Hemorrhagic vasculitis (Henoch-Schönlein disease). Hemorrhagic vasculitis, or Henoch-Schönlein disease (purpura): causes, symptoms, treatment

Henoch-Schönlein disease

Hemorrhagic vasculitis- one of the most common hemorrhagic diseases, which is based on multiple microthrombovasculitis, affecting the vessels of the skin and internal organs. The disease often occurs in childhood and among children under 14 years of age it is observed with a frequency of 23-25 ​​per 10,000.

Currently, it has been proven that hemorrhagic vasculitis belongs to immune complex diseases in which microvessels undergo aseptic inflammation with more or less deep damage to the walls, thrombosis and the formation of circulating immune complexes(CEC).

Pathogenesis

The reason for the development of this pathology is the formation of circulating immune complexes in the bloodstream. These substances settle on the inner surface of blood vessels, thereby causing their damage.

Classification

This reference book provides the classification of hemorrhagic vasculitis by G. A. Lyskina (2000).

Form (evolution) of the disease:

1. initial period;

   improvement;

   exacerbation.

Clinical forms:

2. mixed.

Clinical syndromes:

2. articular;

   abdominal;

   renal.

Severity.

general condition - satisfactory;

mild rashes;

arthralgia is possible.

Medium heavy:

general condition - moderate;

profuse rashes;

arthralgia, arthritis;

periodic abdominal pain;

microhematuria;

slight proteinuria (traces of protein in the urine).

general condition - serious;

profuse confluent rashes with elements of necrosis;

chronic angioedema;

persistent abdominal pain;

gastrointestinal bleeding;

gross hematuria;

nephrotic syndrome;

acute renal failure.

Character of the current:

acute (up to 2 months);

prolonged (up to 6 months);

chronic.

Clinic

With hemorrhagic vasculitis, blood vessels in any area can be affected, including the lungs, brain and its membranes.

Skin syndrome is the most common. It symmetrically affects the limbs, buttocks, and less commonly the torso. A papular-hemorrhagic rash occurs, sometimes with blisters. The rashes are of the same type, at first they have a distinct inflammatory basis, in severe cases are complicated by central necrosis and become covered with crusts, leaving pigmentation for a long time. When pressed, the elements of the rash do not disappear.

Joint syndrome often occurs together with skin syndrome or several hours or days after it in the form of pain of varying intensity in large joints (knees, elbows, hips). After a few days, the pain goes away, but with a new wave of rashes it can occur again. In some cases, articular damage is persistent and stubborn, reminiscent of rheumatoid polyarthritis.

Abdominal syndrome is more often observed in childhood (in 54-72% of patients), in approximately 1/3 it predominates in clinical picture, in some cases precedes skin changes, which makes diagnosis very difficult. The main symptom is severe abdominal pain, constant or cramping, sometimes so intense that patients cannot find a place in bed and scream for many hours. The pain is caused by hemorrhages in the intestinal wall. These hemorrhages may be associated with blood soaking intestinal wall and mucous membrane, bleeding from it and from areas of necrosis, bloody vomiting, melena (admixture of blood in the stool) or fresh blood in the stool, as well as false urges with frequent bowel movements or, conversely, with its delay. From the very beginning, fever and more or less pronounced leukocytosis (an increase in the number of leukocytes in the blood) are detected. With heavy bleeding, collapse develops ( fainting) and acute posthemorrhagic anemia. In some cases frequent vomiting leads to a large loss of fluid and chlorides. The coagulogram reveals hyperthrombocytosis and hypercoagulation.

In a significant proportion of patients, abdominal syndrome is short-lived and goes away on its own in 2-3 days. Periods of severe pain may alternate with pain-free intervals lasting about 1-3 hours. This helps to distinguish abdominal syndrome from acute surgical diseases of the organs abdominal cavity. Such differentiation is especially difficult in patients without skin and joint manifestations and with symptoms of peritoneal irritation. More often abdominal syndrome imitates acute intestinal obstruction (intussusception), appendicitis, ovarian torsion and cysts, perforation of intestinal ulcer.

Comparative diagnostics can cause certain difficulties for the doctor - this is due to the fact that hemorrhagic vasculitis itself can cause all of the listed surgical diseases of the abdominal organs. For example, many cases of intussusception (invasion of one section of the intestine into another) and intestinal obstruction due to compression or closure of its lumen by a hematoma (especially in children under 2 years of age), intestinal necrosis and perforation (formation of a through defect), acute appendicitis have been described. and other complications requiring surgical intervention. Difficulties differential diagnosis in such a situation, they lead to the fact that some patients with hemorrhagic vasculitis undergo unnecessary surgical interventions.

In adult patients, abdominal syndrome is observed less frequently and in most cases does not serve as a basis for diagnostic laparotomy, and is rarely complicated by intestinal obstruction and peritonitis (inflammation of the peritoneum). In old age

age, an abdominal variant of the disease is sometimes observed with vague and not always pronounced abdominal pain and persistent intestinal bleeding, the source of which cannot be determined. Looking for a malignant neoplasm, hidden intestinal ulcer or bleeding polyp in similar cases Often they go for a trial laparotomy and a wide examination of the abdominal organs. In old age with hemorrhagic vasculitis, such an operation, which does not give any tangible results, ends, as a rule, with intestinal atony (complete lack of tone) and dynamic intestinal obstruction, a sharp increase in general intoxication, the addition of cardiovascular failure and the death of the patient. Meanwhile, correct recognition of Henoch-Schönlein disease in such cases, or even conducting a trial course of treatment for this disease in diagnostically unclear cases, allows you to quickly relieve all symptoms and avoid unindicated and dangerous surgical intervention.

Renal syndrome is found in 1/8-1/2 of the patients and more often develops as acute or chronic glomerulonephritis - with micro- or macrohematuria (blood in the urine), proteinuria (from 0.33 to 30% protein in the urine). Arterial hypertension with this form of kidney pathology is rare. Possible nephrotic syndrome. Kidney damage often does not occur immediately, but 1–4 weeks after the onset of the disease. Signs of nephritis may persist for only a few weeks or months, but there is also a protracted or chronic course of the disease, which sharply worsens the prognosis. In some patients, kidney damage rapidly progresses, resulting in uremia in the first 2 years of the disease. In general, kidney damage is a potentially dangerous manifestation of hemorrhagic vasculitis, and therefore the attending physician must very carefully monitor the composition of urine and kidney function throughout the course of the disease.

