How is systemic vasculitis treated?

Treatment of systemic vasculitis in the active (acute) period must be carried out in a specialized (rheumatological) hospital; upon achieving remission, the patient must continue treatment on an outpatient basis, under the supervision of a pediatrician, rheumatologist and, if necessary, specialists.

Effective treatment can improve the prognosis. Early diagnosis and therapy are required to prevent tissue damage. The choice of disease treatment methods involves influencing the possible cause and underlying mechanisms of disease development.

Usually a combination of anti-inflammatory, immunosuppressive drugs, anticoagulants, antiplatelet agents, symptomatic agents is used. In this case, it is necessary to strive to achieve a balance between the effectiveness 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 parameters. Activity indicators are signs of general inflammatory syndrome (leukocytosis, increased ESR, "acute phase" proteins), hypercoagulation, which is most pronounced in severe cases of diseases, immunological changes (increased levels of IgA, IgG, CIC and cryoglobulins, ANCA). After in-patient treatment of the acute phase of the disease, the patient continues outpatient treatment with mandatory dispensary observation.

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

Medium-acting glucocorticosteroids, prednisolone and methylprednisolone (MP), are commonly used to treat systemic vasculitis. Glucocorticosteroid therapy options for systemic vasculitis include:

1. Daily morning oral administration of the drug in an individually selected dose - initially the maximum (suppressive) for at least 1 month (even in the case of an earlier onset of a positive effect), then a maintenance dose for several years, which most effectively "preserves" remission and prevents relapses.
2. According to indications, in severe cases, pulse therapy with metipred is administered by intravenous drip administration of high doses of the drug as a monotherapy, in combination with cyclophosphase or synchronously with plasmapheresis. Doses of glucocorticosteroids, indications for use and treatment methods vary depending on the activity and clinical features of the disease.

In systemic vasculitis, with the exception of Kawasaki disease (in which glucocorticosteroids are not indicated), prednisolone doses of 0.5 to 1.0 mg/kg are effective. In classical nodular polyarteritis, prednisolone is prescribed for a short course (not prescribed at all in malignant hypertension); the basic treatment is cyclophosphamide therapy. Cyclophosphamide is mandatory in combination with prednisolone in Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, and methotrexate in nonspecific aortoarteritis. In Henoch-Schonlein disease, prednisolone is used for a short course only in the case of mixed variants, a pronounced allergic component, or in the treatment of nephritis against the background of basic therapy with heparin and antiplatelet agents. The latter are also used in other vasculitis in case of hypercoagulation. Heparin is used in an individually selected dose subcutaneously 4 times a day under the control of determining blood clotting 2 times a day. The duration of treatment is 30-40 days. For all nosological forms in the case of a severe (crisis) course, plasmapheresis is additionally carried out - 3-5 sessions daily synchronously with pulse therapy.

Glucocorticosteroids are not effective enough for a number of vasculitides, as has already been said, therefore, when it is necessary to influence immunological disorders, cytostatics (immunosuppressants) are used in treatment - cyclophosphamide, azathioprine and methotrexate. Immunosuppressive agents suppress the synthesis of antibodies by B-lymphocytes, the activity of neutrophils, 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 a proliferative and granulomatous process characteristic, for example, of nonspecific aortoarteritis, Wegener's granulomatosis.

Cyclophosphamide is the main drug in the treatment of classic nodular polyarteritis, Wegener's granulomatosis, microscopic polyarteritis and Churg-Strauss syndrome, it is also used in the four-component therapy of Schonlein-Henoch nephritis in the form of nephritic syndrome. The drug is prescribed orally 2-3 mg / kg daily or intermittently (intravenously monthly at 10-15 mg / kg). Methotrexate is used to treat patients with nonspecific aortoarteritis, in recent years - as an alternative to cyclophosphamide - for Wegener's granulomatosis. The drug is prescribed in a dose of at least 10 mg per square meter of standard body surface once a week, the duration of treatment is at least 2 years of remission.

Unfortunately, the anti-inflammatory and immunosuppressive effect of glucocorticosteroids and cytostatics is inseparable from the modeling and cytotoxic effect on metabolic processes. Long-term use of glucocorticosteroids and cytostatics entails the development of severe side effects. In the treatment with cytostatics, these are agranulocytosis, hepato- and nephrotoxicity, infectious complications; in the treatment with glucocorticosteroids, drug-induced Itsenko-Cushing's syndrome, osteoporosis, delayed linear growth, infectious complications. Therefore, in order to ensure the safety of cytostatics, before prescribing them, it is necessary to exclude the presence of persistent manifest infections, chronic liver and kidney diseases in the patient; the dose should be selected under the control of laboratory parameters, combine methotrexate with plaquenil to mitigate its hepatotoxicity.

Calcium carbonate, myacalcic and alfacalcidol are currently used for the prevention and treatment of osteopenia and osteoporosis. Infectious complications develop both during treatment with glucocorticosteroids and during treatment with cytostatics. They not only limit the adequacy of the dose of the basic drug, but also maintain the activity of the disease, which leads to prolongation of treatment and an increase in its side effects.

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

Indications for their use 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. Standard, enriched IgM (pentaglobin) and, if indicated, hyperimmune drugs are used for treatment. The drug should be administered at a rate of no more than 20 drops per minute, the patient should be observed during the infusion and for 1-2 hours after its completion, the level of transaminases and nitrogenous waste products should be monitored in patients with initial liver and kidney pathology. The course of treatment is from 1 to 5 intravenous infusions, the course dose of standard or enriched IVIG is 200-2000 mg/kg of body weight. According to indications, IVIG is additionally administered 4-2 times a year at a dose of 200-400 mg/kg. IVIG occupies a special place in Kawasaki syndrome. Only treatment using IVIG in combination with aspirin reliably helps prevent the formation of coronary aneurysms and complications.

Outpatient observation

Children suffering from systemic vasculitis should be registered with a rheumatologist. If necessary, a neurologist, ophthalmologist, dentist, ENT specialist, and surgeon are involved in the examination. Monthly examinations are recommended for a year after discharge from the hospital, every 3 months during the second year, and then once every 6 months. The objectives of the medical examination: registration of disability, development of an individual regimen, systematic clinical and laboratory examination, monitoring of treatment, prevention of drug complications, sanitation of foci of infection. Preventive vaccinations are contraindicated for patients with systemic vasculitis. Only during the period of remission, according to epidemiological indications, vaccinations with inactivated vaccines can be administered. Continuity is necessary between pediatric, adolescent and therapeutic rheumatology services with the development of tactics for long-term management of patients with systemic vasculitis.

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