Hemorrhagic vasculitis is a disease with a primary lesion of the capillaries of the skin, joints, gastrointestinal tract and kidneys.
Hemorrhagic vasculitis can begin at any age. However, up to 3 years, children rarely get sick. The maximum number of cases of hemorrhagic vasculitis occurs at the age of 4-12 years.
The onset of the disease is possible in 1-4 weeks after angina, ORVI , scarlet fever or another infectious disease. Vaccines, drug intolerance, food allergies , injury, cooling.
Hemorrhagic vasculitis is based on increased production of immune complexes, activation of the complement system, increased vascular permeability, damage to the capillary wall.
In most children, hemorrhagic vasculitis begins with typical skin rashes – these are small, symmetrically located bruising elements that do not disappear when pressed. The rash is localized on the extensor surfaces of the limbs, around the joints, on the buttocks. Rash on the face, body, palms and feet are less common.The intensity of the rash varies from single to multiple elements with a tendency to merge. With the disappearance of the rash, pigmentation remains, in place of which, with frequent relapses, scaling appears.
The lesion of the joints is the second characteristic sign of hemorrhagic vasculitis, which is observed in 2/3 of patients. It usually appears simultaneously with a rash on the 1st week of illness, or at a later time. The nature of joint damage varies from short-term joint pain to inflammation. It affects mainly large joints, especially the knee and ankle. Periarticular edema develops with a change in the shape of the joints and tenderness; pains last from several hours to several days. Persistent deformation of the joints in violation of their function does not happen.
Abdominal pain is the third most common symptom of hemorrhagic vasculitis. It can appear simultaneously with damage to the skin and joints, and may precede skin and joint changes. Some patients complain of moderate abdominal pain, which are not accompanied by digestive disorders, do not cause much suffering and go away on their own or in the first 2-3 days from the start of treatment. Other pains in the abdomen are paroxysmal in nature, occur suddenly according to the type of intestinal colic, do not have a clear localization. Painful attacks can be repeated many times during the day and last up to several days. Patients complain of nausea, vomiting, unstable stool, sometimes fever. In rare cases, against the background of the specified clinical picture, episodes of intestinal and gastric bleeding are noted.
Kidney damage in hemorrhagic vasculitis is less common than other manifestations of the disease. Kidney damage can be different – from those that quickly disappear during therapy, to a pronounced picture glomerulonephritis ( Schonlein-Henoch nephrite ).
Much less often with hemorrhagic vasculitis, damage to other organs is detected. Pulmonary syndrome may occur in the form of a cough with a small amount of sputum and streaks of blood, sometimes shortness of breath. Described hemorrhagic pericarditis, endocardial hemorrhage. As a rule, these changes are reversible.
More often in children, on the background of moderate and severe disease, systolic murmur appears in the heart of a functional nature. Damage to the central nervous system is caused by inflammation of the cerebral vessels and the meninges and usually appears at the height of skin changes. Patients complain of headache, dizziness, irritability. In some cases, boys with hemorrhagic vasculitis have testicular lesions (often bilateral) – swelling, tenderness.
Changes in laboratory parameters are not specific for hemorrhagic vasculitis. ESR and leukocyte increases, dysproteinemia with an increase in the level of alpha-2-globulins, an increase in non-specific indicators characterizing inflammation, such as DPA, seromucoid, C-reactive protein, titers are possible. antistreptolysin o and antihyaluronidases.
Immunological changes in the form of increased levels of immunoglobulin A, increased circulating immune complexes and cryoglobulins , reduced levels of immunoglobulin G, activity of complement.
Laboratory manifestations of hypercoagulation (increased fibrinogen , soluble fibrin monomer complexes , induced platelet aggregation, inhibition of fibrinolysis ) is most pronounced in severe disease.
The nature of therapy for hemorrhagic vasculitis varies depending on the phase of the disease –
- debut, relapse, remission;
- clinical form – simple (skin), mixed, with kidney damage;
- the severity of clinical manifestations – mild (satisfactory health, scanty rashes, possible pain in the joints), moderate (multiple rashes, joint pain or arthritis, recurrent abdominal pain, traces of blood or protein in the urine ), severe (drainage rash, elements of necrosis, recurrent 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 cases, two drugs are prescribed at the same time to enhance the antiaggregation effect.
The duration of treatment of hemorrhagic vasculitis depends on the clinical form and severity: 2-3 months – for mild; 4-6 months – with moderate; up to 12 months – in case of severe recurrent course and jade of Schönlein – Genoh ; in case of chronic course, they are treated with repeated courses for 3-6 months.
The dose of anticoagulants is selected individually, focusing on the positive clinical dynamics of symptoms (stabilization of skin rashes, the disappearance of abdominal pain, a decrease in the amount of blood excretion in the urine), as well as laboratory parameters (lengthening the blood clotting time or activatedrecalcification time by 2-3 times compared to with the original, the translation of positive paracoagulation tests in negative).
