Vestnik dermatologii i venerologiiVestnik dermatologii i venerologiiВестник дерматологии и венерологии0042-46092313-6294Rossijskoe Obschestvo Dermatovenerologov i Kosmetologov1680810.25208/vdv16808REVIEWSОБЗОР ЛИТЕРАТУРЫResearch ArticleImmunosuppressive therapy of patients with pemphigus with ineffectiveness of systemic corticosteroidsИммуносупрессивные препараты для лечения больных пузырчаткой при неэффективности системной терапии кортикостероидамиhttps://orcid.org/0000-0002-9688-27273385-4723ChikinVadim V.ЧикинВадим Викторович
Russian Federation

MD, Dr. Sci. (Med.), Senior Researcher

д.м.н., старший научный сотрудник

chikin@cnikvi.ruhttps://orcid.org/0000-0003-3805-84893604-6491KaramovaArfenya E.КарамоваАрфеня Эдуардовна
Russian Federation

MD, Dr. Sci. (Med.), Senior Researcher

к.м.н., доцент

karamova@cnikvi.ruState Research Center of Dermatovenereology and CosmetologyГосударственный научный центр дерматовенерологии и косметологии221120241512202410066211007202430092024Copyright ©; 2024, Vadim V. Chikin, Arfenya E. KaramovaCopyright ©; 2024, Чикин В.В., Карамова А.Э.2024Vadim V. Chikin, Arfenya E. KaramovaЧикин В.В., Карамова А.Э.https://creativecommons.org/licenses/by-nc/4.0https://vestnikdv.ru/jour/article/view/16808

Therapy of patients with pemphigus vulgaris with systemic corticosteroids may not be effective enough or lead to the development of severe adverse events that make it necessary to reduce the dose of corticosteroid. In such cases, it is possible to use the immunosuppressants. The literature review analyzes case reports and the results of studies that evaluated the effectiveness and safety of drugs used for immunosuppressive therapy of patients with pemphigus vulgaris. The drugs doses, the studies design, and criteria for evaluating the effectiveness of therapy in patients with pemphigus are considered. As a result of the analysis, it was shown that case reports and non-compatarive studies demonstrate the effectiveness and steroid-sparing effect of azathioprine, dapsone, methotrexate, mycophenolate mofetil, cyclosporine and cyclophosphamide in the treatment of patients with pemphigus vulgaris. Nevertheless, comparative studies show an increase in the effectiveness of therapy in patients with pemphigus and a faster achievement of therapeutic effect when prescribing cyclophosphamide, but not azathioprine, dapsone, methotrexate, mycophenolate mofetil or cyclosporine. However, the toxicity of cyclophosphamide limits its use in pemphigus. Thus, adjuvant drugs can be used in the treatment of patients with pemphigus in situations where it is not possible to reduce the dose of a systemic corticosteroid, especially in cases of severe adverse events of corticosteroid therapy.

Терапия больных вульгарной пузырчаткой кортикостероидами системного действия может быть недостаточно эффективной или приводить к развитию тяжелых нежелательных явлений, которые делают необходимым уменьшение дозы кортикостероида. В таких случаях возможно применение лекарственных препаратов иммуносупрессивного действия. В обзоре литературы анализируются описания случаев и результаты исследований, в которых оценивалась эффективность и безопасность лекарственных препаратов, использовавшихся для иммуносупрессивной терапии больных вульгарной пузырчаткой. Рассматриваются дозы лекарственных препаратов, особенности дизайна исследований, критерии оценки эффективности терапии больных пузырчаткой. В результате проведенного анализа показано, что описания случаев и несравнительные исследования демонстрируют эффективность и стероидсберегающий эффект азатиоприна, дапсона, метотрексата, микофенолата мофетила, циклоспорина и циклофосфамида как дополнительных иммуносупрессивных препаратов в лечении больных вульгарной пузырчаткой. Тем не менее сравнительные исследования свидетельствуют об увеличении эффективности терапии больных пузырчаткой и более быстром достижении терапевтического эффекта при назначении циклофосфамида, но не азатиоприна, дапсона, метотрексата, микофенолата мофетила или циклоспорина. Однако токсичность циклофосфамида ограничивает его применение при пузырчатке. Таким образом, иммуносупрессивные препараты могут применяться в лечении больных пузырчаткой в ситуациях, когда не удается уменьшить дозу кортикостероида системного действия, особенно в случаях развития тяжелых нежелательных явлений кортикостероидной терапии.

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