Cherdz-Strauss Syndrome

Granulomatous inflammatory angiitis - Cherdz-Strauss syndrome refers to the group of systemic vasculitis with 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 a specific immunization.

A genetic predisposition to a deficiency of a trypsin inhibitor is presumed, resulting in an increased formation of ANCA with a specificity for proteinase-3.

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

Symptoms. In typical cases, the disease begins with an allergic rhinitis followed by the addition of sinusitis and polypous growths of the nasal mucosa - this is the first stage of the Cherdja-Strauss syndrome. It can last a fairly long period with the subsequent addition of bronchial asthma. The second phase is eosinophilia of 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 advent of systemic vasculitis, there is fever, severe intoxication, weight loss. Pulmonary infiltrates are localized in several segments, they quickly undergo reverse development in the appointment of glucocorticosteroids, which is of diagnostic significance. According to computer tomography, parenchymal infiltrates are located mainly on the periphery and are similar to "frosted glass". With the reduction of computer tomography of high resolution, the vessels are enlarged, with pointed extremities.

Laboratory diagnostics. Severe eosinophilia of blood (up to 30-50% and more). The number of eosinophils exceeds 1.5-10 ⁹ / l. With the appointment of corticosteroids, the content of blood eosinophils rapidly decreases. Significantly increased the total IgE in the blood. A large diagnostic value is the increased amount in the blood of ANCA. Significantly increased ESR.

Diagnostic criteria of the American College of Rheumatology (1990):

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

If there are 4 signs out of 6, sensitivity is 85%, specificity is 97%. For diagnostics, the special severity of bronchial asthma with frequent exacerbations and unstable course also matters.

Treatment. Systemic glucocorticosteroids - prednisolone 1 mg / kg / day with a decrease in a month from the start of treatment. The course of glucocorticosteroids - 9-12 months. With persistent clinical remission and positive dynamics of laboratory indicators - an alternating scheme of glucocorticosteroids. In severe cases with the defeat of internal organs, the combination of glucocorticosteroids with cyclophosphamide at the rate of 2 mg / kg / day during the year with dose adjustment depending on the white blood.

Forecast. As a rule, they are not involved in the kidney process. In cases of involvement in the process of kidney and heart damage, the forecast is unfavorable. The CNS and gastrointestinal tract lesions are also prognostic.