Collagenosis is an immunopathological process, accompanied by the development of degenerative disorders with a predominant involvement of connective tissue, characterized by a progressive course and a wide range of clinical manifestations.
Diagnosis of collagen, occurring in any clinical version, usually requires clinical thinking by the treating specialist and a sufficient level of equipment of the medical institution. The complex of diagnostic measures is based on the use of various laboratory techniques, however, only puncture biopsy with subsequent histological and cytological analysis of the punctate has a 100% certainty in this situation.
In the treatment of collagenosis, the principle of individual approach, continuity and expediency of prescribing a particular component of drug therapy is applied. All clinical variants of collagenosis are united by unified pathomorphological and etiopathogenetic mechanisms of its development.
Structures of connective tissue in a greater or lesser quantitative ratio are part of all organs and structures of the human body, which explains the second name of this pathology - "systemic collagenosis" or "diffuse connective tissue disease."
A large group of collagens are the so-called genetically determined variants, which are caused by an inborn violation in the structure of collagen and accompanying systemic metabolic disturbances. As a confirmation of the priority role of the hereditary factor in the development of collagenosis, the fact of registration of a systemic diffuse disease in representatives having a first-degree related relationship is evidence.
When establishing the conclusion "acquired collagenosis" it is almost impossible to determine the etiologic factor of its occurrence, in connection with which, the majority of specialists in this field reject the theory of the acquired origin of this pathology. Nevertheless, there are many factors that are provocateurs of the debut of clinical manifestations of congenital collagenosis, which include: adverse effects of environmental factors, infectious disease of the body and imbalance of the hormonal status of a person.
Currently, a large number of randomized studies are being conducted that confirm the correlation between the development of collagenosis and infectious and viral agents.
The provoking factor in the development of the debut of collagenosis is the violation of hormonal regulation, which occurs in pregnancy, puberty and menopause in women. Trigger mechanisms that trigger the development of manifestations of collagenosis are excessive psychoemotional load, and collagenoses in children can develop even after vaccination.
With all pathogenetic and clinical variants of collagenosis, the mechanism of development of pathomorphological changes in the structure of connective tissue occurs on a single principle and consists in the gradual replacement of immunoproliferative changes. Under the influence of provoking factors, or triggers in the form of bacterial-infectious sensitization of the body, the formation of pathogenic immune complexes that settle on the membranes of the vascular walls and serous membranes, where nonspecific allergic inflammation develops, starts. These processes entail the development of auto-allergy and autosensibilization to the tissues of the body, which is accompanied by a violation of the immunogenesis of the cellular and humoral type and the hyperproduction of antibodies to the cellular structures of collagen, vascular endothelium, and muscle fibers.
The result of the distortion of the immune mechanisms, as well as the existing inflammatory changes, is pathological disorganization of the connective tissue, proceeding gradually as a pathological process change (mucoid swelling, fibrinoid necrosis, cell proliferation and sclerosis).
Various pathomorphological variants of collagenosis are accompanied by a lesion of connective tissue structures of this or that localization to a greater or lesser extent. For example, for nodular periarteritis, the connective tissue of the vessels is more common, and with scleroderma, there is a primary lesion of connective tissue with localization in internal organs and subcutaneous fat. The broadest spectrum of lesions is systemic lupus erythematosus , which is undifferentiated collagenosis.
Classification of collagens
The basis of etiopathogenetic classification of collagenosis is the principle of origin of degenerative involvement of connective tissue. Accordingly, the congenital and acquired form of this pathology is distinguished. Congenital degenerative connective tissue diseases are very rare and their diagnosis is extremely difficult ( mucopolysaccharidosis , elastic pseudocanthoma, Marfan syndrome and others).
Acquired collagenoses are represented by a wide range of pathomorphological variants of the flow, and therefore the international classification divides them into "large collagenoses" in the form of dermatomyositis , systemic lupus erythematosus, nodular periarteritis and scleroderma, characterized by a severe course and a non-derestricted approach to connective tissue damage. The remaining forms of collagenosis are referred to as "small", which are characterized by latent flow and the absence of serious complications.
The most difficult for the patient's health prognosis is the syndrome of Sharp syndrome, which combines pathogenetic and clinical signs of various forms of diffuse involvement of connective tissue.
Each of the pathomorphological variants of collagenosis differs in the specificity of clinical manifestations, but there are common features characteristic of all forms of this pathology (intermittent flow with the obligatory presence of periods of exacerbations and complete remission with simultaneous progression of pathomorphological changes).
