The Charge-Strauss Syndrome
The Charge-Strauss syndrome is named after the scientists Charge and Strauss who first described the disease. The disease is a separate type of vasculitis - allergic angiitis and granulomatosis - affecting mainly medium and small vessels. The disease is also combined with pathologies such as pneumonia, eosinophilia and bronchial asthma. Previously, this pathology was considered an asthmatic type of periarteritis of the nodular form, but now it is isolated into an independent nosological type.
In 1951, Charles and Strauss (Strauss) first described the syndrome in 13 patients who had asthma, eosinophilia, granulomatous inflammation, necrotic systemic vasculitis and necrotizing glomerulonephritis. In 1990, the American College of Rheumatology (ACR) proposed the following six criteria for the diagnosis of the Chang-Strauss syndrome:
- Asthma (wheezing, wheezing in exhalation).
- Eosinophilia (more than 10%).
- Sinusitis.
- Pulmonary infiltrates (may be temporary).
- Histological evidence of vasculitis with extravascular eosinophils.
- Multiple mononeuritis or polyneuropathy.
The presence of four or more criteria has a specificity of 99.7%.
Epidemiology
Approximately 20% of vasculitis from the nodular periarteritis category occur in the Charge-Strauss syndrome. This pathology on average begins to develop at about 44 years. At the same time, men are slightly more likely to fall ill - 1.3 times.
The frequency of the Chang-Strauss syndrome in the United States is 1-3 cases per 100,000 adults per year and about 2.5 cases per 100,000 adults per year in the world.
Causes of the the Charge-Strauss syndrome
The causes of this syndrome are not yet clear. The mechanism of development is based on immune inflammation, destructive and proliferative processes, and in addition to reducing the permeability of the walls of blood vessels, the formation of thrombi, the development of ischemia in the field of vascular destruction and hemorrhage. In addition, with the development of the syndrome, an important ANCA titer is important, which performs antigen neutralization of neutrophil enzymes (mainly proteinase-3 and myeloperoxidase). At the same time, ANCA causes a violation of the transendothelial movement of activated granulocytes, and provokes premature degranulation. Due to changes in the vessels in the organs and tissues, pulmonary infiltrates appear with the subsequent development of necrotizing nodular inflammation.
The starting factors for the development of the Churg-Strauss syndrome can be a bacterial or viral infection (eg, nasopharynxal lesions of staphylococci or hepatitis type B), various allergens, intolerance of individual drugs, vaccination, excessive cooling, stress, pregnancy or childbirth, and insolation.
The presence of HLA-DRB4 may be a genetic risk factor for the development of the Chang-Strauss syndrome and may increase the likelihood of developing vasculitic manifestations of the disease.
Risk factors
This disease is quite rare, and although many people fall into risk factors, only a small percentage of the population develops. Among these factors are the following:
- Age - most patients with this syndrome are in the age group of 38-52 years. Only occasionally, this pathology develops in the elderly people or children;
- Presence in the anamnesis of the patient of an allergic rhinitis or asthma. The majority of patients with the Charge-Strauss syndrome previously had one of these diseases (often it was severe).
Pathogenesis
The pathogenesis of the disease is not yet fully understood. But in any case, the fact that it is allergic and immunopathological in nature, often occurs as a result of bronchial asthma, and is similar in symptoms to the nodular form of periarteritis, which makes it possible to conclude that the basis for its development are various immunological disorders.
Symptoms of the the Charge-Strauss syndrome
In addition to nonspecific manifestations, which are also observed in the case of nodular periarteritis (fever, loss of appetite, weight loss and malaise), the main symptom is a malfunction of the lungs. As a result, bronchial asthma begins in severe form, and darkening also occurs in the lungs, which are adhered to the X-ray of the lungs, which often quickly disappear-volatile ELI.
Among the main symptoms of the pathology is GD syndrome, which is usually a precursor of the emergence of clinical signs of systemic vasculitis. Also, infectious pathology of the lungs is frequently observed, followed by the development of bronchial asthma of an infectious-dependent type, as well as bronchiectasis. Pulmonary infiltrates are observed in 2/3 patients with the syndrome. About a third of patients are diagnosed with pleurisy with an increase in the number of eosinophils in the pleural fluid.
When lesions of the gastrointestinal tract begin to appear pain in the abdomen, as well as diarrhea and, in rare cases, bleeding. The development of these symptoms is provoked by either eosinophilic gastroenteritis or vasculitis of the intestinal wall. The latter disease can cause peritonitis, end-to-end destruction of the intestinal wall or intestinal obstruction.
Clinical manifestations of cardiac disorders occur in 1/3 of all patients, but on autopsy they are found in 62% of cases, and the cause of death is in 23% of cases. A variety of changes in the ECG readings are observed in about half of the patients. Approximately 1/3 of all cases of the disease is accompanied by the development of heart failure or pericarditis in acute or constrictive form. Occasionally, patients will notice a rise in blood pressure and myocardial infarction.
Skin diseases are observed in 70% of patients - with ESS this is a more frequent symptom than with nodular periarteritis. Among the manifestations:
- Hemorrhagic rash;
- Nodules on the skin and under the skin;
- Redness of the skin;
- Hives;
- Microabscesses of the skin;
- Reticular livedo.
Kidney diseases are observed less often than all other symptoms. However, they do not proceed as dangerously as in the case of Wegener's granulomatosis or nodular periarteritis. Approximately half of patients with the syndrome suffer from focal nephritis, which often becomes the cause of hypertension. In patients with antineutrophil antibodies, necrotizing glomerulonephritis may develop in the plasma.
