Cerge-Strauss syndrome

Granulomatous inflammatory angiitis - Churg-Strauss syndrome belongs to a group of systemic vasculitis with damage to small-caliber vessels (capillaries, venules, arterioles), associated with the detection of antineutrophil cytoplasmic autoantibodies (ANCA). In children, this form of systemic vasculitis is rare.

Etiology and pathogenesis . The disease is preceded by infectious diseases caused by viruses and bacteria, a tendency to allergic reactions is noted, the development of the disease was observed after specific immunization.

A genetic predisposition to trypsin inhibitor deficiency is suggested, resulting in increased production of ANCA with specificity for proteinase-3.

Histology.Small necrotic granulomas and necrotic vasculitis of small arteries and veins are characteristic. Granulomas are located extravascularly near arterioles and venules and consist of a central eosinophilic core and radially surrounding macrophages and giant cells. Of the inflammatory cells, eosinophils predominate, neutrophils are less, and the number of lymphocytes is insignificant.

Symptoms. In typical cases, the disease begins with allergic rhinitis followed by sinusitis and polypous growths of the nasal mucosa - this is the first stage of Churg-Strauss syndrome. It can last for quite a long period with subsequent addition of bronchial asthma. The second phase is eosinophilia of the peripheral blood and their migration into tissues: transient pulmonary infiltrates, chronic eosinophilic pneumonia or eosinophilic gastroenteritis with periodic exacerbation for several years. The third stage is characterized by frequent exacerbations and severe attacks of bronchial asthma, the appearance of signs of systemic vasculitis. With the appearance of systemic vasculitis, fever, severe intoxication, and weight loss appear. Pulmonary infiltrates are localized in several segments, they quickly undergo regression when glucocorticosteroids are prescribed, which has diagnostic value. According to CT data, parenchymatous infiltrates are located predominantly on the periphery and resemble "ground glass". When high-resolution CT is used, the vessels are dilated, with pointed ends.

Laboratory diagnostics.Severe blood eosinophilia (up to 30-50% or more). The number of eosinophils exceeds 1.5-10 ⁹ /l. With the administration of corticosteroids, the content of blood eosinophils quickly decreases. The content of total IgE in the blood is significantly increased. An increased amount of ANCA in the blood is of great diagnostic importance. ESR is significantly increased.

Diagnostic criteriaAmerican College of Rheumatology (1990):

1. Asthma.
2. Eosinophilia more than 10%.
3. Mono- or polyneuropathy.
4. Volatile pulmonary infiltrates.
5. Sinusitis.
6. Extravascular tissue eosinophilia.

In the presence of 4 signs out of 6, the sensitivity is 85%, specificity is 97%. The particular severity of bronchial asthma with frequent exacerbations and unstable course is also important for diagnosis.

Treatment. Systemic glucocorticosteroids - prednisolone 1 mg/kg/day with a decrease after a month from the start of treatment. The course of glucocorticosteroids is 9-12 months. In case of persistent clinical remission and positive dynamics of laboratory parameters - an alternating scheme of glucocorticosteroids. In severe cases with damage to internal organs, a combination of glucocorticosteroids with cyclophosphamide at the rate of 2 mg/kg/day for a year with dose adjustment depending on the white blood cell count.

Forecast.As a rule, the kidneys are not involved in the process. In cases of kidney involvement and heart damage, the prognosis is unfavorable. CNS and gastrointestinal tract damage are also prognostically unfavorable.