Vasculitis - Causes, symptoms and treatment. MF.

Vasculitis is a heterogeneous group of diseases with a similar pathogenesis, in the structure of which the basis is the multiple lesion of the inflammatory vascular wall with subsequent involvement in the inflammatory process of various body systems. During the whole period of studying the group of diseases, various scientists made attempts to create their classification.

The causes of vasculitis.

Since primary vesiculites are a fairly heterogeneous group, it is difficult to talk about any single reasons for the development of this pathology. In most cases, the cause of the disease remains unknown and subsequently has no significant effect on the course of the pathological process. The infectious theory of the origin of vasculitis remains dominant. With a number of nosological forms, vasculitis has a chronological connection with various infectious agents. However, it must be remembered that the presence of untreated foci of chronic infection, even if they did not cause vasculitis, can lead to recurrence of the disease and the development of secondary infectious complications, which makes it difficult to study the patient. A prerequisite for discussing the role of viruses as the cause of vasculitis was the work that described cases of vasculitis after hepatitis B with the persistence of the virus in the blood, and sometimes we find immune complexes containing the virus antigens in the wall of the affected vessel.

Some causes of vasculitis have been known for a long time( the effect of various serums and medications).The role of drugs in the development of vasculitis is beyond doubt. Signs of systemic damage can appear immediately after contact of the drug with the patient's body or come as a result of restructuring of chronic drug disease. Currently, more than 150 medicines are known, the administration of which triggers the development of vasculitis. Most often, this type of reaction can play on the use of sulfanilamide preparations, antibiotics, iodine preparations, radiocontrast substances, B group vitamins, analgesics, tuberculostatics. You can not exclude hereditary predisposition, which is due to a defect in the immune response and altered reactivity of the vessel walls.

Symptoms of vasculitis

Principles of clinical diagnostics of vasculitis.

The basic principle of diagnosing vasculitis is clinical polymorphism. Early diagnosis is necessary for the timely initiation of treatment, since, the later the diagnosis is established, the more likely the development of irreversible processes in organs and systems. The main role in the diagnosis should be attributed to the clinical manifestations of the disease, i.e. complaints and changes detected during examination and an objective examination of the patient - symptoms. The sex of the patient, his age is of great importance. So, some forms of vasculitis are mainly men( obliterating thrombangiitis of Burger), others( nonspecific aortoarteritis) - young girls. Lesion of the temporal arteries, as a rule, develops in persons older than 55 years. Hemorrhagic vasculitis occurs mainly in children or young people.

Complaints of patients with vasculitis are diverse and depend on the localization of the affected vessel, the phase of the disease course and the severity of functional disorders of the organs. Of particular importance is the sequence of the onset of symptoms and the time of illness. As a rule, vasculitis begins acutely or subacute, with the initial signs of the inflammatory process and the subsequent development of organ pathology. Among the first symptoms of vasculitis, general nonspecific( or constitutional) and local characteristic syndromes can be distinguished, which in combination determine the clinical picture of a particular nosological form of the disease.

The general symptoms of vasculitis include: weight loss, fever, headache, fatigue. Characterized by a constant low-grade fever, which does not decrease with the use of antibiotics. The body temperature can be lowered independently or with the appointment of glucocorticoids. Weight loss in some variants of vasculitis is a classic feature, and a significant reduction in body weight in a short period of time is not only diagnostic, but also is evidence of disease activity.

Skin syndrome is the earliest and most diverse and occurs in 40 to 50% of cases. Nodules, petechiae, spots, cutaneous purpura, ulcers can be observed. In a number of cases, a net pattern of the skin appears as a marble type - a net levido. And the intensity of cyanotic color can vary from moderately to pronounced. This symptom reflects the defeat of the vessels of the skin. Most net levido is observed with nodular periarteritis, Wegener's granulomatosis, as well as with antiphospholipid syndrome. All these skin lesions can be transformed into ulcerative-necrotic form. In this case, the resulting trophic ulcers, as a rule, are painless and have a scanty discharge. The speed of their healing is not so much dependent on local therapy as on the effectiveness of treatment of vasculitis itself. In clinical practice cases of the development of gangrene of fingers and feet are described.

Dermal manifestations of vascular lesions in vasculitis

Muscle pain or so-called myology, occurs in more than half the cases of vasculitis and is due to impaired circulation in certain muscle groups. The pronounced pain syndrome in the muscles can precede the development of lesions to the peripheral nervous system, which will manifest itself later in a violation of the sensitivity of the distal parts of the fingers and toes.

