Diagnosis and treatment of hemorrhagic vasculitis in a modern hospital in Moscow

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11/01/2021 The article was checked by rheumatologist Borodin O.O. , is for general informational purposes only and does not replace specialist advice. For recommendations on diagnosis and treatment, consultation with a doctor is necessary.

At the Yauza Clinical Hospital, laboratory (coagulogram, increased levels of the enzyme antistreptolysin-O, IgA) and instrumental (biopsy) research methods are used to diagnose hemorrhagic vasculitis (Henoch-Schönlein disease, purpura).
Based on the results of the examination, as well as taking into account the clinical picture of the disease, the rheumatologist will develop an individual treatment regimen for the patient, if necessary, using innovative methods of extracorporeal hemocorrection.

140 cases per 1 million population - incidence rate of hemorrhagic vasculitis
More than 60% of cases of hepatitis in adults are accompanied by involvement of the joints, 50% of the kidneys, and 65% of the digestive tract.
In 95% of cases, timely treatment of Henoch-Schenelein disease contributes to the patient’s complete recovery

About the development of the disease

Hemorrhagic vasculitis or Schenelein-Henoch disease is a disease in which autoimmune damage to small arteries occurs - they become fragile, their permeability increases, which causes impaired microcirculation, microthrombosis, multiple small hemorrhages (hemorrhages) as in the skin (hemorrhagic rashes on the skin and mucous membranes) , and in internal organs (gastrointestinal tract, kidneys, joints).

Most often the disease develops in childhood (6-7 years), but can also affect adults. Boys get sick 1.5 times more often than girls.

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Hemorrhagic vasculitis (synonyms: Henoch-Schönlein purpura, Henoch-Schönlein disease, rheumatic purpura, allergic purpura) is the most common disease from the group of systemic vasculitis. It is based on aseptic inflammation of the walls of microvessels, multiple microthrombotic formation, affecting the vessels of the skin and internal organs (most often the kidneys and intestines).

The main reason that causes this disease is the circulation of immune complexes and activated components of the complement system in the blood. In a healthy body, immune complexes are removed from the body by special cells - cells of the phagocytic system. Excessive accumulation of circulating immune complexes under conditions of predominance of antigens or insufficient formation of antibodies leads to their deposition on the endothelium of the microvasculature with secondary activation of complement system proteins along the classical pathway and secondary changes in the vascular wall.

As a result, microthrombovasculitis develops and changes occur in the hemostatic system: activation of platelets, circulation of spontaneous aggregates in the blood, pronounced hypercoagulation, decrease in plasma antithrombin III, thrombopenia, increased level of von Willebrand factor, depression of fibrinolysis.

In 1837, the famous German physician JL Schönlein described “anaphylactic purpura.” In 1874 his compatriot E.N. Henoch published a valuable work on the same disease.

The name “ hemorrhagic vasculitis ,” used only in Russia, was introduced in 1959 by the outstanding rheumatologist V. A. Nasonova. Henoch-Schönlein purpura still prevails .

Etiology

In the majority of patients (66-80%), the development of the disease is preceded by an upper respiratory tract infection.

The manifestation of the disease after typhus, paratyphoid A and B, measles, and yellow fever is described.

Other potential triggers for the disease may include:

medications (penicillin, ampicillin, erythromycin, quinidine, enalapril, lisinopril, chlorpromazine)
food allergy
insect bites
hypothermia

Sometimes hemorrhagic vasculitis complicates the development of pregnancy, periodic illness, diabetic nephropathy, liver cirrhosis, and malignant neoplasms.

Classification

By form

cutaneous and skin-articular:
necrotic
with cold urticaria and swelling
abdominal and cutaneous-abdominal
renal and cutaneous-renal
mixed

With the flow

fulminant course (often develops in children under 5 years of age)
acute course (resolved within 1 month)
subacute (allowed up to three months)
prolonged (allowed up to six months)
chronic.

