Rarely enough, no more than 3 cases per 100 thousand people, there is a special type of vascular inflammation or angiitis, which is called Charge-Strauss syndrome. You can meet the name "Cherge-Strauss Syndrome" according to English pronunciation. Two scientists discovered it independently of one another at once in 1951. Until now, there is no absolutely accurate data that causes this type of angiitis or vasculitis, which is basically the same thing. It has been reliably found that it can be observed in any organs that are associated with small and medium blood vessels, that is, in almost the entire human body.
Medical definition of a disease
In different sources you can find different formulations of what is the sophisticated syndrome of Charles-Strauss. We offer the most appropriate pathogenesis and etiology. This is necrotizing vasculitis (angiitis) of capillaries, venules and arterioles in combination with asthma and eosinophilia, as well as granulomatous and eosinophilic inflammation of the respiratory system.
What does necrotizing mean? When inflammation occurs in small vessels, their very small gaps in diameter quickly become clogged, as a result of which the flow of blood to nearby tissues is disrupted. As a result, both these sites and the vessels themselves die, their necrosis is obtained.
What does eosinophilia mean? White blood cells or leukocytes are in the blood of people, and among them are eosinophils, so named because they are stained with eosin. Normally, these white blood cells should be up to 350 pcs. for 1 ฮผl. If there are more of them, they talk about eosinophilia, which is considered a sign of many diseases and the syndrome of Charge-Strauss including.
What does granulomatous mean? When nodules (granules) are formed in the focus of inflammation, they say what is granulomatous inflammation. It arises due to the proliferation and alteration of certain cells.
Causes
As noted above, the exact answer that causes the syndrome of Charles-Strauss is not yet available.
Scientists suggest that it may occur in the following circumstances:
- genetic predisposition;
- poor environment;
- some infections that enter the human body;
- tumors;
- side effects when taking certain medications.
As a result of these factors, a balanced and smooth functioning of the immune system occurs, in which the development of autoimmune antibodies and killer cells that attack normal cells and tissues in their own body begins. Presumably, this causes an increased formation of eosinophils, since histology reveals extensive and multiple eosinophilic infiltrates, especially in the walls of the respiratory system.
Infections of the Char-Strauss syndrome can provoke infectious diseases such as acute respiratory infections, hepatitis, as well as vaccination, allergies, stress, pregnancy, insolation, hypothermia, childbirth.
Types and Types
Charge-Strauss syndrome can occur acutely (with a sharp response of the body to an irritant, develops in a few days) and chronically (develops over a period of months). Most often, the chronic course of this syndrome affects the respiratory system and resembles bronchial asthma in symptoms. It is such an erroneous diagnosis that is made most often. With a combination of adverse circumstances, the disease in a chronic form is capable of relapsing. This sometimes happens with the cancellation of certain drugs, for example, chronic eosinophilic pneumonia may be aggravated with the cancellation of "Prednisone". If it is in a chronic form that Char-Strauss syndrome occurs, the symptoms of the disease often affect the respiratory tract and are as follows:
- cough;
- dyspnea;
- weight loss;
- wheezing
- labored breathing.
The syndrome can manifest itself with different activity, which is divided into 4 groups:
- zero;
- minimum;
- average;
- high.
Charge-Strauss syndrome: symptoms, photo
The most striking signs of the disease in acute form depend on which organ tissue is affected. It can be the heart, gastrointestinal tract, lungs, musculoskeletal system, skin, nervous system, kidneys.
So, if it is a heart, the patient has arrhythmia, pericarditis (constructive or acute), endocarditis, heart failure up to a heart attack, vascular hypertension.
If the kidneys are affected, then the symptoms are: nephritis (focal) and glomerulonephritis (necrotic).
The defeat of the gastrointestinal tract is characterized by gastritis, colitis, enteritis, peritonitis, perforation of the intestines.
Very characteristic on the skin appears syndrome Charles Strauss. The photo shows ulcerative defects on the lower limbs of the patient, which may be dominant or concomitant with other symptoms, for example, bronchopulmonary (cough, shortness of breath). Other signs of skin lesions are purpura, urticaria, necrotic ulcers, erythema, nodules.
Damage to the respiratory system is accompanied by rhinitis, asthma, infiltrates in the lungs.
