Syndrome of Cherdzha-Stross
Syndrome of Cherdzha-Stross – the inflammatory and allergic defeat of small and average vessels (capillaries, faded, ) proceeding with formation of nekrotiziruyushchy eozinofilny granulomas. The gipereozinofiliya, defeat of bronchopulmonary system, heart, GIT, the central and peripheral nervous system, skin and joints are characteristic of a syndrome of Cherdzha-Stross. The diagnosis of a syndrome of Cherdzha-Stross is based on data of the anamnesis, a clinical picture, laboratory researches, a X-ray analysis of bodies of a thorax, a biopsy of lungs. As the main therapy of a syndrome of Cherdzha-Stross purpose of system glucocorticosteroids and tsitostatik is shown.
Syndrome of Cherdzha-Stross
Syndrome of Cherdzha-Stross – a kind of a system vaskulit with a granulematozny inflammation of vessels of average and small caliber and primary defeat of a respiratory path. The syndrome of Cherdzha-Stross belongs to the polysystem violations most often mentioning bodies with rich blood supply - skin, lungs, heart, nervous system, a GIT, kidneys. The syndrome of Cherdzha-Stross in many respects reminds nodular periarteriit, but unlike it strikes not only small and average arteries, but also capillaries, veins and venula; it is characterized by an eozinofiliya and a granulematozny inflammation, primary damage of lungs. In rheumatology the syndrome of Cherdzha-Stross meets seldom, annual incidence makes 0,42 cases on 100 thousand population. People from 15 to 70 years suffer from a syndrome of Cherdzha-Stross, average age of patients makes 40-50 years; at women the disease comes to light a little more often than at men.
Reasons of a syndrome of Cherdzha-Stross
The reasons of a syndrome of Cherdzha-Stross are unknown. The pathogenesis is connected with an immune inflammation, proliferative and destructive changes and increase in permeability of a vascular wall, a tromboobrazovaniye, hemorrhages and ischemia in a zone of damage of vessels. An important role in development of a syndrome of Cherdzha-Stross is played by the raised caption of antineytrofilny cytoplasmatic antibodies (ANCA) which anti-gene targets are enzymes of neutrophils (mainly, proteinase-3 and myeloperoxidase). ANCA cause premature degranulation and violation of transendotelialny migration of the activated granulocytes. Vascular changes lead to emergence of numerous eozinofilny infiltrates in fabrics and bodies with formation of nekrotiziruyushchy inflammatory granulomas.
To the forefront at a syndrome of Cherdzha-Stross there is a damage of lungs. At a histologic research interstitsialny and perivaskulyarny eozinofilny infiltrates in walls of pulmonary capillaries, bronchial tubes, bronchioles and alveoluses, perivazalny and perilimfatichesky fabrics come to light. Infiltrates have the various form, are usually localized in several segments of a lung, but can spread to all pulmonary share. Except ostrofazny inflammatory reactions, cicatricial sclerous changes in vessels and pulmonary fabric are noted.
Can provoke development of a syndrome of Cherdzha-Stross a viral or bacterial infection (for example, hepatitis B, staphylococcal damage of a nasopharynx), vaccination, an organism sensitization (allergic diseases, medicinal intolerance), stresses, cooling, insolation, pregnancy and childbirth.
Symptoms of a syndrome of Cherdzha-Stross
The syndrome of Cherdzha-Stross passes three stages in the development. The Prodromalny stage can last several years. At a typical current the syndrome of Cherdzha-Stross begins with defeat of a respiratory path. There are allergic rhinitis, symptoms of nasal obstruction, polipozny growths mucous a nose, recurrent sinusitis, long bronchitis with an asthmatic component, bronchial asthma.
The second stage of a syndrome of Cherdzha-Stross is characterized by increase in level of eosinophils in peripheral blood and fabrics; it is shown by severe forms of bronchial asthma with strong fits of coughing and expiratory suffocation, a blood spitting. Attacks of a bronchospasm are followed by the expressed weakness, long fever, a mialgiya, weight loss. Chronic eozinofilny infiltration of lungs can lead to development of a bronkhoektatichesky disease, eozinofilny pneumonia, eozinofilny pleurisy. At emergence of a pleural exudate thorax pains at breath are noted, strengthening of short wind.
