Пузырчатка: актуальные звенья патогенеза и терапии


DOI: https://dx.doi.org/10.18565/pharmateca.2022.8.9-17

Н.В. Зильберберг, М.М. Кохан, А.Н. Шакирова, М.А. Шпак, И.Д. Кузнецов

1) Уральский НИИ дерматовенерологии и иммунопатологии, Екатеринбург, Россия; 2) Пермский государственный медицинский университет им. акад. Е.А. Вагнера, Пермь, Россия
Пузырчатка – группа буллезных дерматозов, в основе которых лежит акантолиз клеток эпидермиса, при этом в крови циркулируют аутоантитела, направленные против антигенов десмосом в многослойном плоском эпителии. В статье описаны основные механизмы развития болезни: генетические факторы, влияние приема некоторых лекарственных препаратов, роль аутоантигенов и аутоантител, механизмы клеточной сигнализации, влияние клеточного иммунитета. Также представлены актуальные варианты лечения данной патологии, которые включают иммунодепрессанты, такие как глюкокортикостероиды и кортикостероид-сберегающие средства: азатиоприн, микофенолата мофетил, циклофосфамид. Новые методы лечения, направленные на снижение циркулирующих аутоантител против десмоглеинов, к которым относится ритуксимаб, представляющий собой химерное моноклональное антитело против CD20 типа I, может связываться с антигеном CD20 и снижать уровень активированных B-лимфоцитов.

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Об авторах / Для корреспонденции


Автор для связи: Анастасия Николаевна Шакирова, младший науч. сотр. научного клинического отдела дерматологии, Уральский НИИ дерматовенерологии и иммунопатологии, Екатеринбург, Россия; shakirova.a96@yandex.ru; eLibrary SPIN: 4825-7475


ORCID:
Н.В. Зильберберг (Zilberberg N.V.), https://orcid.org/0000-0002-7407-7575
М.М. Кохан (Kokhan M.M.), https://orcid.org/0000-0001-6353-6644
А.Н. Шакирова (Shakirova A.N.), https://orcid.org/0000-0003-0198-1739
М.А. Шпак (Shpak M.А.), https://orcid.org/0000-0002-7359-4458
И.Д. Кузнецов (Kuznetsov I.D.), https://orcid.org/0000-0003-1138-4890 


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