Hemorrhagic vasculitis - a disease mainly affecting the capillaries of the skin, joints, gastrointestinal tract and kidneys.
Hemorrhagic vasculitis may begin at any age. However, up to 3 years old children rarely get sick. The maximum number of cases of hemorrhagic vasculitis occurs between the ages of 4-12 years.
Onset of the disease is possible after 1-4 weeks after sore throat, SARS, scarlet fever or other infectious disease. In some patients the disease is preceded by the development of vaccination, drug intolerance, alimentary allergy, Trauma, cooling.
The basis hemorrhagic vasculitis lie increased production of immune complexes, complement activation system, increased vascular permeability, damage to the capillary walls.
Manifestations of hemorrhagic vasculitis
Most children hemorrhagic vasculitis starts with the typical skin rash - it melkopyatnistye symmetrically arranged sinyachkovye elements do not disappear when pressed. The rash is localized on the extensor surfaces of the extremities, around the joints, on the buttocks. Rashes on the face, body, hands and feet are less likely. The intensity of the eruption varies - from single to multiple elements with a tendency to merge. At the expiring of the rash remains pigmentation, on the spot where peeling occurs when frequent relapses.
Joint disease - a second characteristic feature of hemorrhagic vasculitis observed in 2/3 patients. Usually it appears simultaneously with a rash on the 1st week of the disease, or at a later date. The nature of joint damage ranges from short-term joint pain to inflammation. It affects predominantly large joints, particularly the knee and ankle. Periarticular edema develops with changes in the shape and tenderness of the joints; pain kept from several hours to several days. Resistant strain of joints in violation of their functions do not happen.
Abdominal pain - the third frequency indication hemorrhagic vasculitis. He can appear simultaneously with skin lesions and joints, and may precede cutaneous articular changes. Some patients complain of mild abdominal pain, which is not accompanied by digestive disorders do not cause much suffering and are alone or in the first 2-3 days of starting treatment. In other abdominal pains are paroxysmal in nature, appear suddenly on the type of intestinal colic, there is no precise localization. Painful attacks can be repeated many times during the day and last up to several days. Patients complain of nausea, vomiting, unstable chair, and sometimes fever. In rare cases, the background of this clinical picture observed episodes of intestinal and gastric bleeding.
Photo: site of the Department of dermatology Tomsk Military Medical Institute
Kidney damage in hemorrhagic vasculitis is less common than other manifestations of the disease. Kidney damage may be different - from those that disappear quickly during therapy, to severe picture glomerulonephritis (Henoch-Schonlein purpura nephritis).
Much less often in hemorrhagic vasculitis revealed damage to other organs. Pulmonary syndrome can occur in the form of cough with little phlegm and blood veins, and sometimes shortness of breath. described hemorrhagic pericarditis, Bleeding in the endocardium. Usually, these changes are reversible.
More often in children with moderate and severe disease appears systolic murmur in the heart of a functional nature. Damage to the central nervous system caused by inflammation of the brain vessels, meninges, and usually appears at the height of the skin changes. Patients complain of headache, dizziness, irritability. In some cases, boys with hemorrhagic vasculitis observed testicular lesion (often bilateral) - swelling and pain.
Diagnostics
Changes in laboratory parameters are nonspecific during hemorrhagic vasculitis. Possible increase in ESR and leukocyte Dysproteinemia with increased levels of alpha-2-globulin, increased nonspecific indicators characterizing the inflammation - DPA, seromucoid, C-reactive protein, titres About antistreptolisin and antigialuronidazy.
Immunological changes in the form of increased levels of immunoglobulin A, increase of circulating immune complexes and cryoglobulins, lower levels of immunoglobulin G, the complement activity.
Laboratory manifestations hypercoagulable (raising fibrinogenSoluble complexes fibrinmonomerov induced platelet aggregation, inhibition of clot lysis) most pronounced in severe disease.
Treatment of hemorrhagic vasculitis
Character therapy of hemorrhagic vasculitis differs depending on the phase of disease -
- debut, relapse, remission;
- Clinical forms - Easy (skin), mixed with renal disease;
- the severity of the clinical manifestations - light (satisfactory state of health, neobilnye rashes, may joint pain), mid (multiple rashes, joint pain or arthritis, recurrent abdominal pain, traces blood or squirrel in urine), Heavy (drain rash, necrosis elements retidivirute angioedema, Persistent abdominal pain, gastrointestinal bleeding, blood in the urine, nephrotic syndrome, acute renal failure);
- the nature of the disease - acute (up to 2 months), prolonged (up to 6 months), chronic (relapsing or developing nephritis Henoch - Schönlein).
Antiplatelet agents are used in all forms of the disease. Curantil (dipyridamole, persantin) 5-8 mg / kg per day in 4 divided doses; Trental (pentoxifylline, agapurin) 5-10 mg / kg per day in 3 divided doses. In severe antiagregatsionnogo to enhance the effect of the two drugs administered simultaneously.
Duration of treatment of hemorrhagic vasculitis depends on the clinical form and severity: 2-3 months - in less severe; 4-6 months - with moderate; up to 12 months - for severe recurrent course and nephritis Henoch - Schönlein; in chronic treatment repeated courses conducted for 3-6 months.