The third stage of a syndrome of Cherdzha-Stross is characterized by development and domination of signs of a system vaskulit with polyorgan defeat. At generalization of a syndrome of Cherdzha-Stross severity of bronchial asthma decreases. The period between emergence of symptoms of bronchial asthma and a vaskulit averages 2-3 years (the interval is shorter, the forecast of a disease is more adverse). The high eozinofiliya (35-85%) is noted. From cardiovascular system development of myocarditis, a koronarit, konstriktivny perikardit, insufficiency of mitralny and three-leaved valves, a myocardial infarction, pristenochny fibroplastichesky endocarditis of Leffler is possible. Defeat of coronary vessels can become the reason of sudden death of patients with a syndrome of Cherdzha-Stross.
Are characteristic of defeat of nervous system peripheral neuropathy (mononeuropathy, disteel polyneuropathy "as gloves or stockings"; radikulopatiya, neuropathy of an optic nerve), TsNS pathology (hemorrhagic stroke, epileptic attacks, emotional frustration). From a GIT development of an eozinofilny gastroenteritis (abdominal pains, nausea, vomiting, diarrhea) is noted, is more rare - bleedings, perforation of a stomach or intestines, peritonitis, intestinal impassability.
At a syndrome of Cherdzha-Stross there is a polymorphic damage of skin in the form of painful hemorrhagic purples on the lower extremities, hypodermic small knots, an eritema, a small tortoiseshell and necrotic bubbles. Poliartralgiya and not progressing migrating arthritis are often observed. Damage of kidneys meets seldom, carries not expressed character, proceeds in the form of a segmentary glomerulonefrit and HPN is not followed.
Diagnostics of a syndrome of Cherdzha-Stross
Patients with a syndrome of Cherdzha-Stross usually ask for primary help various experts - the otolaryngologist, the pulmonologist, the allergist, the neurologist, the cardiologist, the gastroenterologist and late get to the rheumatologist. Diagnostics of a syndrome of Cherdzha-Stross is based on clinical laboratory yielded and results of tool researches. As diagnostic criteria of a syndrome of Cherdzha-Stross are considered: a gipereozinofiliya (> 10% of total number of leukocytes), bronchial asthma, mono - or polyneuropathy, sinusitis, eozinofilny infiltrates in lungs, ekstravaskulyarny nekrotiziruyushchy granulomas. Existence not less than 4 criteria confirms the diagnosis in 85% of cases.
At a syndrome of Cherdzha-Stross anemia, , increase in SOE and level of the general IgE also comes to light. Detection of perinuklearny antibodies with antimiyeloperoksidazny activity (pANCA) is characteristic of more than a half of cases of a syndrome of Cherdzha-Stross
The X-ray analysis of bodies of a thorax at a syndrome of Cherdzha-Stross allows to find quickly disappearing, limited blackouts and focal shadows in lungs, existence of a pleural exudate. At a biopsy of a lung the granulematozny inflammation of small vessels, infiltrates in okolososudisty space containing eosinophils is defined. Differential diagnostics of a syndrome of Cherdzha-Stross should be carried out with nodular polyarteritis, granulematozy Wegener, chronic eozinofilny pneumonia, an idiopathic gipereozinofilny syndrome, a microscopic poliangiit.
Treatment and forecast of a syndrome of Cherdzha-Stross
Treatment of a syndrome of Cherdzha-Stross assumes long purpose of high doses of system glucocorticosteroids. In process of improvement of a state the dose of medicines is reduced. In the presence of defeats of cardiovascular system, lungs, multiple mononeuritis application pulse therapy methylprednisolonum is possible. At inefficiency of glucocorticosteroids cytostatics (, , ) which promote more bystry remission and decrease in risk of a recurrence are used, but create high risk of infectious complications. Before therapy all medicines to which at the patient the sensitization is revealed are cancelled.
Without treatment forecast of a syndrome of Cherdzha-Stross adverse. At polyorgan defeat there is a bystry progressing of a syndrome of Cherdzha-Stross to high risk of death from warm and pulmonary violations. At adequate treatment 5-year survival makes 60-80%.