By degree of activity

I degree of activity - the condition is satisfactory, body temperature is normal or subfebrile, skin rashes are not abundant, all other manifestations are absent, ESR is increased to 20 millimeters per hour.
II degree of activity - a state of moderate severity, severe skin syndrome, body temperature rises above 38 degrees (fever), severe intoxication syndrome (headache, weakness, myalgia), severe articular syndrome, moderate abdominal and urinary syndrome. In the blood, the number of leukocytes, neutrophils, eosinophils is increased, ESR will be increased to 20-40 millimeters per hour, albumin content decreases, dysproteinemia.
III degree of activity - the condition will be severe, symptoms of intoxication are pronounced (high fever, headache, weakness, myalgia). Skin syndrome, articular, abdominal (paroxysmal abdominal pain, vomiting, mixed with blood), severe nephritic syndrome will be expressed, and there may be damage to the central nervous system and peripheral nervous system. In the blood there is a pronounced increase in leukocytes, an increase in neutrophils, an increase in ESR above 40 millimeters per hour, there may be anemia, a decrease in platelets.

Clinical picture As a rule, hemorrhagic vasculitis is benign. Usually the disease ends in spontaneous remission or complete recovery within 2-3 weeks from the moment the first skin rash appears. In some cases, the disease becomes relapsing. Severe complications due to damage to the kidneys or intestines are possible.

There are several clinical forms of hemorrhagic vasculitis:

Simple (cutaneous) form; Articular (rheumatoid) form; Abdominal form; Kidney form; Lightning form; Combined lesion (mixed form). Clinically, the disease manifests itself with one or more symptoms:

Skin lesions are the most common symptom[7] and are one of the diagnostic criteria for the disease. A characteristic hemorrhagic rash is observed - the so-called palpable purpura, the elements of which rise slightly above the surface of the skin, which is invisible to the eye, but is easily identified by touch. Often individual elements merge and can form continuous fields of significant area. Sometimes individual elements become necrotic. At the onset of the disease, the rash may be petechial in nature.

At the onset of the disease, the rashes are always localized in the distal parts of the lower extremities. Then they gradually spread to the thighs and buttocks. Very rarely the upper limbs, abdomen and back are involved in the process.

After a few days, the purpura in most cases fades, acquires a brown color due to pigmentation and then gradually disappears. With a recurrent course, areas of pigmentation may persist. There are never scars (with the exception of isolated cases with necrotization of elements and the addition of a secondary infection).

Joint syndrome - often occurs together with skin syndrome, occurs in 59-100% of cases[5]. Joint damage develops more often in adults than in children.

The favorite localization is the large joints of the lower extremities; the elbow and wrist joints are less often involved.

Characterized by migrating pain in the joints, occurring simultaneously with the appearance of skin rashes. In about a quarter of cases (especially in children), joint pain or arthritis precedes skin lesions.

A combination of articular syndrome with myalgia (muscle pain) and swelling of the lower extremities is possible.

The duration of articular syndrome rarely exceeds one week.

Abdominal syndrome, caused by damage to the gastrointestinal tract, occurs in approximately 2/3 of all patients. It manifests itself as spastic abdominal pain, nausea, vomiting, gastrointestinal bleeding (moderate, non-dangerous bleeding occurs frequently - up to 50% of cases; severe - less often, life-threatening - in no more than 5% of cases). Severe complications such as intussusception, perforation, and peritonitis are possible. An endoscopic examination reveals hemorrhagic or erosive duodenitis, less often erosion in the stomach or intestines (any localization is possible, including the rectum).

Renal syndrome: the prevalence has not been precisely established; there is a significant range of data in the literature (from 10 to 60%). More often it develops after the appearance of other signs of the disease, sometimes one to three weeks after the onset of the disease, but in isolated cases it may be its first manifestation. The severity of renal pathology, as a rule, does not correlate with the severity of other symptoms. Clinical manifestations of kidney damage are varied. Usually isolated micro- or macroglobulinuria is detected, sometimes combined with moderate proteinuria. In most cases, these changes pass without a trace, but some patients may develop glomerulonephritis[7]. Nephrotic syndrome may develop.

Morphological changes in the kidneys range from minimal to severe nephritis with crescents. Electron microscopy reveals immune deposits in the mesangium, subendothelium, subepithelium, and glomeruli of the kidneys. They include IgA, mainly the 1st and less often the 2nd subclass, IgG, IgM, C3 and fibrin.

