains feigning cholecystitis, appendicitis, etc.
Polymorphism of a clinical picture is explained by a defeat organospetsifichnost at various forms of kollagenoz. Exacerbations of diffusion diseases of connecting fabric are usually connected with infections, overcooling, hyper insolation, injuries.
Diagnostics of kollagenoz
The collagenase forms the basis for the assumption of this or that form existence of classical clinical laboratory signs. Emergence in blood of nonspecific markers of an inflammation is typical: S-jet protein, increase α2-, fibrinogen, seromukoid, SOE, etc. Great diagnostic value has definition of the immunological markers characteristic of each disease: The CEC, anti-nuclear and rheumatoid factors, antibodies to one - and two-spiral DNA, antistreptolizina-0, antibodies to nuclear anti-genes, complement level, etc. Quite often for statement of the patomorfologichesky diagnosis it is necessary to resort to a biopsy of skin, muscles, a sinovialny cover of joints, kidneys.
The radiological research of bones and joints at which the general come to light (osteoporosis, narrowing of articulate cracks), and also private radiological signs (an uzuration of articulate surfaces can give certain help in diagnostics of kollagenoz at rheumatoid arthritis, aseptic necroses of articulate surfaces at hard currency, osteoliza of disteel phalanxes at a sklerodermiya, etc.). For detection of character and extent of damage of internals ultrasonic methods of diagnostics (EhoKG, ultrasonography of a pleural cavity, ultrasonography of kidneys, ultrasonography of abdominal organs), are used by MPT, KT. Differential diagnostics of various forms of kollagenoz is carried out by the rheumatologist; in need of the patient other experts advise: cardiologist, pulmonologist, immunologist, dermatologist, etc.
Treatment and forecast of kollagenoz
The course of the majority of collagenic diseases progressing and recuring that demands stage-by-stage, long, quite often lifelong treatment. Most often for therapy of various forms of kollagenoz the following groups of medicines are used: steroid (glucocorticoids) and nonsteroid resolvents, cytostatics, aminokhinolinovy derivatives, gold medicines. The dosage and duration of courses is defined strictly individually with type of a disease, sharpness and weight of a current, age and specific features of the patient.
During the periods of aggravations application of extracorporal methods of a gemokorrektsiiya is effective (a plasma exchange, a cascade filtration of plasma, haemo sorption). During remission of kollagenoz physical rehabilitation can be recommended: LFK, medicinal electrophoresis, ultrasound, , magnetotherapy, DMV-therapy, radonic, carbonic, hydrosulphuric medical bathtubs, sanatorium treatment.
The chronic progressing current with multisystem defeat is characteristic of all types of kollagenoz. Purpose of kortikosteroidny or immunosupressivny therapy helps to reduce sharpness of clinical symptoms, to lead to more or less long remission. The most transient development and a heavy current have so-called big collagenases. Death of patients can be caused by renal, cardiovascular, respiratory failure, accession of an interkurrentny infection. For prevention of aggravations of kollagenoz it is important to eliminate the centers of a chronic infection, to undergo dispensary inspection, to avoid excess insolation, overcooling and other provocative factors.