avkАрхивъ внутренней медициныThe Russian Archives of Internal Medicine2226-67042411-6564“SINAPS” LLC10.20514/2226-6704-2020-10-1-38-46avk-998Research ArticleОБЗОРНЫЕ СТАТЬИREVIEW ARTICLESФокально-сегментарный гломерулосклероз: современное состояние проблемыFocal segmental glomerulosclerosis: current status of the problemhttps://orcid.org/0000-0001-8513-9279МуркамиловИ. Т.MurkamilovI. T.

Илхом Торобекович Муркамилов

Бишкек

Bishkek

murkamilov.i@mail.ruhttps://orcid.org/0000-0002-8387-5800СабировИ. С.SabirovI. S.

Бишкек

Bishkek

https://orcid.org/0000-0002-2682-4417ФоминВ. В.FominV. V.

Москва

https://orcid.org/0000-0002-7653-0433МуркамиловаЖ. А.MurkamilovaZh. A.

Бишкек

Bishkek

Кыргызская государственная медицинская академия имени И.К. Ахунбаева; ГОУ ВПО Кыргызско-Российский Славянский университетКыргызстанI.K. Akhunbaev Kyrgyz State Medical Academy; Kyrgyz Russian Slavic UniversityKyrgyzstanГОУ ВПО Кыргызско-Российский Славянский университетКыргызстанKyrgyz Russian Slavic UniversityKyrgyzstanФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. СеченоваРоссияI.M. Sechenov First Moscow State Medical UniversityRussian Federation2020310120201013846Copyright © Муркамилов И.Т., Сабиров И.С., Фомин В.В., Муркамилова Ж.А., 20202020Муркамилов И.Т., Сабиров И.С., Фомин В.В., Муркамилова Ж.А.Murkamilov I.T., Sabirov I.S., Fomin V.V., Murkamilova Z.A.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/998

Одним из наиболее прогностически неблагоприятных вариантов гломерулопатии является фокально-сегментарный гломерулосклероз (ФСГС), который выявляется при нефробиопсии у 5-20% больных с нефротическим синдромом (НС) и у 15% взрослых больных с хроническим гломерулонефритом. ФСГС рецидивирует в трансплантированной почке у 30-50% больных. Среди взрослых больных ФСГС преобладают мужчины. Плохой прогноз ФСГС объясняется неоднородностью заболевания и усугубляется плохим ответом на лечение. По современным данным ФСГС характеризуется склерозом мезангиальной матрицы, гиалинозом, повреждением капилляров, увеличением пенистых клеток и их адгезией между гломерулярным пучком и капсулой Боумена. В 2004 году было предложено следующие гистологические варианты ФСГС: верхушечный, перихилярный, коллабирующий, клеточный и классический. Каждый гистологический вариант ФСГС различается по этиологии, ответу на лечение и по прогнозу. Клиническая диагностика первичного ФСГС должна базироваться на исключении вторичных причин заболевания. Очаговые склеротические изменения в клубочках могут быть вызваны разными факторами и встречаться при различных состояниях, в том числе, и при уже имеющейся патологии почек. По данным международных рекомендаций при терапии ФСГС следует ориентироваться на величину суточной протеинурии. Для больных с ФСГС без выраженной протеинурии рекомендуется использование ингибиторов ангиотензинпревращающего фермента (иАПФ) или блокаторов рецепторов ангиотензина II (БРА). При ФСГС и НС наряду с иАПФ или БРА II используется иммуносупрессивная терапия. Взрослым больным глюкокортикоиды (ГК) назначаются ежедневно в один прием в дозе 1 мг/кг сутки, максимальная доза составляет 80 мг при ежедневном приеме и 120 мг при альтернирующем режиме. Резистентность к ГК констатируется при отсутствии эффекта через 16 недель. При наличии противопоказаний или непереносимости ГК используется ингибиторы кальцинейрина. Рекомендуемая начальная доза циклоспорина составляет 2мг/кг/сутки, принимается два раза в сутки с постепенным увеличением до 3,5-4 мг/кг/сутки. Длительность терапии при удовлетворительной переносимости циклоспорина составляет более шести месяцев. После достижения полной ремиссии дозу циклоспорина снижают постепенно по 0,5 мг/кг/сутки до минимально эффективной дозы (1,5-2 мг/кг/сутки) и проводят такую поддерживающую терапию в течение 1-2 лет. Возможен вариант терапии с использованием более низких доз ГК и циклоспорина, либо комбинация микофенолата мофетила с высокой дозой дексаметазона.

One of the most prognostically unfavorable variants of glomerulopathy is focal segmental glomerulosclerosis (FSHC), which is detected by nephrobiopsy in 5-20% of patients with nephrotic syndrome (NS) and in 15% of adult patients with chronic glomerulonephritis. FSGS recurs in a transplanted kidney in 30-50% of patients. Among adult patients with FSH, men predominate. A poor prognosis of FSHC is explained by the heterogeneity of the disease and is exacerbated by a poor response to treatment. According to current data, FSGS is characterized by sclerosis of the mesangial matrix, hyalinosis, damage to capillaries, an increase in foam cells and their adhesion between the glomerular bundle and the Bowman capsule. In 2004, the following histological variants of FSGS were proposed: apical, perichillary, collaborating, cellular and classical. Each histological variant of FSGS differs in etiology, response to treatment, and prognosis. The clinical diagnosis of primary FSHC should be based on the exclusion of secondary causes of the disease. Focal sclerotic changes in the glomeruli can be caused by various factors and occur in various conditions, including the existing kidney pathology. According to international recommendations for the treatment of FSHS, one should focus on the amount of daily proteinuria. For patients with FSHS without pronounced proteinuria, the use of angiotensin converting enzyme inhibitors (ACE inhibitors) or angiotensin II receptor blockers (ARBs) is recommended. In FSGS and NS, immunosuppressive therapy is used along with ACE inhibitors or ARB II. For adult patients, glucocorticoids (HA) are prescribed daily in a single dose at a dose of 1 mg / kg per day, the maximum dose is 80 mg with a daily intake and 120 mg with an alternating regimen. Resistance to HA is detected in the absence of effect after 16 weeks. In the presence of contraindications or intolerance to HA, calcineurin inhibitors are used. The recommended initial dose of cyclosporine is 2 mg / kg / day, taken twice a day with a gradual increase to 3.5-4 mg / kg / day. The duration of therapy with satisfactory tolerance to cyclosporine is more than six months. After achieving complete remission, the dose of cyclosporin is gradually reduced by 0.5 mg / kg / day to the minimum effective dose (1.5-2 mg / kg / day) and such maintenance therapy is carried out for 1-2 years. A treatment option is possible using lower doses of HA and cyclosporine, or a combination of mycophenolate mofetil with a high dose of dexamethasone.

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