Lung damage: occurs in isolated cases. Patients with pulmonary hemorrhage and pulmonary hemorrhages are described. Damage to the nervous system: occurs in isolated cases. Patients with the development of encephalopathy, with minor changes in mental status, are described; there may be severe headaches, seizures, cortical hemorrhages, subdural hematomas and even cerebral infarction. The development of polyneuropathy has been described. Damage to the scrotum: occurs in children, no more than 35%, and boils down to swelling of the scrotum (which is associated with hemorrhages in its vessels). Lightning form. It is based on a hyperergic reaction, the development of acute necrotizing thrombusculitis. The disease most often develops in the first or second year of life, 1-4 weeks after a childhood infection (chickenpox, rubella, scarlet fever, etc.). Characterized by symmetrical extensive hemorrhages, necrosis, and the appearance of cyanotic areas of the skin (hands, feet, buttocks, face), which are confluent in nature. In the future, gangrene of the hands and feet, coma, and shock may develop.

Features of hemorrhagic vasculitis in children:

The severity of the exudative component; Tendency to generalize; Limited angioedema; Development of abdominal syndrome; Acute onset and course of the disease; Tendency to relapsing course.

Laboratory signs Nonspecific. An important sign to suspect the disease is an increase in the concentration of IgA in the blood serum.

RF is detected in 30% - 40% of patients. In children, an increase in ASL-O titer is observed in 30% of cases. Increases in ESR and CRP correlate with the degree of vasculitis activity.

Diagnostic criteria[edit | edit wiki text] There are classification criteria for hemorrhagic vasculitis recognized by the international community of rheumatologists, which have been successfully used in diagnosis for many years (since 1990)[8].

There are four of them, each given a clear definition.

Palpable purpura. Slightly raised hemorrhagic skin changes not associated with thrombocytopenia. Age less than 20 years. The age of onset of the disease is less than 20 years. Stomach ache. Diffuse abdominal pain, worse after eating. or intestinal ischemia (there may be intestinal bleeding). Detection of granulocytes during biopsy. Histological changes revealing granulocytes in the wall of arterioles and venules. The presence of 2 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%.

Other systems of classification and differential diagnostic criteria have also been proposed [9] [10].

Treatment First, a diet is necessary (allergenic foods are excluded). Secondly, strict bed rest. Thirdly, drug therapy (antiplatelet agents, anticoagulants, corticosteroids, immunosuppressants - azathioprine, as well as antithrombotic therapy). The following drugs are used:

disaggregants - chimes 2-4 milligrams/kilogram per day, trental intravenous drip. heparin in a dosage of 200-700 units per kilogram of body weight per day subcutaneously or intravenously 4 times a day, gradually withdrawn with a decrease in the single dose. activators of fibrinolysis - nicotinic acid. In severe cases, plasmapheresis or glucocorticosteroid therapy is prescribed. In exceptional cases, cytostatics such as Azathioprine or Cyclophosphamide are used. In general, the course of the disease is favorable, and immunosuppressive or cytostatic therapy is rarely used (for example, in the development of autoimmune nephritis).

Children must be registered at a dispensary. Conducted over 2 years. For the first 6 months, the patient visits the doctor monthly, then once every 3 months, then once every 6 months. Prevention is carried out by sanitation of foci of chronic infection. Regularly examine stool for helminth eggs. Such children are contraindicated in sports, various physical procedures and exposure to the sun.

Causes

The exact cause of hemorrhagic vasculitis (HV) has not yet been clarified, however, most scientists are inclined to the multi-etiological theory of the development of pathology. The leading factors provoking the development of the disease are:

viral and bacterial infections,
chronic infections,
food or drug allergies,
vaccines.

The mentioned reasons provoke a malfunction of the immune system, which begins to produce pathogenic immune complexes (IgA) that affect the inner lining of small vessels and contribute to the development of inflammation and microthrombosis.

Types of hemorrhagic rash

The division of the rash according to general rules is divided into primary and secondary elements. Spots that appear on unaltered areas of the skin, that is, not exposed to any diseases, are classified as the primary element of a hemorrhagic rash. The evolution of such spots is a secondary element of the rash.

The primary element is divided into cavity or cavityless. Hemorrhagic type rashes are classified as non-inflammatory processes, since pressing on the spot does not contribute to its disappearance or change, while inflammatory spots first disappear and then reappear.