A syndrome in the nervous system can trigger a stroke, polyneuropathy, encephalopathy.
In the musculoskeletal system, the syndrome is manifested by polyarthritis, arthropathy, myopathy. The occurrence of each of these complications has its own symptoms.
In all cases, patients note deterioration of health, fatigue, temperature, weakness, loss of appetite, weight loss.
Criteria
In 1990, the ACR proposed 6 criteria for clinically diagnosing Charge-Strauss syndrome:
- Choking, exhale dry rales.
- The eosinophil count is 10% higher than normal.
- Neuropathy (mono or poly), that is, impaired function in the nerves.
- Pathology of the sinuses.
- Infiltrates in the lungs, which can be migratory, permanent, intermittent, transient, detected on radiographs.
- A micropreparation of a small blood vessel fixes accumulations of eosinophils in areas near the vessels being tested.
If 4 of the above criteria are available, the presence of the syndrome of Charles-Strauss is almost not in doubt.
Laboratory diagnostics
People who have symptoms of the above diseases initially go to the appropriate profile for doctors - a gastroenterologist, cardiologist, dermatologist, neurologist, pulmonologist, general practitioner or ENT specialist. After making an anamnesis, patients can be assigned tests that confirm or deny the syndrome of Charles-Strauss. The diagnosis is established based on:
- blood test (determine the increase in ESR, eosinophils);
- chest x-ray;
- CT of the lungs;
- ECG;
- microscopic examination of exudate in the lungs;
- biopsies of the skin or bronchi;
- general analysis of urine;
- immunological tests (for ANCA antibodies, LgE level).
Stages
Charge-Strauss syndrome, the pathogenesis of which depends on the location of the inflamed vessels, is divided into three stages of the course:
1. It lasts a long time, several years, and in some cases, the course of the preromal period of 10 years is recorded. Symptoms fixed in the first stage in most patients:
- frequent allergic reactions;
- rhinitis;
- manifestations of asthma;
- the appearance in the nose of polyps;
- sinusitis;
- protracted, often recurring bronchitis.
2. A striking sign of the second stage is an increase in eosinophils in the blood and in the affected tissues. Patients may be diagnosed with eosinophilic gastroenteritis, Lefler's syndrome, eosinophilic pneumonia or pleurisy. Symptoms manifested in most patients:
- coughing;
- lack of air (choking);
- hemoptysis;
- weakness;
- myalgia;
- fever;
- weight loss;
- with gastrointestinal tract damage, stool disturbance, nausea, peritoneal pain.
3. A characteristic sign of the third stage is the symptomatology of systemic angiitis (vasculitis). Values โโshowing the number of eosinophils in the blood can jump up to 80% against the norm. There are signs of acute heart failure. And if coronary vessels are affected, the patient may die. The development of Charge-Strauss syndrome in the central nervous system in the third stage is fraught with stroke, epilepsy attacks. If the organs of the gastrointestinal tract are affected, perforation of the intestine or stomach, intestinal obstruction are possible.
Treatment
A fairly serious disease is the syndrome of Charge-Strauss. Treatment is carried out for a long time and necessarily comprehensively. The main treating doctor is a rheumatologist, but often a consultation with a cardiologist, optometrist, pulmonologist and other specialists is required. To restore the functions of the affected vessels, a rheumatologist appoints glucocorticoids and cytostatics, as well as plasmapheresis and hemosorption. Together with these drugs, anti-inflammatory drugs are prescribed. Among the commonly prescribed drugs are: Prednisone (course up to 12 months), Cyclophosphamide or analogues (course up to 1 year), Metipred or analogues, antibacterial and other drugs according to indications. To prevent complications, patients are prescribed anticoagulants, vasodilators and antiplatelet agents. During treatment, the patient is prohibited from smoking and drinking alcohol.
Forecast
Rarely enough, more often than men, women are diagnosed with Charge-Strauss syndrome. How much they live with it depends on how quickly treatment is started and which vessels are affected. Statistics say that it appears mainly in people over 40 years old. In children, this disease is not diagnosed. Without treatment, the prognosis is only unfavorable. With timely and effective treatment, survival for more than 5 years is up to 80%. Death most often occurs from damage to the coronary vessels and impaired heart function.