Vascular damage to the lungs is detected much less frequently, sometimes leading to the development of fatal pulmonary hemorrhage. Also, in quite rare cases, a cerebral form of the disease develops, occurring with headaches, meningeal symptoms (hemorrhages in the membranes of the brain), epileptiform seizures (resembling seizures in epilepsy).

An increase in temperature (initially up to 38-39 ° C, then subfebrial, i.e. below 38 ° C), a small and unstable initial leukocytosis, an increase in ESR, an increase in the content of globulins in the serum, hyperfibrinogenemia (increased fibrinogen content in the blood plasma) are often observed. . Anemia develops due to blood loss.

Diagnostics

The diagnosis of hemorrhagic vasculitis is made on the basis of clinical data, and it does not require additional studies for confirmation. In the analysis of peripheral blood, leukocytosis, increased acceleration of ESR, neutrophilia (increased number of neutrophil leukocytes), eosinophilia (increased number of eosinophils), thrombocytosis (increased number of platelets) are detected to varying degrees of severity. Given the frequent kidney damage, all patients need to undergo systematic urine tests. If there are changes in the urine, studies are performed to assess the functional state of the kidneys. Due to the fact that 1/3 of patients may have DIC, it is advisable to regularly count the number of platelets, and during the height of the disease to study the state of the patient’s hemostasis (venous blood clotting time, heparin resistance, fibrinogen and fibrin levels in the blood).

Great difficulties are caused by timely diagnosis of complications of abdominal syndrome - appendicitis, intussusception, intestinal perforation and peritonitis. Such children require joint monitoring by a pediatrician and a pediatric surgeon over time.

A prerequisite for therapy is hospitalization and bed rest for at least 3 weeks, then it is gradually expanded, since exacerbations of purpura are possible, explained as orthostatic purpura.

Cooling and additional allergization of patients with food products and medications should be avoided in every possible way. Cocoa, coffee, chocolate, citrus fruits, fresh berries (strawberries, strawberries) and dishes made from them, as well as individually intolerant types of food are excluded from the diet.

The use of antibiotics, sulfonamides and other allergenic drugs (including all vitamins) that can maintain hemorrhagic vasculitis or contribute to its exacerbation should be avoided. Low-allergenic antibiotics (ceporin, rifampicin) are prescribed only for background or concomitant acute infectious diseases (for example, lobar pneumonia). Joint syndrome, increased body temperature, leukocytosis and increased ESR are not an indication for the prescription of antibiotics and other antibacterial drugs, since they are characterized by immune aseptic inflammation.

All patients with hemorrhagic vasculitis are recommended to be prescribed enterosorbents, such as activated carbon, cholestyramine or polyphepane orally. In addition, gastric drops, antiallergic drugs (antihistamines), calcium pantothenate, rutin, medium doses of ascorbic acid are prescribed, and herbal medicine is also used. With all this, the effectiveness of the above drugs in the treatment of this pathology remains very doubtful.

Patients develop abdominal pain that does not go away while taking gastric drops, and resort to using drugs with an analgesic effect, such as no-spa, baralgin.

The use of antiplatelet agents, such as chimes and pentoxifylline (trental), is considered justified. The duration of treatment is 3 months. For moderate hemorrhagic vasculitis, it is recommended to use 2 antiplatelet agents, and for the chronic course of the pathology in question, add Plaquinil (Delagil) to the therapy. The duration of such therapy can last up to 1 year. It is also recommended to prescribe drugs with a membrane-stabilizing effect (vitamins A, E, dimephosphone).

High activity of the process with pronounced abdominal, skin and joint syndrome is an indication for prescribing a combination of the following drugs: prednisolone and heparin. Isolated administration of prednisolone is dangerous, since it increases blood clotting, and there is always a tendency to develop disseminated intravascular coagulation syndrome in this disease (even if there are no clear signs of its presence). Prednisolone is usually prescribed at a dose of 1 mg/kg, and heparin - 200-300 units/kg per day, divided into 4-6 injections, under the skin of the abdomen. If, during heparin therapy, the clotting time of venous blood continues to remain shortened (less than 8 minutes), then the dose can be increased by 1.5 times. Heparin should not be administered 2 or 3 times a day, as this provokes the development of intravascular blood clots. Heparin withdrawal should be gradual, but by reducing the dose rather than reducing the number of injections. Sometimes, with a stormy clinical picture, it is necessary to resort to infusion therapy, and in this case it is possible to achieve optimal administration of heparin - intravenous drip with its uniform intake into the body throughout the day.

In severe cases, in addition to heparin therapy and glucocorticoids, 5-8 sessions of plasmapheresis are prescribed. The first three sessions of plasmapheresis are carried out daily, the subsequent ones – once every 3 days. Fresh frozen plasma, albumin and glucose solutions are used as replacement drugs.

A combination of pulse therapy with prednisolone (15-20 mg/kg/day for 3 days) and plasmapheresis is possible.

In patients with subacute nephritis or with a rapid course of glomerulonephritis, they resort to the combined administration of immunosuppressants (azathioprine or cyclophosphamide) with glucocorticoids and heparin, antiplatelet agents (Curantil). Cytostatics should not be prescribed only in connection with a protracted or undulating course of the disease. Such patients are recommended to undergo examination for the presence of helminths and foci of infection, i.e., to look for the cause.

With proper treatment, abdominal syndrome is usually eliminated most quickly, the intensity of which often decreases within a few hours after intravenous administration heparin. The simplest (cutaneous-articular) variants of vasculitis occur most persistently. The periodic appearance of a small number of rash elements on the legs and feet without other symptoms often cannot be treated at all. Sometimes they are treated with local applications. These rashes are harmless and go away spontaneously after a while.

There is evidence of repeated occurrence of exacerbation of hemorrhagic vasculitis in connection with psycho-emotional stress, hysterical background, and stressful situations. The patient is provided with psychological rest and, if necessary, recommended sedatives and tranquilizers, which increases the effectiveness of complex therapy.