Depending on the size, hemorrhagic rash is divided into three types:

petechiae;
purpura;
ecchymosis.

The smallest rash, which looks like a simple dot, is called petechiae. Purples are slightly larger in size, measuring about 3 mm in diameter and have a round shape. Ecchymoses, in turn, are the largest spots of the hemorrhagic type. They have an irregular shape and exceed 5 mm in size.

In any situation, the occurrence of a hemorrhagic rash is caused by an increase in the permeability of the walls of blood vessels, a violation of the integrity of these walls under the influence of toxins, injuries, and metabolic disorders.

Symptoms of hemorrhagic vasculitis

There are several forms of hepatitis B:

Skin or simple : characterized by the appearance of a specific itchy rash on the lower extremities and buttocks (small pinpoint hemorrhages that rise above the skin and do not disappear with pressure). Over time, the red rash darkens and disappears, leaving areas of increased pigmentation.
Joint: patients complain of pain in the area of large joints (knees, elbows, hips), their swelling and dysfunction.
Abdominal : nausea and vomiting, severe abdominal pain (often cramping), possible intestinal bleeding, development of intestinal gangrene (due to thrombosis).
Renal : urine becomes pink or red due to the admixture of red blood cells, its quantity decreases, protein appears in tests, which are signs of the development of glomerulonephritis and the threat of developing chronic renal failure.
Fulminant : characterized by the development of DIC syndrome and high blood loss.

Often the disease begins with an increase in body temperature to 38-390C, and patients also note general weakness and increased fatigue. Characteristic wave-like course of the disease.

Typical localization of hemorrhagic rash

Photo

The most common place for hemorrhagic rashes to occur is the legs.

However, the rash can also appear on other areas, for example, as shown in the picture below, on the face:

Photo

The rash, when it appears on the legs, is usually localized not on the front surface, and has a fairly symmetrical pattern, as can be seen in the photo:

Photo

But there are often cases when a rash on the body appears on the thighs or buttocks:

Diagnosis of hemorrhagic vasculitis

At the Yauza Clinical Hospital, patients with suspected Henoch-Schönlein disease are prescribed the following set of examinations:

general blood analysis;
general urine analysis;
coagulogram;
determination of the level of antistreptolysin-O, IGA in peripheral blood;
tissue biopsy of the affected organ or part of the body, followed by histological examination of the biopsy.

To make a diagnosis of hemorrhagic vasculitis, the patient must have two or more diagnostic criteria:

specific rashes not associated with low platelet levels;
manifestation of the disease before the age of 20 years;
widespread abdominal pain that worsens after eating, intestinal bleeding;
granulocytic infiltration of the walls of microvasculature vessels, which is confirmed histologically.

Diagnostic methods

Since in children the disease manifests itself with bright, acute symptoms from the very beginning, there are, as a rule, no problems with making a diagnosis. Diagnosis of hemorrhagic vasculitis in adults is much more difficult, especially in the absence of a characteristic rash at the onset of the disease. As a rule, it is based on laboratory tests, which include:

blood tests - general, biochemical, coagulogram;
urine examination for hematuria, proteinuria, cylindruria, Nechiporenko and Zimnitsky tests, biochemical analysis;
stool test for the presence of blood.

An important stage of diagnosis, which allows us to establish the degree of damage to internal organs, is instrumental studies - ultrasound of the abdominal cavity and kidneys, ultrasound of the renal vessels, gastroscopy. In severe cases of the disease, a biopsy of the skin and kidneys is prescribed to determine the size of immunoglobulin deposits and the permeability of the vascular wall.

Therapeutic measures

At the Yauza Clinical Hospital, treatment of hemorrhagic vasculitis is aimed at achieving the following goals:

elimination of clinical signs of pathology;
reducing the risk of complications;
preventing damage to vital organs;
complete recovery of the patient or achievement of stable long-term remission.

To do this, our rheumatologist develops an individual treatment regimen for each patient, which includes:

bed rest for at least 3 weeks;
avoiding contact with allergens;
diet therapy;
prescribing enterosorbents, antihistamines, antispasmodics, hemostatic agents and antiplatelet agents;
in some cases, the use of hormones and cytostatics is justified.