Diseases of the joints (such as polyarthralgia or polyarthritis) occur in half of all patients with this syndrome. Usually, they develop non-progressive type migratory arthritis, which affects both small and large joints. In some cases, there is myositis or myalgia.
Stages
The Charge-Strauss syndrome in the course of its development usually passes through 3 main stages (conditionally).
In the initial period (it can last for 30 years), patients suffer from frequent allergies, including asthma, hay fever, and rhinitis.
At the second stage, an increase in the number of eosinophils in tissues and blood begins. At this stage, patients are often diagnosed with Lefler's syndrome, pulmonary infiltration with eosinophilia or eosinophilic gastroenteritis.
In the third stage, the clinical manifestations of systemic vasculitis are observed in patients.
Complications and consequences
Among the complications of the disease the most dangerous is pneumonia, which develops under the influence of Pneumocystis carini.
ESS is dangerous because it affects a variety of organs, including the heart, lungs, skin, GIT, muscles, joints, and kidneys. If this disease is not treated, it can lead to death. Possible complications of the disease:
- Violation of the peripheral nerves;
- Itching of the skin and its ulceration, as well as infectious complications;
- Inflammation of the pericardium, the development of myocarditis, and in addition to heart failure and heart attacks;
- Glomerulonephritis, in which kidneys gradually lose the function of filtration, as a result of which kidney failure develops.
Risk stratification
The French Vasculitis Study Group has developed a five-point (five-factor) system that predicts the risk of death in the Charge-Strauss syndrome using clinical manifestations. These factors are:
- Decreased renal function (creatinine> 1.58 mg / dL or 140 μmol / L)
- Proteinuria (> 1 g / 24 hours)
- Gastrointestinal bleeding, heart attack, or pancreatitis
- The defeat of the central nervous system
- Cardiomyopathy
The absence of any of these factors indicates an easier course, with a five-year mortality rate of 11.9%. The presence of one factor indicates a serious illness, with a five-year mortality rate of 26%, and two or more indicate very serious diseases: 46% of deaths within five years.
Diagnostics of the the Charge-Strauss syndrome
Among the main diagnostic criteria are such symptoms as eosinophilia, bronchial asthma, as well as allergies in the anamnesis. In addition, eosinophilic pneumonia, neuropathy, inflammation of the paranasal sinuses, extravascular eosinophilia. If the patient has at least 4 of these diseases, the ESS can be diagnosed.
Analyzes
The blood is taken for general analysis: for diagnosis of the disease, there should be an excess of the number of eosinophils 10% higher than normal, as well as acceleration of the process of erythrocyte sedimentation.
At the general or common research of urine there should be occurrence in it of the protein, and also increase in number of erythrocytes.
Transbronchial and cutaneous biopsy, as well as biopsy of subcutaneous tissue: in this case, pronounced infiltration of eosinophils in the walls of small vessels, as well as necrotizing granulomatous processes in the respiratory organs.
Immunological analysis to detect an increased number of ANCA antibodies. The focus here is on increasing the total IgE, as well as perinuclear antibodies that have antimyeloperoxidase activity.
Instrumental diagnostics
CT of the lungs - visualization of parenchymal accumulations is carried out in the process, mainly located along the periphery; It is also noticeable that the bronchial walls thickened, with expansion in some places, bronchiectasis is noticeable.
Microscopy of exudate located in the pleural fluid, as well as conduction of medical bronchoscopy to detect eosinophilia.
Carrying out ECHO-CG to detect symptoms of mitral regurgitation, as well as proliferation of connective tissue with the appearance of scars in the heart muscle.
Treatment of the the Charge-Strauss syndrome
Treatment of ESS is complex - during the therapy, cytostatics and glucocorticosteroids are used. As a whole, the treatment is similar to the principles of therapy of the Uzel periarteritis.
Medicinal treatment
In case of involvement of the digestive tract, as well as lungs, kidneys and skin, treatment should be started with the use of prednisolone. The therapeutic dose is 60 mg / day. After 1-2 weeks of such reception there is a transition to the periodic use of prednisolone with a gradual decrease in the dose. After 3 months, if there was an improvement in the condition, the dosage is continued to decrease, leading to maintenance, which is left for a long time.
If prednisolone had no effect, as well as in case of the appearance of symptoms of diffuse CNS disorder, insufficient blood flow and conduction disorders, internal administration of hydroxyurea is prescribed at a dosage of 0.5-1 g / day. If the number of white blood cells decreased to 6-10x109 / l, you need to switch to using the drug in 2 days. On the third or cancel his admission for 2-3 months. Other cytostatics, such as cyclophosphamide or azathioprine, can also be used.
If a patient has bronchial asthma, bronchodilators are prescribed.
As a prophylactic measure for the development of thromboembolic complications, anticoagulants or antiplatelet agents (such as acetylsalicylic acid at a dosage of 0.15-0.25 g / day or quarantil at a dosage of 225-400 mg / day) are used.
A systematic analysis conducted in 2007 showed the effectiveness of the use of shock doses of steroids.
Vitamins
Since corticosteroids adversely affect the strength of bones, it is necessary to compensate for the loss of intake of the necessary amount of calcium, and also use about 2000 IU of vitamin D per day.
Forecast
The Churg-Strauss syndrome without timely treatment has an unfavorable prognosis. In the case of multiple organ dysfunction, the disease progresses very quickly, increasing the risk of death due to abnormalities in the heart and lungs. Adequate treatment allows to provide a 5-year survival rate in 60-80% of patients.
The main causes of death in the Charge-Strauss syndrome are myocarditis and myocardial infarction.