Cherj-Strauss syndrome

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

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Epidemiology

A fairly rare disease, it accounts for only a fifth of all vasculitis of the nodular polyarteritis group. It is more common in middle-aged people, but cases of the disease have been recorded in children and the elderly.

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Causes of Churg-Strauss syndrome

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

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Symptoms of Churg-Strauss syndrome

The initial signs of the disease are characterized by inflammatory allergic reactions: rhinitis, asthma. Later, eosinophilia, eosinophilic pneumonia ("flying" eosinophilic pulmonary infiltrates, severe broncho-obstructive syndrome), eosinophilic gastroenteritis develop. In the advanced stage, clinical manifestations of systemic vasculitis dominate: peripheral mono- and polyneuritis, various skin rashes, gastrointestinal tract damage (abdominal pain, nausea, vomiting, diarrhea, less often bleeding, perforation, eosinophilic ascites). Joint damage can manifest itself as arthralgia or arthritis, similar to that in nodular polyarteritis. Kidney damage is quite rare and is benign, but focal nephritis may develop, leading to hypertension.

Cardiac pathology occurs in more than half of patients and is the most common cause of death. The spectrum of lesions is very diverse - most often diagnosed are coronary artery disease, often complicated by myocardial infarction, as well as myocarditis (10-15%), DCM (14.3%), constrictive pericarditis, Leffler's mural fibroplastic endocarditis (characterized by endocardial fibrosis, damage to the papillary muscles and chords, mitral and tricuspid valve insufficiency, formation of mural thrombi with subsequent thromboembolic complications). Congestive heart failure develops in 20-30% of patients. Infective endocarditis may occur.

Diagnosis of Churg-Strauss syndrome

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

Other laboratory findings include normochromic normocytic anemia, leukocytosis, increased ESR and C-reactive protein (CRP) levels. A typical change is an increase in serum ANCA levels, especially those that react with myeloperoxidase, in contrast to the ANCAs characteristic of Wegener's granulomatosis.

Echocardiography is highly effective for diagnosing cardiac lesions.

Classification criteria for Charge-Strauss syndrome (Masi A. et al., 1990)

• Asthma - difficulty breathing or diffuse wheezing on exhalation.
• Eosinophilia - eosinophil content >10% of all leukocytes.
• History of allergy - an unfavorable allergic history in the form of hay fever, allergic rhinitis and other allergic reactions, with the exception of drug intolerance.
• Mononeuropathy, multiple mononeuropathy, or glove or stocking polyneuropathy.
• Pulmonary infiltrates are migratory or transient pulmonary infiltrates diagnosed by radiographic examination.
• Sinusitis - pain in the paranasal sinuses or radiographic changes.
• Extravascular eosinophils are accumulations of eosinophils in the extravascular space (according to biopsy data).

The presence of 4 or more criteria in a patient allows for a diagnosis of Churg-Strauss syndrome (sensitivity - 85%, specificity - 99%).

Differential diagnosis includes polyarteritis nodosa (asthma and atypical lung damage), Wegener's granulomatosis, chronic eosinophilic pneumonia, and idiopathic hypereosinophilic syndrome. Idiopathic hypereosinophilic syndrome is characterized by a higher level of eosinophils, absence of bronchial asthma, allergy history, endocardial thickening of more than 5 mm with the development of restrictive cardiomyopathy, and resistance to glucocorticoid treatment. In Wegener's granulomatosis, necrotic changes in the ENT organs are combined with minimal eosinophilia and frequent kidney damage; allergies and bronchial asthma are encountered, in contrast to Churg-Strauss syndrome, no more often than in the population.

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Treatment of Churg-Strauss syndrome

The basis of treatment is glucocorticoids. Prednisolone is prescribed at a dose of 40-60 mg/day, the drug can be discontinued no earlier than a year after the start of treatment. If treatment with prednisolone is insufficiently effective or in severe, rapidly progressing cases, cytostatics are used - cyclophosphamide, azathioprine.

Prevention

Since the etiology of vasculitis is unknown, primary prevention is not performed.

Prognosis of Churg-Strauss syndrome

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

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History of the issue

The disease was first described by J. Churg and L. Strauss in 1951, who suggested that it was based on allergy. Until recently, Churg-Strauss syndrome was considered an asthmatic variant of polyarteritis nodosa, and in recent decades it has been identified as an independent nosological form.

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