Dose anticoagulants are selected individually, focusing on the positive dynamics of clinical symptoms (stabilization of skin rashes, disappearance of abdominal pain, a decrease in the degree of separation of blood in the urine), as well as laboratory parameters (prolongation of clotting time blood or activated recalcification time 2.3 fold in comparison with the initial translation of positive tests in paracoagulation negative).
In the absence of adequate clinical and laboratory effects increase the dose of 50-100 IU / kg / day. Use subcutaneous administration of heparin in the stomach tissue of 3-4 times a day (fraxiparin - 2 times), or intravenous injection.
When to moderate hemorrhagic vasculitis treatment generally lasts up to 25-30 days; in severe to persistent relief of clinical syndromes must be 45-60 days of heparin therapy; when razvivshemsya nephritis Henoch - Schönlein he lengthened. Abolition of drugs is carried out gradually by 100 U / kg / day every 1-3 days.
Activators of fibrinolysis - nicotinic acid and its derivatives (xantinol nicotinate, teonikol, komplamin) - dose is selected taking into account the individual sensitivity, it is generally 0.3-0.6 g per day.
Glucocorticoids are effective in severe disease. Glucocorticosteroid should be carried out against the background of anticoagulant-antiplatelet therapy. At moderate and light during their use is not justified. In simple or mixed form without kidney damage prednisolone dose is 0.7-1.5 mg / kg per day and used a short course of 7-20 days. With the development of nephritis Schonlein - Henoch administered 2 mg / kg per day for 1-2 months, followed by reduction of 2.5-5.0 mg of 1 every 5-7 days until complete cancellation.
Cytostatics suitable for severe forms with renal disease in the absence of positive dynamics of treatment corticosteroids, and the presence of severe cutaneous syndrome with patches of skin necrosis at the background of high immunological activity. Used for receiving azathioprine 2 mg / kg / day, 2 mg cyclophosphamide / kg / day for up to 4-6 months. Treatment is carried out under the control of peripheral blood: white blood cell count decreases cytostatics overturned.
Transfusion therapy: is conducted in children with severe hemorrhagic vasculitis for 5-15 days during the acute phase of the disease when clinical manifestations are most pronounced. The composition of transfusion therapy include: low molecular Plasma substitutes solutions (reopoligljukin, reoglyuman, reomakrodeks) at the rate of 10-20 ml / kg / day; glucose-novocaine mixture (5% glucose solution and 0.25% novocaine solution ratio of 2: 1 or 3: 1) in an amount of 10 ml per 1 kg of body weight, but not more than 100 ml; antispasmodics - aminophylline (5 mg / kg / day), no-spa (2 ml of a 2% solution) in 150-250 ml of isotonic sodium chloride solution; inhibitors of proteolytic enzymes (contrycal of 20 000 - 40 000 IU / kg / day, trasilol 50,000 - 100,000 U / day). Administration of drugs carried drip at a rate of 10-15 drops per minute.
plasmapheresis is directed to the removal of circulating toxins, bacteria, inflammatory substances, antibodies, immune complexes, cryoglobulins and shown in severe hemorrhagic vasculitis, undulating continuous or recurrence symptoms. Plasmapheresis helps normalize blood properties, removes vasospasm, improve microcirculation, increases the functional activity of immune cells, increases the sensitivity of patients to drugs.
Eksfuziruemoy plasma volume is 10-30 ml per 1 kg body weight (per course of treatment consisting of treatments 3-8 plasmapheresis removed from 2 to 5 volumes of circulating plasma). The first 3-4 sessions conducted daily, and follow-up - 1 every 3 days. exfusion rate 50 ml per minute. To prevent clotting heparin is used the rate of 100-300 U / kg. As replacement solutions using low molecular weight dextrans, albumin, glucose, saline.
Antihistamines are effective in children with food, medicine or household history of allergy, the manifestations of exudative-catarrhal diathesisAllergic diseases (pollen disease, angioedema, acute obstructive bronchitis, asthma). Use tavegil, suprastin, diazolin, fenkarol and other drugs in the age dosages for 7-10 days.
Chelators are required for patients with allergic history, in the presence of abdominal pain, in cases where dietary agents were precipitating factor disease. Chelators bind toxins in the lumen of the intestine and biologically active substances, thereby preventing their penetration into the systemic circulation. For this purpose, appoint Polyphepanum, smectite enterosorb, activated carbon 3-4 times a day for 5-10 days.
Membrane prescribed repeated courses with kidney disease or relapsing course cutaneous syndrome. Drugs in this group may reduce the permeability of the vascular wall, improve trophic processes have immunomodulatory effects. The most widely used retinol, tocopherol (vitamin E), rutin, dimephosphone for 1 month.
Complications and prognosis
The outcome of hemorrhagic vasculitis in children generally favorable. Recovery after the opening notes of more than half of the patients. Possible long-term relapsing course of disease, and the recurrence rate ranges from single a few years before monthly. Over time, however, as a rule, the disease takes on the character monosindromnogo: only skin rash (sometimes - with articular syndrome) or develop chronic kidney disease. In this case, kidney function remains intact for a long time. outcome in chronic renal failure It is extremely rare, with a mixed form of glomerulonephritis or rapidly progressive form.