Интерстициальные заболевания легких и аутоиммунитет

Поражение легких относится к числу частых системных проявлений системных аутоиммунных ревматических заболеваний (САРЗ), при которых в патологический процесс вовлекаются все компоненты респираторной системы. Особенно важное место занимают интерстициальные заболевания легких (ИЗЛ), нередко приводящие к развитию прогрессирующего легочного фиброза (ПЛФ). В рамках ИЗЛ, асоциированных с САРЗ, условно выделяются следующие клинические категории пациентов: ИЗЛ у пациентов с достоверным диагнозом САРЗ; ИЗЛ как первое проявление САРЗ; ИЗЛ или интерстициальные пневмонии с аутоимунными признаками. Клинические фенотипы ИЗЛ при САРЗ (ИЗЛ-САРЗ) варьируют от «бессиптомных» до «быстро прогрессирующих», в разной степени связанных с факторами риска поражения легких, что следует учитывать при проведении целенаправленного клинико-лабораторного и инструментального скрининга и назначении противовоспалительной и антифиброзной терапии. В практике пульмонолога пациент с ИЗЛ может иметь уже установленное ранее САРЗ, либо этот диагноз может быть заподозрен на основании ряда клинических симптомов, характерных для ревматических заболеваний. Диагностика ИЗЛ, ассоциированных с САРЗ, представляет сложную проблему, что диктует необходимость мультидисциплинарного подхода, основанного на взаимодействии ревматологов, пульмонологов и рентгенологов. Возможности и перспективы фармакотерапии ИЗЛ-САРЗ основаны на рациональном использовании противовоспалительных, иммуномодулирующих и антифиброзных препаратов. Следует акцентировать внимание на следующих основных проблемах, связанных с фармакотерапией ИЗЛ-САРЗ: выделение пациентов с быстро прогрессирующим в отношении легочного фиброза фенотипом заболевания; вклад активности воспаления; эффективность терапии в отношении ведущих «внелегочных» проявлений САРЗ с одной стороны и токсичности в отношении легких – с другой в рамках реализации концепции «лечение до достижения цели». Крупнейшее достижение фармакотерапии аутоиммунных болезней связано с применением CAR (chimeric antigen receptor) Т-клеточной терапии, механизм действия которой связан с элиминацией патогенных аутореактивных В-клеток. Предварительные данные свидетельствуют о ее высокой эффективности в отношении широкого спектра клинических проявлений при САРЗ, в том числе при прогрессировании ИЗЛ у пациентов с системной склеродермией и антисинтетазным синдромом, и являются веским доказательством важной роли аутоиммунных механизмов в патогенезе ИЗЛ.

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