What is Hemorrhagic Vasculitis (Schonlein-Henoch Disease)?
Hemorrhagic vasculitis (HBV) is a systemic inflammatory disease primarily of the capillaries, arterioles and venules, mainly of the skin, joints, abdomen and kidneys.
Epidemiology
HB is not uncommon. In children younger than 14 years, the prevalence of HBV reaches 23-25 per 10,000 population. Boys get sick more often, but TV is found in all age groups. The peak of the annual incidence is noted in the spring.
Pathomorphology
Characterized by systemic lesion of small vessels, accompanied by hemorrhages in the skin, the sublimate membrane and subserous areas with ulcerations and perforation of the intestinal wall. The kidneys show signs of focal or diffuse glomerulonephritis.
Causes of Hemorrhagic Vasculitis (Schonlein-Henoch Disease)
As etiological (launching) in HB, many environmental factors are important. Nearly 2/3 of patients, especially children, have a connection with postponed respiratory diseases, streptococcal nc sections (angina, scarlet fever), with increased titers of anti-streptococtide antibodies. In a number of patients, the disease over time is associated with food and drug allergies. The development of HBV after vaccination, cooling, insect bites is described. The abundance of environmental factors allows you to give them the role of disease-provoking effects.
Pathogenesis during Hemorrhagic Vasculitis (Schonlein-Henoch Disease)
The pathogenesis of HB is associated with immune disorders. Thus, patients in the active phase of the disease show an increase in serum IgA and CIC containing IgA, and in the wall of blood vessels of the affected and even unaffected skin, in the kidneys also IgA and NW component of the complement, which allowed T. S. Coops and A. S Fauci is considered to be HV IgA as an immunocomplex disease. IgA immune complexes, binding to the corresponding receptors on neutrophils, activate an alternative path to the complementary cascade. Apparently, the inflammation of blood vessels, the increase in their permeability, edema and the development of purpura of various localization are associated with the deposition of IgA immune complexes. These changes in the microvasculature, according to N. P. Shilkina, with HB are accompanied by a change in blood aggregation, DIC with a number of patients with acute disease.
Symptoms of Hemorrhagic Vasculitis (Schonlein-Henoch Disease)
The disease manifests itself, as a rule, by a triad of symptoms: hemorrhagic rash on the skin (purpura), arthralgia and (or) arthritis (mainly large joints) and abdominal syndrome, which is observed in almost 2/3 of patients. With the last two symptoms, an increase in body temperature is usually observed.
The initial lesions on the skin are erythema toxic, sometimes itchy papules, which are located on the extensible surfaces of the extremities, most often on the legs, buttocks, and rarely the body. Subsequently, the papules turn into a typical purpura, passing through all stages of development up to hyper-pigmentation, which persists for a long time. In severe cases, develop areas of necrosis, covering the crusts.
The lesion of the joints is observed in more than 2/3 patients. Usually large joints are involved in the process. Patients can be disturbed only by arthralgia of varying intensity – from aches to acute pains leading to immobility, or polyarthritis due to periarthritis and synovitis. Characterized by the volatility and symmetry of the lesion.
Abdominal syndrome (abdominal purpura) usually develops in children, it is characterized by pain like intestinal colic, localized around the navel, but often in other parts of the abdomen – in the right iliac region, right hypochondrium, epigastric, simulating appendicitis, cholecystitis, pancreatitis. Simultaneous patients observed a typical picture of the abdominal syndrome – pale skin, sunken eyes, pointed facial features, dry tongue, symptoms of peritoneal irritation. Patients usually lie on their side, crouching, pressing their legs to their stomachs, or rushing about in bed. Pain in the abdomen can be unsharp, without a certain localization, and patients remember them only during questioning. Simultaneously with colic appear bloody vomiting, loose stools, often with streaks of blood. Palpation of the abdomen always increases the pain, revealing signs of peritoneal lesions. All variety of abdominal purpura can be put in the following options:
- typical intestinal colic;
- abdominal syndrome, simulating appendicitis or perforation of the intestine;
- abdominal syndrome with the development of invagination of the intestine.
Patients with abdominal syndrome should be monitored by the therapist and surgeon in order to resolve the issue of surgery if necessary.
Not so rarely (usually in patients with abdominal syndrome) the kidneys are involved in the pathological process, hematuric glomerulonephritis develops due to the defeat of the glomerular capillaries. According to the severity of the pathological process, glomerulonephritis can occur in various ways – from isolated urinary syndrome to diffuse glomerulonephritis of hypertensive or mixed type, rarely with nephrotic syndrome. Usually there is a favorable course of glomerulonephritis, possible outcomes in chronic progressive nephritis with renal insufficiency.