Forecast

It is believed that 60% of patients with hemorrhagic vasculitis recover within a month, and 95% within a year.

Chronic nephritis develops in 1-2% of patients suffering from this pathology. Mortality in hemorrhagic vasculitis is about 3% and even less due to forms with organ complications and cases of chronic nephritis.

Dispensary observation

If there is no kidney damage, children are monitored by a local pediatrician for 5 years. Every six months, the child is shown to a dentist or otolaryngologist for timely diagnosis and treatment of the most common foci of infection. Feces are also regularly examined for helminth eggs. At least once a quarter and after each acute respiratory infection, urine tests are done. Medical exemption from vaccinations is given for 2 years. Planned therapy is not indicated

Prevention

Prevention of exacerbations plays an important role in preventing exacerbations of the disease. chronic infection, refusal to take antibiotics and other drugs without compelling indications (taking tetracyclines and chloramphenicol is especially undesirable), avoiding contact with allergens. Vaccinations and tests with bacterial antigens (for example, tuberculin) are contraindicated for patients, since they often cause severe relapses of the disease. Relapses can also be provoked by cooling, physical activity, eating disorders, and alcohol.

Description

Henoch-Schönlein disease or hemorrhagic vasculitis is a disease characterized by inflammation of the walls of blood vessels.

This type of vasculitis is the most common. It is based on aseptic inflammatory processes, which are localized in the walls of microvessels, and microscopic thrombus formation, of a multiple nature, which affects the skin vessels and vessels of internal organs. Hemorrhagic vasculitis is caused by an activated component of the complement system, which circulates in the human blood.

In medicine, there is another name for hemorrhagic vasculitis - Henoch-Schönlein purpura. You can be affected by this disease at any age. But this disease is extremely rare in children under three years of age. The most common age group for patients with hemorrhagic vasculitis is between four and twelve years of age.

The onset of the disease is usually recorded three to four weeks after suffering a sore throat, acute respiratory viral disease, scarlet fever, and other infectious diseases. Most often, the precursor to the onset of vasculitis is vaccination, trauma, food allergy, hypothermia or drug intolerance.

Hemorrhagic vasculitis refers to autoimmune diseases. Its onset is characterized by the production of a large number of immune complexes, which damages the walls of blood vessels and capillaries and increases their permeability.

There are several types of hemorrhagic vasculitis: fulminant, acute, subacute, protracted and chronic. There is also a classification, depending on the location of the lesion: cutaneous and cutaneous-articular form of hemorrhagic vasculitis, abdominal and cutaneous-abdominal, renal and cutaneous-renal, mixed form.

Symptoms

Diagnosis of Henoch-Schönlein disease is based on the patient’s complaints and characteristic symptoms. Because the symptoms of this disease are not specific, then suspicion of vasculitis of this type may occur when two or more symptoms are present, for example, a characteristic rash and abdominal pain. Also, when diagnosing this type of vasculitis, the age of the patient is taken into account.

Diagnostics

Henoch-Schönlein disease Diagnosis of hemorrhagic vasculitis is based on the patient's complaints and characteristic symptoms. Since the symptoms of this disease are not specific, suspicion of this type of vasculitis may arise if two or more symptoms are present, for example, a characteristic rash and abdominal pain. Also, when diagnosing this type of vasculitis, the age of the patient is taken into account.

To confirm the diagnosis, laboratory tests are performed: general analysis blood, endoscopic examination.

Differential diagnosis is carried out to separate hemorrhagic vasculitis from thrombocytopenic purpura, cryoglobulinemia and serum sickness.

Prevention

Prevention of hemorrhagic vasculitis is timely detection And competent treatment infectious diseases, strengthening the human immune system, hardening the body, eliminating all kinds of allergens and avoiding contact with them, taking any medicines must be carried out under the supervision of a physician.

Correct healthy eating and a daily routine, that is, a healthy lifestyle will help avoid not only hemorrhagic vasculitis, but also many infectious diseases.

Treatment

Henoch-Schönlein disease is treated with a whole complex medical events and methods.

First of all, a certain diet is prescribed: all allergenic foods are excluded from the diet (citrus fruits, coffee, cocoa, chocolate, red berries, and other foods for which the patient has an appetite). individual intolerance). Then bed rest is prescribed. TO drug treatment Hemorrhagic vasculitis refers to the use of substances that prevent blood clotting. Fibrinolysis activators are sometimes prescribed.

Treatment of internal organs is prescribed according to indications. Anti-inflammatory drugs are usually used steroid hormones and immunosuppressants.

In the most severe cases of the disease, cytostatic or immunosuppressive therapy methods are used.

Children suffering from hemorrhagic vasculitis must be registered at the dispensary. During this period, they are not allowed to stay in the sun, engage in active sports, or do physical therapy.

Henoch-Schönlein disease

Henoch-Schönlein disease is a systemic vasculitis that affects capillaries, post-capillary venules and arterioles of all areas of the body, including the lungs, brain and brain membranes. The disease is characterized by the deposition of immune deposits on the walls of microcirculatory vessels, consisting mainly of immunoglobulins A. This disease is also called hemorrhagic vasculitis.

Who is susceptible to the disease?

Hemorrhagic vasculitis can affect people from 5 months to old age. But Henoch-Schönlein disease most often occurs in children aged 4 to 6 years - in 70 out of 100,000.

As for gender, no peculiarities were noticed; the disease affects both men and women equally. However, the older a person gets, the less likely it is to develop the disease. The contingent of adult patients is usually represented by persons under 30 years of age. Hemorrhagic vasculitis is very rare in people over 60 years of age.

The lowest number of cases of the disease is observed in the summer. During other times of the year, the number of patients increases significantly. It is important to note that very often, before developing hemorrhagic vasculitis, children suffered an acute respiratory infection, which confirms its infectious nature.

Hemorrhagic vasculitis in children - anaphylactic purpura of Henoch-Schönlein. Infectious diseases

Systemic inflammatory disease microvasculature vessels (capillaries, pre- and post-capillaries) due to hypersensitivity reaction immediate type. Mostly boys are affected preschool age. The disease often begins 3 weeks after a respiratory tract infection. History of frequent scarlet fever, vaccination, food allergy. The first symptoms are skin rashes in the form of a polymorphic exanthema with hemorrhages, localized mainly on the outer surface lower limbs, less often on upper limbs and face.

