Перспективы использования пирфенидона при идиопатическом легочном фиброзе и прогрессирующих легочных фиброзах

Идиопатический легочный фиброз (ИЛФ) и прогрессирующие легочные фиброзы (ПЛФ) характеризуются неуклонным прогрессированием, крайне неблагоприятным прогнозом и высокой летальностью. Антифибротические препараты, такие как пирфенидон и нинтеданиб, замедляют снижение легочной функции, улучшают выживаемость пациентов и являются основой в лечении ИЛФ. В последние годы активно изучается применение антифибротических препаратов при ПЛФ. Пирфенидон, обладающий антифибротическим, противовоспалительным и антиоксидантным действием, ингибирует ключевые пути фиброгенеза, включая ТФР-β. В данном обзоре представлены современные данные о применении пирфенидона при ИЛФ и ПЛФ, в т. ч. при гиперчувствительном пневмоните, силикозе, интерстициальных заболеваниях легких, ассоциированных с болезнями соединительной ткани. В работе рассмотрены механизмы действия пирфенидона, его доказательная база (включая результаты ключевых клинических исследований CAPACITY, ASCEND и RELIEF), а также данные реальной клинической практики, подтверждающие, что пирфенидон замедляет прогрессирование заболевания и снижение легочных функциональных показателей как при ИЛФ, так и ПЛФ. В сравнительной оценке пирфенидона и нинтеданиба оба препарата демонстрируют сопоставимую эффективность, однако пирфенидон имеет более предсказуемый профиль безопасности с дозозависимыми желудочно-кишечными нарушениями и фотосенсибилизацией в качестве основных нежелательных явлений. Серьезные побочные эффекты наблюдаются редко, что позволяет рассматривать пирфенидон как относительно безопасный вариант терапии. Особое внимание уделено новым стратегиям терапии, таким как комбинированное применение с нинтеданибом, использование низких доз и ингаляционных форм препарата. Приводятся рекомендации по оптимизации лечения с учетом профиля безопасности пирфенидона и индивидуальных особенностей пациентов. Обсуждаются перспективы дальнейшего использования пирфенидона при различных вариантах ПЛФ.

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