To reduce the destructive influence of circulating immune complexes and enhance the effectiveness of drug therapy, patients with hepatitis B undergo extracorporeal hemocorrection.

Pigment

Pigment spots on the skin are spots that appear as a result of an increase or decrease in the amount of melanin pigment. In case of increased pigmentation, the spots are called hyperpigmented, in case of lack of pigment or its absence, depigmented or hypopigmented.

Hyperpigmented spots on the skin are divided into:

congenital (these include birthmarks, lentigo);
acquired (freckles, chloasma).

Depigmented spots are divided into:

congenital (for example, albinism);
acquired (vitiligo, leucoderma).

Secondary depigmentation can occur in patients with psoriasis, eczema, rosea or pityriasis versicolor at the site of the primary painful rash. These rashes turn pale against the background of tanned areas of the skin because in the area of inflammation, the ability of melanocytes to produce pigment is weakened. Such areas of depigmentation are called secondary leukoderma, or pseudoleukoderma.

Possible complications

Timely treatment contributes to the favorable course of the disease, and with proper therapy there are no scars left. But if you start the process of this disease, as well as in case of improper treatment, dangerous complications can arise:

pulmonary hemorrhage;
formation of diathesis;
intestinal obstruction;
disorders of the liver, heart and kidneys;
peritonitis.

Dangerous complications can be caused by corticosteroid drugs, which are analogues of the hormone cortisol. If its level increases, pathological processes begin to occur in the body. It is possible to develop such disorders as:

insomnia and mood swings;
exacerbation of ulcers and gastritis;
increased blood pressure;
weight gain in certain parts of the body;
swelling and fluid retention in the body;
osteoporosis.

The greatest danger comes from damage to the bone marrow, since it is the bone marrow that is involved in the process of formation of blood cells. To monitor this process, you need to undergo tests periodically. It is important to follow preventive measures. A healthy lifestyle, proper nutrition, giving up bad habits, and maintaining a work and rest schedule help eliminate many diseases. It is also extremely important to monitor your health and the well-being of your loved ones, especially if there are small children in the family.

Folk remedies

Instead of medications, you can use folk remedies. Garlic oil helps to cope well with any type of rash. You can use a decoction of oak bark for baths. This procedure helps speed up the healing process and also has a beneficial effect on the body.

To treat the affected area and make the skin shiny and smooth, it is recommended to use a decoction of barley grain.

Diagnosis of rashes on the body

With HIV and AIDS, it is difficult to visually determine the type of rash - the nature of skin inflammation in infected people is different.

To determine the type of rash and its cause, the following diagnostic measures are carried out:

Clinical analysis of blood and urine.
Blood test for sexually transmitted viruses.
Skin scraping. The method is used to detect intradermal mites and fungi that cause skin diseases.
Blood tests for antibodies to allergens and allergy skin tests.
Hormonal studies.
Biopsy of skin lesions. They detect the presence of cancer cells, which is important for diagnosing Kaposi's sarcoma.

Diet

If there is a hemorrhagic rash, it is imperative to adjust the patient’s diet. The principles of the dietary diet largely depend on the individual characteristics of each specific case, but there are certain general rules. The following must be observed:

reduce consumption of protein-rich foods;
eliminate allergens;
limit salt intake;
portions should be small;
you need to eat small and regularly;
the temperature of the food should be normal;
diet - balanced;
the cooking method is used to prepare dishes;
exclude flavorings and dyes.

The patient needs to give up bad habits and adhere to a healthy lifestyle. Taking vitamins is also important.

Features of the rash

A hemorrhagic rash, a photo of which is in the article, refers to the formation of small non-inflammatory rashes on the skin of different parts of the body. This is how the body reacts to the rupture of capillaries, the subsequent penetration of red blood cells into the upper layers of the skin.

When it appears, a person does not experience any discomfort at all, since the rash does not itch and does not cause pain. When pressing on the affected area, the color of the rash does not change. The number of points largely depends on the cause of the lesion and the severity.

In a child under 5 years of age, the disease can be triggered by vascular pathologies, and in a child from 5 to 15 years old - by colds and infectious diseases. In addition, the hereditary factor is important.