Marked swelling of the feet and hands. swelling of the eyelids and face, arthralgia with swelling of the joints (purpura rheumatica), hematomesis, abdominal pain (purpura abdominalis), symptoms of intussusception, micro- and macrohematuria. The duration of the disease is on average 3-4 weeks, but sometimes several months (1-2 years). Etiology and pathogenesis have not been established. Sensitization is important with the development of an allergic reaction in the microvasculature and subsequent hemorrhages.

Attached the importance of increasing the level of IgA in the blood of patients, as well as intravascular coagulation, especially in the pathogenesis of renal damage [Serov V.V. et al. 1978; KincaicbSmith P. 1975]. Vasculitis and hemorrhages are found in the area of ​​skin rashes.

Massive hemorrhages into the intestinal wall can lead to intussusception, ulceration with perforation and the development of fecal peritonitis. However, this is rarely observed. More often there is kidney damage in the form of focal or diffuse mesangio-proliferative glomerulonephritis. Characteristics of glomerulonephritis in Shenliya-Henoch vasculitis are microthrombi in the capillaries of the glomeruli and infiltration of the glomeruli with leukocytes. In the mesangium, subendothelial deposits of IgA, M, complement and fibrin are detected. The outcome may be fibroplastic changes with the development of nephrosclerosis.

Infectious diseases

Infectious diseases are diseases that arise as a result of interaction pathogenic microorganisms and macroorganisms, characterized by high contagiousness, the presence of an incubation period, a cyclical course and the formation of post-infectious immunity.

Very big contribution Soviet pathologists A. I. Abrikosov, I. V. Davydovsky, M. A. Skvortsov, A. P. Avtsyn, B. N. Mogilnitsky, A. I. Strunoy, Ya. L. contributed to the study of the patho- and morphogenesis of infectious diseases Rapoport et al.

All pathology of infectious diseases in the middle of the last and at the beginning of the present century it was studied from the standpoint of the etiological principle. Since the time of I.P. Pavlov, Soviet pathology began to pay attention to the study of not only etiology, but also pathogenesis, i.e. mechanism of disease processes.

Throughout their lives, the highest organisms contact with microorganisms, however, the infectious process can only be caused by pathogenic microbes, which make up negligible a small part representatives of the microbial world of the planet [Pokrovsky V.I. et al. 1979].

When a pathogen enters microflora an infectious process develops in the body, which can be in the form infectious disease, manifested by clinical signs or asymptomatic, as well as in the form of carriage (bacterio-, virus- and mycoplasma carriage), in which the interaction of the organism and the microbe is not externally manifested, but is accompanied by hidden adaptive and protective reactions, including often the accumulation of specific antibodies.

Both of these forms infectious process can transform one into another. What differs from an asymptomatic infection is a latent infection, a hidden infection characteristic of chronic diseases(malaria, tuberculosis), in which phases clinical manifestations illnesses alternate with remissions.

This disease primarily affects the microvasculature and is considered as a generalized microthrombovasculitis. A family predisposition to HLA antigens B8, Bw35, A1, A2, A10, as well as a connection with C7 complement deficiency and with the development of nephritis in hemorrhagic vasculitis (HV) with deficiency of the C3 fraction of complement. In this article we will look at Henoch Schönlein disease - symptoms and treatment, diagnosis, photo of the disease.

Causes

Henoch's Schonlein disease occurs in children 2 to 10 years old. Etiology unknown. There is a number of indirect evidence that the cause of the disease is a hyperergic immune response to a viral or bacterial infection. Symptoms of the disease appear mainly in the spring. In many patients after an upper respiratory tract infection (respiratory viruses, group A beta-hemolytic streptococcus, mycoplasmas). In children with the abdominal and articular form, positive results were obtained by detecting antibodies to Bartonella henselae using indirect immunofluorescence at a titer of 1:64. A provoking role may be played by food allergens, insect bites, vaccines, immunoglobulins. Treatment of Henoch's Schonlein disease should be started promptly.

Pathogens. The pathogenesis is based on immune complex inflammation. Hyperproduction of IgA and CEC containing IgA is detected. It is believed that in the initial period of breastfeeding, in response to antigenic stimulation of the mucous membranes by exogenous and endogenous antigens, the regulatory mechanisms of IgA synthesis are disrupted, followed by the formation of IgA-containing immune complexes. These complexes subsequently settle in the mesangium and walls of blood vessels, causing exudative and proliferative changes [Panchenko E. L., 1999].

The main target for hepatitis B is endothelial cells of the vascular bed. The presence of IgA deposits in skin biopsies of patients distinguishes the disease from other types of systemic vasculitis. When damaged, the vascular endothelial cell is capable of synthesizing cytokines as well as adhesion molecules. In patients with hepatitis B, hyperproduction of proinflammatory cytokines and tumor necrotizing factor (TNF-alpha), as well as the ability to synthesize a large number of IL-2, IL-4, IL-6. During the height of hepatitis B, platelet aggregation and adhesion are increased, and the content of antithrombin III and plasminogen in the blood serum is reduced. Fibrin degradation products are found in the urine, that is, signs of disseminated intravascular coagulation appear, and hepatitis B is characterized by a phase of prolonged hypercoagulation. At the same time, traditional coagulogram parameters do not reveal violations. In sick children, there is an increase in the level of von Willebrand factor in the blood.

An essential link in the pathogenesis is an increase in vascular permeability under the influence of the resulting histamine, serotonin, and thromboxanes. An increase in the permeability of the vascular wall affected by hepatitis B causes the development of hemorrhagic syndrome and allergic edema.

Henoch's Schonlein disease primarily affects terminal blood vessels: arterioles, capillaries, venules. The disease is characterized primarily by damage to the microvessels of the skin, joints, mesentery, intestinal mucosa, and renal vessels. Thrombosis is also observed in the disease lymphatic vessels, which is accompanied by an increase in their permeability, cessation of resorption of protein and fluid from tissues and the development of acute edema.