In the absence of proper clinical and laboratory effect, the dose is increased by 50-100 U / kg / day . Subcutaneous administration of heparin into the fiber of the abdomen 3-4 times a day ( Fraxiparine – 2 times) or intravenous injections is used.
With moderate hemorrhagic vasculitis, the course of treatment usually lasts up to 25-30 days; in case of severe to persistent stopping of clinical syndromes, 45-60 days of heparin therapy are necessary ; With the developed jade of Schönlein – Genoh, it lengthens. The abolition of drugs carried out gradually at 100 IU / kg / day every 1-3 days.
Fibrinolysis activators – nicotinic acid and its derivatives ( xanthinol nicotinate , teonikol , komplamin ) – the dose is selected taking into account individual sensitivity, it is usually 0.3-0.6 g per day.
Glucocorticosteroids are effective in severe disease. Treatment with glucocorticosteroids should be carried out against the background of anticoagulant-antiplatelet therapy. With moderate and light during their use is not justified. With a simple and mixed form without kidney damage, the dose of prednisone is 0.7-1.5 mg / kg per day and is used in 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 are appropriate for severe forms of kidney damage in the absence of positive dynamics of treatment with glucocorticosteroids , as well as in the presence of severe skin syndrome with areas of skin necrosis on the background of high immunological activity. For reception use azathioprine 2 mg / kg / day ,cyclophosphamide 2 mg / kg / day for up to 4-6 months. The treatment is carried out under the control of the peripheral blood: with a decrease in the number of leukocytes, cytostatics are canceled.
Transfusion therapy: is carried out in children with severe hemorrhagic vasculitis for 5-15 days during the acute period of the disease, when the clinical manifestations are most pronounced. The structure of transfusion therapy includes: low molecular weight plasma – replacing solutions ( reopolyglukine , reoglyuman ,reomacrodex ) at the rate of 10-20 ml / kg / day ; glucose -novokainovaya 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 2% solution) in 150-250 ml of isotonic sodium chloride solution; inhibitors of proteolytic enzymes ( kontikal 20 000 – 40 000 U / kg / day , trasiolol 50 000 – 100 000 U / day ). The introduction of drugs carried out drip at a rate of 10-15 drops per minute.
Plasma exchange It is aimed at removing toxins, bacteria, inflammatory substances, antibodies, immune complexes, cryoglobulins from the circulation and is indicated for severe hemorrhagic vasculitis, for continuous or undulating recurrence of symptoms. Plasmapheresis contributes to the normalization of blood properties, relieves vasospasm, improves microcirculation, increases the functional activity of immunocompetent cells, increases the sensitivity of patients to drugs.
The volume of exuded plasma is 10-30 ml per 1 kg of body weight of the child (for a course of treatment consisting of 3-8 sessions of plasmapheresis, remove from 2 to 5 volumes of circulating plasma). The first 3-4 sessions are carried out daily, and the next – 1 time in 3 days. The rate of exfusion is 50 ml per minute.To prevent thrombosis, heparin is used at the rate of 100-300 U / kg. As replacement solutions using low molecular weight dextrans, albumin, glucose, saline.
Antihistamines are effective in children with a history of food, drug or household allergies, manifestations of exudative-catarrhal diathesis , allergic diseases ( pollinosis, Quincke swelling, acute obstructive bronchitis, bronchial asthma ). Use tavegil, suprastin, diazolin , fenkarol and other drugs in age dosages for 7-10 days.
Chelators are necessary for patients with burdened allergological history, in the presence of pain in the abdomen, in cases when food agents are a provoking factor of the disease. Enterosorbents bind toxins and biologically active substances in the intestinal lumen, thereby preventing their penetration into the systemic circulation. For this purpose, polyphepane , smecta , enterosorb , activated carbon 3-4 times a day for 5-10 days are prescribed .
Membrane stabilizers prescribed repeated courses with kidney damage or recurrent skin syndrome. Preparations of this group help to reduce the permeability of the vascular wall, improve trophic processes, have an immunomodulatory effect. The most widely used are retinol, tocopherol (vitamin E), rutin,dimephosphone for 1 month.
The outcome of hemorrhagic vasculitis in children is generally favorable. Recovery from debut is observed in more than half of patients. Perhaps the long-term relapsing course of the disease, with the frequency of relapses ranging from a single for several years to monthly. However, over time, as a rule, the disease becomes monosyndromic : only a skin rash (less often with articular syndrome) or chronic kidney damage develops. At the same time, the kidney function remains intact for a long time. Exodus in chronic renal failure extremely rare, with a mixed form of glomerulonephritis or a rapidly progressive form.