Joint damage is a common symptom. Typically, the defeat of large joints of the lower and upper limbs - knee, ankle, shoulder.

Kidney damage - one of the most frequent and prognostically unfavorable symptoms, occurs in 20 - 40% of patients, depending on the form of vasculitis. Clinical symptoms of kidney damage appear at the height of the disease and indicate already pronounced changes. Also, various manifestations of the disease from the gastrointestinal tract, respiratory system and heart are possible.

You should see a doctor for a rheumatologist if you have signs of an inflammatory process, such as: fever, general weakness, weight loss, not related to a change in diet. Depending on the localization of the process in the arterial bloodstream, visual impairment, muscle pain, and blood in the urine may occur.

Diagnosis

There are no specific laboratory tests for the diagnosis of vasculitis. Basically, laboratory changes reflect the activity of the disease( increase in blood-clotting proteins, leukocytes and ESR) and the degree of vascular lesions in individual organs. Great importance is given to biopsy, a site where changes are more pronounced. For specific vasculitis has its own characteristics in diagnosis and clinic. For example, with Wegener's granulomatosis, normochromic anemia, mild leukocytosis without eosinophilia, increased ESR, thrombocytosis, and hypergammaglobulinemia are characteristic. The acute phase proteins correspond to the activity of the disease. Immunoglobulins may increase, which is more relevant for IgA.Rheumatoid factor is found in more than half of patients and is most often determined in the developed stage with pronounced disease activity. For each of their subspecies, Vasculitis has its own criteria, the ratio of which and their number can be said with an absolute guarantee of the presence or absence of disease.

Treatment of vasculitis

Almost all vasculitis is a chronic progressive disease, when, periods of exacerbation are replaced by remission, which is achieved only by targeted treatment. At present, immunosuppression( immunosuppression) and anti-inflammatory treatment are given as the main directions of vasculitis treatment, which is provided by the joint application of glucocorticoids and / or cytotoxic drugs that allow limiting or interrupting the development of immune inflammation.

The drug of choice is prednisolone, because it is able to quickly remove edema of the vascular wall, which leads to a decrease in the manifestations of ischemic syndrome. At the same time, prolonged use of glucocorticosteroids in a maintenance dose prevents further stenosis of large vessels.

However, it is considered proven that a significant improvement in the prognosis for life is associated with the use of cytotoxic drugs. Cyclophosphamide - the dose of this drug can vary within fairly wide limits, usually the initial dose in the period of pronounced clinical manifestations of the disease is 2 - 3 mg / kg per day. To intensify the therapy of vasculitis in severe torpid flow, combinations of high doses of methylprednisolone and cyclophosphamide at a dose of 1000 mg intravenously are used.

A good effect in treatment, as concomitant therapy, is vasodilator drugs( nicotinic acid, persantine, compliance) and antithrombotic drugs( quarantil, trental, agapurin) drugs are prescribed courses for a long time.

In recent years, extracorporeal hemocorrection methods have been used extensively to improve microcirculatory disorders( plasmapheresis in combination with hemoxygenation and ultraviolet irradiation of blood).Also with the purpose of correction of microcirculatory disorders, taking into account the leading mechanism that caused them, the following groups of drugs can be used: anticoagulants( direct and indirect) - heparin, fractiparin;methylxanthines - trental, agapurine;group of nonsteroidal anti-inflammatory drugs( small doses of aspirin - 75 - 125 mg / kg).

Carrying out active pathogenetic therapy throughout the disease allows not only to prolong the life of patients, but also to return some of them to work. This pathology can not be treated with herbal preparations, only those drugs listed here have a proven, positive effect.

Possible complications of vasculitis:

• Disseminated intravascular coagulation syndrome( ICD)
• Bleeding and thromboses
• Arterial hypertension
• Nephrotic syndrome
• Pulmonary hypertension
• Infectious complications.

Forecast.

This group of diseases has a very high percentage of complications, timely treatment can slow down the development of many of them. In patients with vasculitis without specific treatment with immunosuppressive therapy, the prognosis is not favorable, a 5-year survival rate is 10% of the total number of patients. With early active therapy with cytostatics in combination with glucocorticosteroids, 5-year survival is 60 to 90%.

Doctor therapist Zhumagaziev E.N.