Symptoms

The following clinical forms are distinguished:

• cutaneous (simple),
• articular,
• abdominal syndrome,
• renal form,
• lightning purpura.

In children, a combination of 2 - 3 forms is observed - a mixed form of hemorrhagic vasculitis.

Simple (skin) form of the disease

The simple (cutaneous) form of Henoch's Schönlein disease is manifested by the following symptoms: a papular-hemorrhagic rash, symmetrical on the extensor surfaces of the feet, legs, thighs, buttocks, extensor surfaces of the forearms, shoulders and around the affected joints. The rash is palpable upon palpation, does not disappear with pressure, and punctate necrosis is noted in the center. Peeling is not typical; after the rash subsides there may be pigmentation. The following symptoms are characteristic of hepatitis B: repeated waves of rashes, so both old and fresh elements can be present on the skin at the same time. Along with the rash, patients may experience angioedema - in the facial area (eyelids, lips).

Articular form of the disease

The following symptoms of Henoch's Schönlein disease are characteristic: pain in the joints, swelling of them, including the periarticular tissues. Affected predominantly large joints, most often knee, ankle, exudative component is pronounced. Arthritis can last from several hours to 1-2 days. Sometimes there may be relapses of articular syndrome. It is atypical for hepatitis B to involve small joints and spinal joints. Unlike rheumatoid arthritis, persistent deformities and contractures are not observed. There may be changes in the heart in the form of muted tones due to impaired myocardial metabolism. Unlike rheumatism, these changes do not increase.

Abdominal disease syndrome

This is a lesion of terminal vessels of various parts gastrointestinal tract. There is perivascular edema of the intestinal tissue, “diapedetic” and thrombohemorrhagic hemorrhages in the intestinal wall, mesentery, and peritoneum. Characteristic symptoms: abdominal pain according to type intestinal colic. There may be vomiting, often repeated with blood, or hidden blood in the stool. The abdomen is swollen and participates in the act of breathing. Localization pain syndrome vague, migrating pain in the navel, right iliac region, in the right hypochondrium, sometimes complaints of pain throughout the abdomen. The abdomen is soft on palpation. May be dynamic obstruction intestines due to swelling of the intestinal wall and thickening, followed by narrowing of the intestinal lumen caused by hemorrhages. Malnutrition of the intestinal wall leads to the formation of necrosis, with bleeding and sometimes intestinal perforation.

Renal form of the disease

More often, a transient urinary syndrome is observed in the form of hematuria (3-7 red blood cells in the field of view when examining urine sediment) and slight proteinuria (from traces to 0.165 - 0.33%, less than 0.3 - 0.5 g/l) due to increased vascular permeability and is detected more often from the first days of the disease. In patients, transient urinary syndrome has a wave-like character, according to the severity of the process. One of the manifestations is glomerulonephritis, which is observed with abdominal syndrome and combined damage to the skin and joints. A predominantly nephritic form is noted, developing after 2-3 weeks. from the beginning of the war. The clinic shows increasing, prolonged and persistent urinary syndrome, pallor skin, increase blood pressure(often short-term), increasing decrease in speed glomerular filtration, hematuria, moderate proteinuria [Sergeeva K. M., 1968], without clinically significant symptoms.

In such cases, dynamic nephroscintigraphy with technetium-99, excreted by glomerular filtration, allows us to identify those changes with which Henoch-Schönlein disease occurs that were not detected by ultrasound. The method allows one to judge the filtration function of the kidneys, local, segmental and diffuse changes in the kidneys [Petrov V. Yu. et al., 2002]. Hematuria is caused by changes in the kidneys themselves, and not just by the pathology of hemostasis.

After 3 - 5 years, symptoms of chronic GN are diagnosed, kidney damage progresses with the development of chronic renal failure. Nephrotic syndrome occurs less frequently with hepatitis B. Morphological changes in the kidneys during hepatitis B - from focal proliferative GN to the formation of extracapillary crescents. Electron microscopy reveals mesangial, subendothelial deposits similar to those in Berger's disease. In adults, it has even been proposed to consider the symptoms of Berger’s disease as a monosyndromic variant of hepatitis B.

Purpura fulminans in Henoch's Schonlein disease

It is observed as a hyperergic reaction of Arthus or Shvartsman with the development of diffuse intravascular coagulation and acute necrotizing thrombusculitis. It is observed mainly in children under 1 year of age and in the first years of life. In 1-4 weeks. after infectious disease(scarlet fever, rubella, chicken pox, streptococcal infection) sudden appearance of extensive hemorrhages, necrosis and small cyanotic areas of skin on the hands, feet, ankles, knees and elbow joints, on the buttocks, stomach, face. Within hours, the lesions merge into vast fields of necrosis and gangrene of the hands and feet. The clinic develops symptoms of acute renal failure. At laboratory research hyperfibrinogenemia, deficiency of blood coagulation factors V, VII, X, increased prothrombin time. From the blood side: anemia, hyperleukocytosis, increased ESR.

Changes in the hemostatic system:

• Impaired microcirculation, changes in the rheological properties of blood.
• Increasing the adhesive and aggregation function of platelets.
• Decreased plasminogen and antithrombin III.
• Increased von Willebrand factor levels.
• Products of fibrin degradation in urine.

Diagnosis of Henoch's Schonlein disease

American College of Rheumatology diagnostic criteria:

• Palpable purpura in the absence of thrombocytopenia.
• Under 18 years of age at onset of illness.
• Stomach ache.
• Granulocytic infiltration of the walls of arteries and veins during biopsy.

Features of the course of the disease:

• severity of exudative manifestations, hyperergic component vascular reactions,
• tendency to generalize the process,
• delimited angioedema,
• more often abdominal syndrome,
• often acute onset and course of the disease,
• often mixed form - persistence of several syndromes,
• tendency to relapse.

Causes of relapses: repeated infections, unsanitized foci of chronic infection, violations of diet, bed rest, emotional stress, fear, worries about the disease.

Treatment

To cure the disease, it is indicated bed rest to improve blood circulation and microcirculation until disappearance pain symptoms, hemorrhagic rash. Gradual expansion motor activity. Aeration of premises.