Clinical signs of CNS damage, hemorrhagic pneumonia, myocarditis and serositis are rarely observed and are recognized by special methods of research.
Diagnosis of Hemorrhagic Vasculitis (Schonlein-Henoch Disease)
Laboratory data
Leukocytosis, most pronounced in the abdominal syndrome with a shift in the formula to metamyelocytes, moderately unstable eosinophilia is usually observed. ESR increased, especially with abdominal syndrome and polyarthritis. More than half of the patients showed an increase in the content of immunoglobulins, primarily IgA. With kidney damage in the urine, micro and gross hematuria (less often) and proteinuria are found.
For the timely recognition of DIC syndrome, it is necessary to determine the products of fibrin degradation, fibrin monomer complexes, plasma free plate factors 4 and 3, spontaneous platelet aggregation and fragmentation of thrombin III consumption, etc. It is also important to determine the content of Willebrand factor in the plasma, reflecting the severity of the disease.
The course is usually acute and recurrent chronic forms of the disease. Acute forms are characterized by a sudden onset after infection, multi-symptom manifestations, often complicated by glomerulonephritis. In the chronic course of the disease, in 5–10% of patients, recurrent viral capillary syndrome (orthostatic purpura of the elderly) is observed.
It is easy to make a diagnosis if there is a characteristic triad of symptoms or only hemorrhagic lesions on the skin. In this case, it is necessary to take into account some peculiarities of HBV developing in children and adults. In children, the disease is much sharper – almost in 3 of children the body temperature rises, abdominal syndrome with melena is observed (almost in half of the patients), kidney damage with microhematuria and small proteinuria. In adults, abdominal syndrome occurs less frequently and is more erased due to lower intensity of pain, rarely with nausea and vomiting, extremely rarely with chalk, but somewhat more often than in children, the kidney with the development of chronic diffuse glomerulonephritis is involved in the pathological process in adults.
Differential diagnosis
The disease must be differentiated from diseases in which it develops, firstly, purpura and, secondly, abdominal syndrome with polyarrhea or polyarthritis. Hemorrhagic vaska leita syndrome can be observed with infective endocarditis, systemic vasculitis, dBST, meningococcemia, etc. Waldenstrom’s macroglobulinemic purpura should be excluded from the elderly. Abdominal syndrome can occur in cases of niosis, colitis, particularly ulcerative colitis, Crohn’s disease, and other diseases that occur with the clinical picture of an acute abdomen.
Treatment of Hemorrhagic Vasculitis (Schonlein-Henoch Disease)
In the acute period of the disease, bed rest is necessary; it is recommended to avoid cooling. Diet with the exception of sensitizing products (coffee, chocolate, citrus, cocoa, strawberries). Avoid the appointment of antibiotics, sulfonamides, vitamins, including ascorbic acid and rutin, antihistamines.
In case of abdominal syndrome, intravenous corticosteroids are prescribed in the form of 6-methylprednisolone at 80-100 mg / day and more drip or prednisolone at 150-300 mg/day or more also drip.
With the development of glomerulonephritis, combination therapy with prednisone in medium doses (up to 30 mg) and cytostatics in standard doses is recommended – 1-2 mg/kg to achieve a clinical effect. In these cases, injections of he-parin and disaggregants (trental, chimes) are recommended.
The use of heparin therapy in HB is based on coagulation disorders, especially in the acute course of the disease. However, the effectiveness of heparinotherapy is closely related to the individual selection of the drug dose, the uniformity of its action throughout the day of treatment, the correct laboratory control of the hypocoagulation effect and, if necessary, the additional administration of antithrombin III.
Heparin is prescribed in the initial dose of 300-400 IU/(kg day). For uniformity of action, it is better to administer it in equal doses every 6 hours under the skin of the abdomen, checking blood clotting before each next injection of the drug. If the initial dose of heparin is insufficient, then it is increased by 100 IU/(kg day), reaching in some cases a daily dose of 800 IU/(kg day), i.e. 40000 IU/day. With the ineffectiveness of complex therapy, including heparin therapy, as well as in general with DFS, daily for 3-4 days pour freshly frozen plasma of 300-400 ml. It is also useful to administer intravenous drip of nicotinic acid at the same time as heparin in the maximum tolerated dose, which facilitates the release of microcirculation by activating fibrinolysis.
In the chronic course of HB according to the type of orthostatic purpura, aminoquinoline preparations (delagil, plaquenil) are indicated. In a number of these patients, as well as in patients with glomerulonephritis, climatotherapy can be recommended in a dry, cool climate (southern Ukraine, the North Caucasus, the southern coast of Crimea).
Prevention consists in the treatment of foci of infection, the prevention of the influence of drug and vaccinal reactions and other sensitizing factors.