How are systemic vasculitis treated?

Treatment of systemic vasculitis in the active (acute) period should be carried out in the conditions of a specialized (rheumatological) hospital, when remission is reached - the patient must continue treatment on an outpatient basis, under the supervision of a pediatrician, a rheumatologist and, if necessary, narrow specialists.

Effective treatment improves prognosis. To prevent tissue damage, early diagnosis and therapy are required. The choice of methods for treating the disease involves an impact on a possible cause and the main mechanisms of the development of the disease.

Usually use a combination of anti-inflammatory, immunosuppressive drugs, anticoagulants, antiplatelet agents, symptomatic agents. At the same time, it is necessary to strive to achieve a balance of efficiency and toxicity of treatment.

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Pathogenetic treatment of systemic vasculitis in children

Treatment is prescribed taking into account the phase (evolution) of the disease and clinical features. The effect of treatment is assessed by the dynamics of clinical syndromes and laboratory indicators. Activity indices are signs of general inflammatory syndrome (leukocytosis, increased ESR, acute phase proteins), hypercoagulation, which is most pronounced in severe disease, immunological changes (elevation of IgA, IgG, CEC and cryoglobulin levels, ANCA). After treatment in an acute phase hospital, the patient continues to receive outpatient treatment with compulsory dispensary supervision.

The basis of basic therapy for most nosological forms are glucocorticosteroid hormones.

For the treatment of systemic vasculitis, glucocorticosteroids of medium duration, prednisolone and methylpredniheolone (MP), are usually used. Variants of glucocorticosteroid therapy in systemic vasculitis:

1. The daily morning intake of the drug inside in an individually selected dose - at first maximum (at least 1 month) (even in the case of an earlier onset of a positive effect), then - supporting for several years, which most effectively "preserves" remission, prevents relapses.
2. According to indications in severe cases, pulse therapy with metipredom is performed by intravenous administration of high doses of the drug in the form of monotherapy, in combination with cyclophosphamide or synchronously with plasmapheresis. Doses of glucocorticosteroids, indications for use and treatment vary depending on the activity and clinical features of the disease.

In systemic vasculitis, with the exception of Kawasaki disease (in which the corticosteroids are not shown), effective doses of prednisolone are 0.5 to 1.0 mg / kg. With classic nodular polyarteritis prednisolone is prescribed a short course (for malignant hypertension is not prescribed at all), the basic treatment is cyclophosphamide therapy. In the complex with prednisolone cyclophosphamide is obligatory for Wegener's granulomatosis, microscopic polyangiitis, Chang-Strauss syndrome, methotrexate - with nonspecific aortoarteriitis. In the case of Shenlaine-Henoch disease, prednisolone is used in a short course only if mixed variants develop, a severe allergic component or in the treatment of nephritis against a background of basic therapy with heparin and antiplatelet agents. The latter are used in other vasculitis in case of hypercoagulation. Use heparin in an individually selected dose subcutaneously 4 times a day under the control of the definition of blood clotting 2 times a day. Duration of treatment is 30-40 days. For all nosological forms, in case of severe (crisis) flow, plasmapheresis is carried out additionally - 3-5 sessions daily synchronously with pulse therapy.

Glucocorticosteroids in a number of vasculitis, as already mentioned, are not effective enough, therefore, if necessary, the effects of immunological disorders in the treatment use cytostatics (immunosuppressants) - cyclophosphamide, azathioprine and methotrexate. Immunosuppressive drugs inhibit the synthesis of antibodies by B lymphocytes, neutrophilic activity, reduce the expression of adhesion molecules on the surface of endothelial cells, and methotrexate also has antiproliferative activity, which is especially important in the development of the proliferative and granulomatous process characteristic of, for example, nonspecific aortoarteritis, Wegener's granulomatosis.

Cyclophosphamide is the main drug in the treatment of classical nodular polyarteritis, Wegener's granulomatosis, microscopic polyarteritis and Chang-Strauss syndrome; it is also used in four-part therapy of Shenlain-Genocha nephritis in the form of nephritic syndrome. The drug is administered inside 2-3 mg / kg daily or by intermittent course (intravenously monthly for 10-15 mg / kg). Methotrexate is used to treat patients with nonspecific aortoarteritis, in recent years - as an alternative to cyclophosphamide - in Wegener's granulomatosis. The drug is prescribed in a dose of at least 10 mg per square meter of the standard body surface once a week, the duration of treatment is not less than 2 years of remission.

Unfortunately, the anti-inflammatory and immunosuppressive effect of glucocorticosteroids and cytostatics is inseparable from the modeling and cytotoxic effects on metabolic processes. Long-term use of glucocorticosteroids and cytostatics entails the development of severe side effects. In the treatment of cytostatics, it is agranulocytosis, hepato- and nephrotoxicity, infectious complications; in the treatment of glucocorticosteroids - medicinal syndrome of Itenko-Cushing, osteoporosis, delay of linear growth, infectious complications. Therefore, in order to ensure the safety of cytostatics, the presence of persistent manifestations of infection, chronic liver and kidney diseases should be excluded before prescribing them; dose to select under the control of laboratory indicators, combine methotrexate with plakvenilom to mitigate its hepatotoxicity.

For the prevention and treatment of osteopenia and osteoporosis, calcium carbonate, miacalcic and alfacalcidol are currently used. Infectious complications develop in the treatment of glucocorticosteroids and in the treatment of cytostatics. They not only limit the adequacy of the dose of the basic drug, but also support the activity of the disease, which leads to lengthening of treatment and the growth of its side effects.

An effective method of correcting not only the activity of the main process, but also the prevention of infectious complications is the use of intravenous immunoglobulins (IVIG).

Indication for their purpose are: high activity of the pathological process of systemic vasculitis in combination with infection and infectious complications against the background of anti-inflammatory immunosuppressive therapy in remission. For treatment, standard, enriched IgM (pentaglobin) and, according to indications, hyperimmune preparations are used. The drug should be administered at a rate of no more than 20 cap per minute, monitor the patient during the infusion and 1-2 hours after its completion, monitor the level of transaminases and nitrogenous slags in patients with the underlying pathology of the liver and kidneys. The course of treatment is from 1 to 5 e / in infusions, the course dose of standard or enriched IVIG is 200-2000 mg / kg of body weight. According to the indications, IVIG addition is administered 4-2 times a year in a dose of 200-400 mg / kg. A special place for IVIG is in the Kawasaki syndrome. Only treatment with IVIG in combination with aspirin reliably helps prevent the formation of coronary aneurysms and complications.

Dispensary supervision

Children suffering from systemic vasculitis should be on a dispensary record with a rheumatologist. If necessary, a neurologist, an oculist, a dentist, an ENT doctor, a surgeon are involved in the examination. Monthly examinations are recommended during the year after discharge from the hospital, during the second year - every 3 months, then - every 6 months. Objectives of the medical examination: registration of disability, development of individual treatment, systematic clinical and laboratory examination, treatment control, prevention of medical complications, sanation of foci of infection. Prophylactic vaccinations for patients with systemic vasculitis are contraindicated, only in the period of remission for epidemiological indications can be vaccinated with inactivated vaccines. There is a need for continuity between pediatric, adolescent and therapeutic rheumatology services with the development of long-term management tactics for patients with systemic vasculitis.

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