The diet is hypoallergenic with the exclusion of obligate and causally significant allergens. For severe abdominal pain, dyspeptic disorders, intestinal bleeding for several days of treatment, antiulcer table No. 1 according to Pevzner is recommended, followed by transfer to table No. 5 (hepatic). For nephritic syndrome, table No. 7 is a salt-free diet with protein restriction for 7-10 days.

Drug therapy

Basic - antiplatelet agents: curantil (35 mg/kg/day), trental (50-100 mg 2-3 times a day), agapurin (0.05-0.1 g), ticlid, ticlopedin (0.25 2 - 3 times a day). In severe cases, antiplatelet agents are administered intravenously. If necessary, anticoagulants are prescribed for treatment: heparin at an initial dose of 250-300 IU/kg per day, subcutaneously 3 times in equal doses, or low molecular weight heparin– fraxiparine, calciparine 5000-7500 units 2 times a day subcutaneously.

Treatment of Henoch's Schonlein disease with corticosteroids

Corticosteroids - indications for treatment: children with abdominal syndrome, with continuously recurrent skin form with elements of necrosis, patients with a high protein content acute phase. The dose of prednisolone is 0.5-1 mg/kg/day for a short course (7-10 days) for up to 2 weeks. Prednisolone is prescribed against the background of heparin.

In the hematuric form of glomerulonephritis, corticosteroids are not prescribed, because They have virtually no effect! For nephritis with nephrotic syndrome Immunosuppressants are prescribed, most often azathioprine 2 mg/kg/day for 6 weeks, then a maintenance dose of 1 mg/kg/day for 6 months under the control of peripheral blood tests. There have been reports of favorable results of intravenous immunoglobulin for the treatment of nephritis in hepatitis B.

Antithrombotic therapy

In severe cases of Henoch's Schönlein disease with pregangrene and necrosis. A combination of plasmapheresis, pulse therapy with methylprednisolone (10-20 mg/kg) and (or) cyclophosphamide (8 mg/g). Daily for 3 days, then 2 times a week for 3-4 weeks. If necessary, combine with constant round-the-clock administration of heparin until abdominal syndrome and severe hematuria are eliminated.

Treatment with membrane stabilizers

Membrane stabilizers: retinol (1 - 1.5 mg), dimephosphone - 50-75 mg/day for 10-14 days, vitamin E 1-1.5 mg for a month as an antioxidant.

In severe cases and when the disease occurs in the renal form, plasmapheresis is used, as well as in the presence of cryoglobulins in the blood and in persistently recurrent forms - from 3 to 6-7 procedures.

Enterosorption in Henoch's Schonlein disease

Enterosorption – biological binding active substances and toxins in the intestines. Use polyphepan 1 g/kg per day in 1-2 doses, thioverol 1 teaspoon 2 times a day. Course of treatment for acute course is 2-4 weeks, with recurrent wave-like from 1 to 3 months.

Foci of infection are being sanitized.

Dispensary observation

Clinical observation for symptoms of Henoch-Schönlein disease by a pediatrician, family doctor, dentist, or ENT specialist for at least two years after the last relapse (in the absence of kidney damage). Once every 3 months. in the first year and once every 6 months. on the second – consultation with an ENT specialist and a dentist. Withdrawal from vaccinations, administration of γ,-globulin, Mantoux test for 2 years. Hyposensitizing diet with the exclusion of obligate allergens, seeds, nuts, pomegranates, Pepsi-Cola and other “fizzy drinks”, canned juices and foods, chewing gum, etc. It is not recommended to travel to regions with hot climates, sunbathe and swim. Patients with kidney damage are under the supervision of a pediatrician and a district nephrologist.

Treatment prognosis. In 70% it is favorable. Worsens the prognosis renal syndrome– jade, receiving chronic course with the development of chronic renal failure.

Now you know the main causes, symptoms and methods of treating Henoch Schönlein disease in a child. Health to your children!

Henoch-Schönlein disease in children and adults is a pathology that is accompanied by damage to the vascular walls. This is one of the types of vasculitis, which is the most popular and occurs more often in childhood.

It is expressed by inflammatory processes that occur in the vessels of the skin and internal organs. The disease is caused by an activated component located in human body.

You can get this disease regardless of age, but it develops extremely rarely in children under 3 years of age.

According to statistics, the disease makes itself felt from 4 years of age to 12 years of age.

The initial stage of the disease, as a rule, occurs in the first 3-4 weeks after an infectious disease, viral pathologies etc. (angina, scarlet fever). The trigger for the onset of the disease is trauma, drug intolerance, allergic reaction And so on.

Henoch-Schönlein disease

The pathology is autoimmune. At the beginning of its development, many immune reactions accompanied by damage to the walls of blood vessels.

Why does it occur

The culprit for the development of the disease is a previous infection, as well as vaccines and various serums that give an inadequate response.

In this case, circulating immune complexes appear, settling in the vessels and provoking their damage. As mentioned above, various bacterial or viral infections, and helminthic infestations, unfavourable conditions environment.

Varieties

If we talk about the clinical picture, it suggests division of the disease into acute and chronic course. According to symptoms, Henoch-Schönlein disease is classified into:

• simple;
• articular;
• with abdominal syndrome;
• lightning-fast form.

According to the stage of severity, they are distinguished:

• mild degree, in which the patient’s health is satisfactory and there are not pronounced rashes;
• moderate severity is manifested by a profuse rash, pathological changes in the joints, moderate abdominal pain;
• a severe form of the disease is accompanied by severe clinical course, extensive lesions with necrotic areas, kidney damage, neurotic edema, as in the photo, in patients with abdominal syndrome is manifested by bleeding in the gastrointestinal tract.

Photo Henoch-Schönlein disease

If we talk about acute form disease, then it cannot last longer than two months, the protracted stage lasts up to 6 months and the chronic form lasts longer than six months.

Clinical manifestations of the disease

Patients with hemorrhagic vasculitis have a high fever. But an increase in temperature is not always observed.

At the beginning of the development of the disease, the so-called skin syndrome makes itself felt. It is typical for absolutely all patients. Spotty and papular formations appear on the skin different sizes, which disappear if you press on them. The rash is often symmetrical and appears in the thighs, buttocks and lower extremities. Less commonly, in severe cases, tissue necrosis occurs.