The period of exacerbation, as a rule, is accompanied by an active febrile syndrome, which is characterized by a significant increase in body temperature above 38 ° C with episodes of chills and profuse sweating. The main "companion" of fever with collagen is asthenic syndrome, manifestations of which is unmotivated progressive weakness, inability to perform usual physical activity and depression of mood.
Characteristic manifestations of collagenosis, which allow an experienced specialist to suspect the presence of this pathology in the patient, is a symptomatic complex of musculo-articular lesion in the form of progressive myalgia of different localization, aching joint pains with accompanying impairment of their motor function. In some situations, the musculo-articular syndrome is combined with the defeat of the skin with the formation of nonspecific rashes in the form of subcutaneous nodules, erythema and petechia .
Due to the fact that connective tissue is present in the development of all internal organs, with collagenesis, the defeat of these structures inevitably progresses with the development of degenerative irreversible changes ( cardiosclerosis , myocardial dystrophy, pneumosclerosis, renal amyloidosis with chronic renal insufficiency). Nonspecific manifestations of collagenosis is the defeat of the organs of the gastrointestinal tract, simulating the course of other diseases in the form of functional dyspepsia, gastrointestinal bleeding and abdominal pain syndrome. Periods of exacerbations, as a rule, are stimulated by the presence of infectious diseases, excessive insolation, hypothermia, or exposure to stress factors.
Unfortunately, in most cases the clinical criteria of collagenosis are not specific, so the presence of symptoms and complaints of the patient is not a predetermining factor in establishing a reliable diagnosis. In this situation, laboratory diagnostic manipulations are useful, allowing to identify specific and nonspecific markers of collagenosis.
Nonspecific inflammatory criteria is the detection in the blood of an elevated level of the globulin fraction of protein, ESR and C-reactive protein, the detection of which does not allow to establish the exact pathomorphologic form of collagenosis, but testifies to the development of diffuse changes in the connective tissue of inflammatory genesis. To determine the pathogenetic variant of collagenosis, a whole range of laboratory techniques is currently used, but only the histological analysis of the punctate, seized from the lesion area, is 100% reliable.
After establishing a reliable diagnosis of "collagenosis", taking place in this or that pathomorphological variant, the initial task of the attending physician is to convince the patient of the need for lifelong treatment with the use of a whole range of therapeutic and preventive measures. The patient should adhere to the rules of the regime of work and nutrition with the mandatory avoidance of periods of excessive hypothermia or hyperinsurance, which are the main agents of aggravation of collagenosis.
When the patient first detects signs of any variant of collagenosis, hospitalization of a rheumatological profile with a view to selecting an individual drug treatment regimen is a prerequisite. After relief of acute manifestations of the disease, the patient undergoes treatment on an outpatient basis, and with the onset of a period of complete remission, it is absolutely necessary to continue the rehabilitation course of therapy in a sanatorium of a specific profile.
Drug treatment is only part of the complex therapy of collagenosis, and its use occurs most often during the period of activity of the inflammatory process. With different pathomorphological variants of collagenoses, individual methods and schemes of drug-induced anti-inflammatory correction have been developed. The drug of choice with high evidentiary efficacy is a group of non-steroidal anti-inflammatory drugs (Brufen in a maximum daily dose of 2.4 g).
In a situation where the patient has signs of high activity of the process, the rapid progression of pathological changes and clinical manifestations, severe visceral lesion, the necessary condition is the appointment of an adequate regimen of glucocorticosteroid therapy (prednisolone at a maximum daily dose of 15 mg). And only in case of development of adverse reactions in response to the intake of corticosteroids is the appointment of cytostatic therapy (Cyclophosphamide in a daily dose of 0.1 g, Azathioprine at the maximum dose of 5 mg).
Episodes of systemic scleroderma and rheumatoid arthritis are well treatable with D-penicillamine 250 mg daily for at least two months, followed by gradual titration of the dose to 750 mg (every 30 days for 250 mg increase the dose of the drug).
Extracorporeal methods of hemocorrection in the form of plasmapheresis, hemosorption and cascade plasma filtration possess good efficacy in terms of arresting acute clinical manifestations of collagenosis.
Rehabilitation complex of measures consists of the application of various kinds of physiotherapy techniques in the form of drug electrophoresis, ultraphonophoresis, magnetotherapy in a sanatorium of a specific profile. As a preventive method of treating collagenosis, sanation of chronic foci of infection is considered.