Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Liu, Gabrielle Y; Budinger, G. R. Scott; Dematte, Jane

doi:10.1136/bmj-2021-066354

Cited by 29 publications

(24 citation statements)

References 164 publications

(337 reference statements)

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“…Концепция объединения нескольких ИЗЛ, не связанных с ИЛФ, в ПЛФ частично возникла из неудовлетворенности существовавшими вариантами лечения этих заболеваний [17]. У многих пациентов с фиброзирующим ИЗЛ заболевание прогрессирует, несмотря на традиционное лечение.…”

Section: основные направления леченияunclassified

“…Представление о патофизиологическом континууме ИЛФ определило два основных направления антифибротической терапии. Антифибротические средства, которые замедляют прогрессирование заболевания при ИЛФ, могут также замедлять прогрессирование и при ПЛФ [17]. Одно из таких антифибротических средств, пирфенидон, является пероральным средством с противовоспалительным, антиоксидантным и антипролиферативным действием, обусловленным подавлением трансформирующего фактора роста бета (ТФР-β) [18].…”

Section: основные направления леченияunclassified

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Prognostic biomarkers of progressive pulmonary fibrosis in patients with interstitial lung diseases

2023

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A high proportion of patients with various interstitial lung diseases (ILD) develop progressive pulmonary fibrosis (PLF) associated with a deterioration in the quality of life, a decrease in functional status and early mortality. PLF is diagnosed in patients with ILD of known or unknown etiology other than idiopathic pulmonary fibrosis (ILF), who have radiological signs of pulmonary fibrosis, and at least two of the three criteria for progression, including clinical, functional and radiological indicators that have arisen over the past year in the absence of alternative causes of deterioration. Subtypes of ILD that are at risk of developing a progressive fibrosing phenotype include ILD associated with connective tissue diseases, such as rheumatoid arthritis and systemic scleroderma; sarcoidosis; hypersensitive pneumonitis, occupational lung diseases. In 2022, new criteria for the diagnosis of PLF appeared. The existing evidence of common pathogenesis mechanisms leading to progressive pulmonary fibrosis suggests the presence of uniform biomarkers of disease activity for a wide range of diseases. Pulmonary fibrosis is initiated by microinjuries of the alveolar epithelium of various etiologies, in the future its progression includes similar pathophysiological mechanisms, which suggests the presence of uniform biomarkers of disease activity for a wide range of diseases. Biomarkers can help not only identify patients at risk of progression, but also allow monitoring the early response to treatment. Biomarker research to date has identified a number of molecular markers that predict the presence of the disease, prognosis and/or response to treatment. The creation of national and international registries allows for long-term monitoring of patients with PLF, to answer questions about the long-term effectiveness of treatment. To date, a number of molecular factors that claim to be predictors are being discussed, allowing to verify the diagnosis, determine the prognosis and response to therapy.

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Section: основные направления леченияunclassified

Prognostic biomarkers of progressive pulmonary fibrosis in patients with interstitial lung diseases

2023

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“…Специалисты по имидж-диагностике предложили новые термины. В клинических рекомендациях Европейского респираторного общества и Американского торакального общества 2018 г. предложено выделять 4 категории паттернов ОИП: определенный, вероятный, неопределенный и альтернативный [2]. Они же сохранены с незначительными уточнениями и в новой редакции клинических рекомендаций 2022 г.…”

unclassified

“…А теперь вернемся к упоминавшимся заболеваниям, исходом которых также является КТ-паттерн ОИП. В последние годы европейские пульмонологи в публикациях используют термин «прогрессирующий легочный фиброз», что является косвенным подтверждением факта исхода целого ряда заболеваний в паттерн ОИП [2]. Можно долго проводить дифференциальную диагностику и наблюдать (что врачи особенно любят, называя этот процесс «динамическим наблюдением») больного с прогрессирующим фиброзом легких и не иметь никакой возможности ему помочь, так как до недавнего времени государство обеспечивало бесплатными для пациентов, но дорогостоящими АФП только при ИЛФ.…”

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Progressive fibrosing lung disease. Discussion aspects of the problem: A review

Ilkovich

Novikova

Speranskaya

et al. 2023

Terapevticheskii arkhiv

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Abstract The authors of the article prove the need to include a new name for the disease "Progressive Fibrosing Lung Disease" into clinical practice. Recognition of the fact that some lung diseases end in a fibrosing process, which does not have any significant differences depending on the initial disease that led to fibrosis, will expand the indications for earlier prescription of antifibrotic drugs, which will undoubtedly improve the prognosis in this extremely severe category of patients.

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Lung regeneration: diverse cell types and the therapeutic potential

Chen,

Li,

et al. 2024

MedComm

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Lung tissue has a certain regenerative ability and triggers repair procedures after injury. Under controllable conditions, lung tissue can restore normal structure and function. Disruptions in this process can lead to respiratory system failure and even death, causing substantial medical burden. The main types of respiratory diseases are chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), and acute respiratory distress syndrome (ARDS). Multiple cells, such as lung epithelial cells, endothelial cells, fibroblasts, and immune cells, are involved in regulating the repair process after lung injury. Although the mechanism that regulates the process of lung repair has not been fully elucidated, clinical trials targeting different cells and signaling pathways have achieved some therapeutic effects in different respiratory diseases. In this review, we provide an overview of the cell type involved in the process of lung regeneration and repair, research models, and summarize molecular mechanisms involved in the regulation of lung regeneration and fibrosis. Moreover, we discuss the current clinical trials of stem cell therapy and pharmacological strategies for COPD, IPF, and ARDS treatment. This review provides a reference for further research on the molecular and cellular mechanisms of lung regeneration, drug development, and clinical trials.

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Advances in the management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

Cited by 29 publications

References 164 publications

Prognostic biomarkers of progressive pulmonary fibrosis in patients with interstitial lung diseases

Prognostic biomarkers of progressive pulmonary fibrosis in patients with interstitial lung diseases

Progressive fibrosing lung disease. Discussion aspects of the problem: A review

Lung regeneration: diverse cell types and the therapeutic potential

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