Also in some patients articular syndrome is observed.Symptoms may be short-lived or last for days and be accompanied by intense pain, which can lead to limited mobility of the joints.

Usually the damage spreads to large joints; in some cases, the knees and ankles may be affected. This syndrome can develop initial stage vasculitis. As a rule, its character is unpredictable, but it does not lead to deformation.

All of the above symptoms are accompanied by abdominal syndrome. Patients complain about severe pain in the abdominal area. It is difficult for patients to answer the exact question about the localization of pain; they also notice a violation of bowel movements, they feel sick and vomit.

The pain syndrome occurs spontaneously and stops in the same way. IN advanced cases intestinal obstruction develops.

Patients with pathology complain of severe pain in the abdominal area

This disease leads to another serious lesion - nephrotic syndrome, which is the most persistent and is fraught with renal failure and can lead to a disease such as chronic nephritis.

As for other organs, they are affected very rarely. Myocarditis, pericarditis, pneumonia, etc. occur.

Damage to the brain and its membranes makes itself felt by confusion, apathy, fatigue, nervousness, and headaches. The symptom is very serious and can lead to the development of meningitis.

How to make a diagnosis

First of all, you need to get an appointment with a doctor. The specialist pays attention to age and general condition, weighs the results of studies and the patient’s stories about symptoms, and excludes other diseases.

Blood tests usually show nonspecific findings aseptic inflammation, eosinophils and platelets will definitely be elevated. It is also necessary to donate urine and blood for biochemistry.

If Henoch-Schönlein disease is suspected, a coagulogram is required.

In case of kidney damage, the patient must consult a nephrologist. The specialist will tell you what tests you need to take.

Treatment methods

The disease is treated with a whole range of measures. Official medicine suggests starting treatment by changing eating habits. All allergenic products are completely excluded. After this, the patient needs to stay in bed. Pharmaceutical treatment involves taking medications that prevent blood from clotting.

Therapy of organs and systems is required according to clinical signs in each individual order.

As a rule, anti-inflammatory drugs, hormonal agents and medications that strengthen the immune system are prescribed.

It is important to register children. During treatment, they should not be in the sun, engage in active sports or undergo physical therapy.

Prevention measures

With timely and effective therapy the disease can be successfully treated

To prevent the disease, it is important to recognize it in a timely manner and carry out effective treatment. Important has hardening and strengthening of the immune system. You also need to regularly sanitize your home, install air purifiers and humidifiers, and ventilate the room to get rid of the accumulation of bacteria and dirt. Children need to be strengthened from childhood. The use of any medications should be discussed with a specialist.

A patient with this diagnosis must have proper nutrition and established daily routine. If you follow the principles healthy image life, then you will protect yourself not only from vasculitis, but also from many serious pathologies.

Forecast

Mild forms of the disease can be successfully treated. However, the fulminant course can be fatal in the first days after the development of the disease, which is explained by damage to blood vessels and the appearance of hemorrhage in the brain. Severe renal syndrome may also be among the causes of death.

Conclusion

Hemorrhagic vasculitis is serious illness which requires the patient immediate reaction. It is important to apply on time medical care, otherwise unexpected consequences may occur. At correct actions the disease is successfully treated and recedes, after which the person recovers and returns to a full life.

Henoch-Schönlein purpura- vasculitis with IgA-immune deposits, affecting small vessels (capillaries, venules, arterioles).

Typical changes in the skin, intestines and kidneys in combination with arthralgia or arthritis.

Henoch-Schönlein purpura is one of the most common systemic vasculitis [V.A. Nasonova, 1959; I.Szer, 1994].

The disease can begin at any age, but it mainly affects children under 16 years of age. The incidence of hemorrhagic vasculitis in them is 13.5 per 100 thousand children. In early childhood, there is a slight predominance of sick boys over girls (ratio 2:1), which is leveled out in adolescents (1:1).

Etiological factors

There is a definite connection between the development of vasculitis and upper respiratory tract infection, which precedes the onset of the disease in 66-80% of patients [N.P. Shilkina et al., 1990; I. Szer, 1994].

The role of various microorganisms has been discussed in the etiology of hemorrhagic vasculitis, including streptococci, mycoplasma, yersinia, legionella, Epstein-Barr and hepatitis B viruses, adenovirus, cytomegalovirus and parvovirus B19. The manifestation of the disease after vaccination against typhus, paratyphoid A and B, measles, and yellow fever is described.

Other potential “trigger” agents are medications (penicillin, ampicillin, erythromycin, quinidine, etc.), food allergies, insect bites, hypothermia. There are reports of the development of hemorrhagic vasculitis while taking enalapril, lisinopril and aminazine. Sometimes the disease complicates the course of pregnancy, periodic illness, malignant neoplasms, liver cirrhosis and diabetic nephropathy.

Immune disorders

High level of IgA in blood serum - characteristic feature hemorrhagic vasculitis. It is believed that it is based on an increase in the number of IgA-secreting cells carrying the gene for the secretory a-chain of the IgA molecule.

The formation of polymeric forms of IgA and monomeric IgA-containing is considered as an important component of the pathogenesis of the disease and IgA nephropathy. circulating immune complexes (CEC). An increase in the concentration of the latter in the blood serum is very characteristic of these diseases.

With hemorrhagic vasculitis, there is an increase in the level of IgA of the 1st subclass, which has RF activity, which contains mainly k-light chains. In addition, complexes consisting of IgA and fibronectin are found in the sera of patients with IgA nephropathy and Henoch-Schönlein disease.

The role of IR-mediated complement activation in the immunopathogenesis of hemorrhagic vasculitis is evidenced by the detection of the membrane attack complex (Cob-C9) in the affected areas of the skin and kidney vessels. Detected in the plasma of patients high level anaphylotoxins (C4a and C3) and C5b-9, which correlates with the inflammatory activity of the disease.

There is evidence of a relationship between the development of glomerulonephritis in hemorrhagic vasculitis and deficiency of the C4b component of the complement system, which plays an important role in the solubilization and clearance of immune complexes from the bloodstream.

