avkАрхивъ внутренней медициныThe Russian Archives of Internal Medicine2226-67042411-6564“SINAPS” LLC10.20514/2226-6704-2023-13-2-85-96avk-1591Research ArticleОБЗОРНЫЕ СТАТЬИREVIEW ARTICLESСовременные представления о клинике и диагностике первичного васкулита центральной нервной системыModern Concepts on the Clinic and Diagnosis of Primary Vasculitis of the Central Nervous Systemhttps://orcid.org/0000-0003-2139-5446ФоминаН. В.FominaN. V.

Кемерово

Kemerovo

https://orcid.org/0000-0002-4487-0521ЯковлевА. Ю.YakovlevA. Yu.

Кемерово

Kemerovo

https://orcid.org/0000-0002-2000-3562УткинаЕ. В.UtkinaE. V.

Екатерина Владимировна Уткина

Кемерово

Ekaterina V. Utkina

Kemerovo

goll1997.2011@mail.ruФГБОУ ВО Кемеровский государственный медицинский университет Минздрава России; ГАУЗ Кемеровская областная клиническая больница им. С.В. БеляеваРоссияKemerovo State Medical University of the Ministry of Health of Russia; Kemerovo Regional Clinical Hospital named after S.V. BelyaevRussian FederationФГБОУ ВО Кемеровский государственный медицинский университет Минздрава РоссииРоссияKemerovo State Medical University of the Ministry of Health of RussiaRussian FederationФГБОУ ВО Кемеровский государственный медицинский университет Минздрава России; ГАУЗ Кемеровская городская клиническая больница № 11РоссияKemerovo State Medical University of the Ministry of Health of Russia; Kemerovo City Clinical Hospital № 11Russian Federation2023040420231328596Copyright © Фомина Н.В., Яковлев А.Ю., Уткина Е.В., 20232023Фомина Н.В., Яковлев А.Ю., Уткина Е.В.Fomina N.V., Yakovlev A.Y., Utkina E.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.medarhive.ru/jour/article/view/1591

Первичный васкулит центральной нервной системы (ПВЦНС) — редкая форма васкулита неизвестной причины, поражающего сосуды головного, спинного мозга и мозговых оболочек без системного поражения. Установлено, что средний возраст начала заболевания приходится на 50 лет. Клинические проявления зависят от калибра пораженных сосудов. Наиболее частыми начальными симптомами являются головная боль и сосудистые когнитивные нарушения, что связано с поражением сосудов малого калибра. Развитие инсульта и очаговых симптомов взаимосвязано с сосудистыми когнитивными нарушениями и проявляется поражением средних/крупных мозговых артерий. Диагностика ПВЦНС затруднена, так как симптомы васкулита за пределами центральной нервной системы встречаются редко, серологические маркеры воспаления находятся в норме. Анализ спинномозговой жидкости обычно не соответствует норме из-за умеренного неспецифического повышения уровня общего белка или количества лейкоцитов. У 97 % пациентов с ПВЦНС выявляются отклонения от нормы (инфаркты, иногда опухолевидные поражения) по данным магнитно-резонансной томографии головного мозга. Ангиография имеет низкую чувствительность и низкую специфичность, так как позволяет верифицировать васкулит только средних и крупных церебральных артерий, выявляя сегментарные сужения. Для выявления воспаления кровеносных сосудов, а также для исключения других заболеваний необходимо выполнить биопсию вещества и мягких оболочек мозга.

Primary vasculitis of the central nervous system (PACNS) is a rare form of unknown cause vasculitis that affects the vessels of the brain, spinal cord and meninges without systemic damage. It was found that the average age of the onset of the disease was 50 years. Clinical manifestations depend on the caliber of the affected vessels. The most common initial symptoms are headache and vascular cognitive impairment associated with small vessel involvement. The development of stroke and focal symptoms is interrelated with vascular cognitive impairment and manifests as the lesion of the middle/large cerebral arteries. PACNS is difficult to diagnose, since symptoms of vasculitis outside the central nervous system are rare, serologic markers of inflammation are normal. The analysis of cerebrospinal fluid is usually abnormal due to a moderate nonspecific increase in the level of total protein or the number of leukocytes. Deviations from the norm (cerebral infarction, sometimes tumor-like lesions) are detected according to the data of magnetic resonance imaging of the brain in 97 % of patients with PACNS. Angiography has low sensitivity and low specificity, since it allows to verify vasculitis of only middle and large cerebral arteries, revealing segmental narrowing. To detect inflammation of the blood vessels, as well as to exclude other diseases, it is necessary to perform a biopsy of the substance and the soft membranes of the brain.

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The authors declare that there are no conflicts of interest present.