Churg-Strauss Syndrome

The Chard-Strauss syndrome is an eosinophilic granulomatous inflammation characterized by systemic necrotizing segmental panagyitis of small vessels (arterioles and venules) with eosinophilic perivascular infiltration. Changes in blood vessels and organs lead to the formation of numerous eosinophilic infiltrates in tissues and organs (especially in lung tissue), followed by the formation of perivascular granulomas.

[1], [2]

Epidemiology

A rare disease, it accounts for only a fifth of all vasculitis group of nodular polyarteritis. It is more common in middle-aged people, but there are cases of illness in children and elderly people.

[3], [4], [5]

Causes of Chard-Strauss syndrome

The etiology of this disease is not clear. Often, patients can identify an unfavorable allergic anamnesis, most often - polyvalent drug allergy.

[6], [7]

Symptoms of the Chard-Strauss syndrome

Initial signs of the disease are characterized by inflammatory allergic reactions: rhinitis, asthma. Later, eosinophilia develops, eosinophilic pneumonia ("volatile" eosinophilic pulmonary infiltrates, severe bronchial obstructive syndrome), eosinophilic gastroenteritis. In the expanded stage, clinical manifestations of systemic vasculitis dominate: peripheral mono- and polyneuritis, a variety of skin rashes, lesions of the gastrointestinal tract (abdominal pain, nausea, vomiting, diarrhea, less often bleeding, perforation, eosinophilic ascites). The defeat of the joints can be manifested by arthralgia or arthritis, similar to that of nodular polyarteritis. The defeat of the kidneys is rare enough and proceeds benignly, but the development of focal nephritis leading to hypertension is possible.

The pathology of the heart is found in more than half of the patients and is the most frequent cause of death. The spectrum of lesions is the most diverse - coronaryitis is often diagnosed, often complicated by myocardial infarction, as well as myocarditis (10-15%), DCMP (14.3%), constrictive pericarditis, Leffler's near-wall fibroplastic endocarditis (characterized by endocardial fibrosis, papillary muscle damage and chords , insufficiency of mitral and tricuspid valves, the formation of parietal thrombi with subsequent thromboembolic complications). In 20-30% of patients develop congestive heart failure. Possible attachment of infective endocarditis.

Diagnosis of Chard-Strauss syndrome

A characteristic laboratory indicator of the Chard-Strauss syndrome is peripheral blood hypereosinophilia (> 10 ⁹ L), but its absence is not a basis for excluding this diagnosis. A correlation was established between the level of eosinophilia and the severity of the symptoms of the disease.

Other laboratory indices are normochromic normocytic anemia, leukocytosis, an increase in ESR and concentration of C-reactive protein (CRP). A typical change is an increase in serum levels, ANCA, especially those reacting with myeloperoxidase, in contrast to ANCA, characteristic of Wegener's granulomatosis.

For the diagnosis of cardiac lesions, echocardiography is highly effective.

Classification criteria for the Chard-Strauss syndrome (Masi A. Et al., 1990)

• Asthma - difficulty breathing or diffuse rales on exhalation.
• Eosinophilia - the content of eosinophils> 10% of all leukocytes.
• An allergy in the history is an unfavorable allergic anamnesis in the form of pollinosis, allergic rhinitis and other allergic reactions, with the exception of drug intolerance.
• Mononeuropathy, multiple mononeuropathy or polyneuropathy by the type of "gloves" or "stockings."
• Pulmonary infiltrates are migratory or transient pulmonary infiltrates, diagnosed by X-ray examination.
• Sinusitis - pain in the paranasal sinuses or radiological changes.
• Extra-vascular eosinophils - accumulation of eosinophils in the extravascular space (according to biopsy data).

The presence of 4 or more criteria allows the patient to diagnose "Chard-Strauss syndrome" (sensitivity - 85%, specificity - 99%).

Differential diagnosis is performed with nodular polyarteritis (asthma and atypical lung injury), Wegener's granulomatosis, chronic eosinophilic pneumonia and idiopathic hypereosinophilic syndrome. For idiopathic hypereosinophilic syndrome, a higher level of eosinophils, absence of bronchial asthma, allergic anamnesis, an endocardium thickening of more than 5 mm with the development of restrictive cardiomyopathy, resistance to treatment with glycocorticoids are characteristic. With Wegener's granulomatosis, necrotic changes in the ENT organs are combined with minimal eosinophilia and frequent renal damage; allergy and bronchial asthma are found, unlike the Chard-Strauss syndrome, not more often than in the population.

[8], [9], [10], [11]

Treatment of Chard-Strauss syndrome

The basis of treatment is glucocorticoids. Prednisolone is prescribed in a dose of 40-60 mg / day, drug cancellation is possible not earlier than one year after the start of treatment. With insufficient effectiveness of treatment with prednisolone or in severe fast-progressing current, use cytostatics - cyclophosphamide, azathioprine.

Prevention

Due to the fact that the etiology of vasculitis is unknown, no primary prevention is performed.

Forecast of the Chard-Strauss syndrome

The prognosis of the Chard-Strauss syndrome depends on the degree of respiratory failure, the nature of cardiac disorders, the activity and generalization of vasculitis; with adequate therapy, the 5-year survival rate is 80%.

[12], [13], [14], [15]

Background

For the first time the disease was described by J. Churg and L. Strauss in 1951, who suggested that it was based on an allergy. Until recently, the Chard-Stross syndrome was considered as an asthmatic variant of nodular polyarteritis, and in recent decades isolated into an independent nosological form.

[16], [17]