Results related to the study antineutrophil cytoplasmic antibodies(ANCA) with Henoch-Schönlein hemorrhagic vasculitis are contradictory. Some authors found IgA ANCA in this disease and IgA nephropathy, others did not.

According to our data, p-ANCA occur in 21.4% of patients and their presence in the blood serum correlates with the clinical activity of vasculitis, damage to the lungs, kidneys and gastrointestinal tract [A.A. Baranov, 1998].

Features of hemorrhagic vasculitis in children and adults

According to R. Blanco et al. (1997), who observed 116 patients with hemorrhagic vasculitis from 1975 to 1994, there are certain differences in the clinical picture of the disease in children and adults (Table 11.1).

Table 11.1. Comparative characteristics(%) between children (

Sign	J.Mills et al.(1990)		R. Bianco et al. (1997)
	Children	Adults	Children	Adults
Taking medications at the onset of illness	7	46*	15	15
Stomach ache	61*	29	64	57
Melena	6	33*	4	24*
Creatinine >1.5 mg/%	0	21*	0	13*
Hematuria	47	74*	22	78*
Palpable purpura	85	96	98	93
Oligoarthritis	39	39	18	24
Leukocytosis			34,5	21,7
Anemia			6,0	4,3
Increase in ESR			40,5	76,1*
Increased IgA			7,7	50*
Antinuclear factor (ANF)			0	6,8
Cryoglobulins			35,7	17,9
Decrease in SZ and/or C4			4,2	9,3

Note. * - R

In children, the onset of the disease is often preceded by an upper respiratory tract infection. In adults, abdominal syndrome and fever are less common, but damage to the joints and kidneys, as well as accelerated ESR, are more common. They are more likely to be prescribed aggressive therapy glucocorticoids (GC) and cytostatics.

Laboratory signs

Laboratory abnormalities in hemorrhagic vasculitis are nonspecific. In children, an increase in ASLO titers is observed in 30% of cases. An important sign An increase in the concentration of IgA in the blood serum allows one to suspect the disease.

In 30-40% of patients it is found rheumatoid factor (RF). Accelerated ESR, high levels of CRP and VWF:Ag in the blood serum correlate with the clinical activity index of vasculitis. An increase in spontaneous platelet aggregation is also characteristic.

Diagnosis

Until recently, hemorrhagic vasculitis was considered a subtype of hypersensitivity vasculitis. However, in 1990, an ACR subcommittee classified it and hypersensitivity vasculitis as two various diseases. The classification criteria for hemorrhagic vasculitis are summarized in Tables 11.2 and 11.3.

Table 11.2. Classification criteria for hemorrhagic vasculitis (J. Mills et al., 1990)

Note. The presence of two or more of any criteria in a patient allows a diagnosis to be made with a sensitivity of 87.1% and a specificity of 87.7%.

Table 11.3. Classification criteria for hemorrhagic vasculitis (N.P. Shilkina et al., 1994)

Note. Reliable hemorrhagic vasculitis - 7 points or more; probable - 5 - 6 points in the presence of purpura.

B. Michel et al. (1992) proposed criteria for the differential diagnosis of hypersensitivity vasculitis and hemorrhagic vasculitis (Table 11.4).

Table 11.4. Criteria for differential diagnosis of hemorrhagic vasculitis and hypersensitivity vasculitis (B. Michel et al., 1992)

Criterion	Definition
1. Palpable purpura	Slightly raised hemorrhagic skin rash not associated with thrombocytopenia
2. Abdominal pain	Diffuse abdominal pain, worse after eating, or intestinal ischemia
3. Gastrointestinal bleeding	GI bleeding, including melena, blood in the stool, or a positive stool test occult blood
4. Hematuria	Macro- or microhematuria (> 1 red blood cell per field of view)
5. Onset of the disease at age	Development of first symptoms at age
6. No treatment	Not taking medications that could cause the development of vasculitis

Note. The presence of three or more criteria in a patient in 87.1% of cases allows a correct diagnosis of hemorrhagic vasculitis. The presence of two criteria makes it possible to diagnose hypersensitivity vasculitis in 74.2% of cases.

According to S. Helander et al. (1995), detection of skin deposits of IgA can improve the diagnosis of this disease.

Treatment

If there are signs of infection, antibacterial therapy is indicated [E.N. Semenkova and O.G. Krivosheev, 1995]. Skin and joint lesions usually respond well to NSAIDs. The development of severe manifestations of the disease (abdominal syndrome, hemoptysis, etc.) dictates the need to prescribe GCs.

A prospective but uncontrolled study found that prednisolone 1 mg/kg/day for two weeks prevented subsequent renal damage. However, other authors were unable to confirm these results.

Approaches to the treatment of nephritis in hemorrhagic vasculitis are controversial. No controlled trials have been conducted yet various methods treatment of this syndrome. When determining the management tactics for patients with nephritis, it is necessary to keep in mind that many of them develop spontaneous remission of the disease.

There are reports of certain effectiveness of the use of GC therapy (including pulse therapy), cyclophosphamideA (CF), plasmapheresis, anticoagulants, disaggregants, cyclosporine A (CsA) and azathioprine [E.N. Semenkova and O.G. Krivosheev, 1995; A.V.Mazurin et al., 1996].

Forecast

Despite the fairly frequent recurrence of the disease (in 40% of cases), the overall prognosis for hemorrhagic vasculitis is favorable. The five-year survival rate of patients is almost 100%. According to R. Blanco et al. (1997), during the first two years of illness full recovery observed in 93.9% of children and 89.2% of adults.

It is believed that a history of “trigger” factors (upper respiratory tract infection or medication use) in patients with hemorrhagic vasculitis is associated with a more favorable course of the disease and a low incidence of kidney damage.

The main factor determining poor prognosis disease is persistent kidney damage, which is observed in 2-5% of patients. Arterial hypertension and significant violation renal function occurs in 44% of patients with nephritic or nephrotic variants of nephritis.

These syndromes occur only in 18% of patients with isolated or combined hematuria. Among all the causes leading to chronic hemodialysis in pediatric practice, glomerulonephritis in hemorrhagic vasculitis accounts for 3-15%.

Nasonov E.L., Baranov A.A., Shilkina N.P.