There have been important advancements in the field of interstitial lung disease (ILD) over the past decade. Our understanding of risk factors, pathobiology, and behavioral phenotypes has guided the diagnostic evaluation, while the identification of effective therapies and a deeper valuation of the patient experience continue to shape clinical care delivery. From preclinical disease to regenerative therapies, there is much to reflect upon and to synthesize. For this Clinics in Chest Medicine series, we have invited an international and diverse group of experts to share their discoveries and insights into a broad range of ILD topics. These insights are both practical and philosophical, offering novel frameworks for disease classification and biology. Overall, each article highlights recent developments, summarizes the current evidence, and outlines ongoing areas of uncertainty in key areas.
To contextualize our current ILD paradigm, Drs Guler and Corte introduce this series with a historical overview of ILD. From early clinical and histopathological descriptions to modern-day approaches, they lay the foundation for our current understanding of pulmonary fibrosis. Drs Podolanczuk and Putman introduce the concepts of “preclinical ILD” and interstitial lung abnormalities, an important and evolving area, as chest imaging is increasingly used, and at-risk individuals are screened for the presence of lung fibrosis. As our knowledge about ILD advances, so do our conceptual frameworks. In this context, Drs Adegunsoye and Ryerson present a clinically relevant approach to ILD that incorporates the latest advancements in disease behavior, etiopathogenesis, and morphology.
The subsequent 3 articles focus on idiopathic pulmonary fibrosis (IPF), starting with Drs Oldham and Vancheri summarizing the current understanding of IPF and how recent developments should guide the diagnostic evaluation. Drs Salisbury and Wijsenbeek follow this up, presenting a comprehensive approach to clinical care and management of patients with IPF. Drs Farrand and Limper identify the challenges that exist in our reliance on traditional randomized clinical trials to identify effective IPF therapeutics. They outline how health systems can contribute to and promote research in IPF, linking knowledge generation to evidence, and ultimately, to clinical practice. Addressing other important forms of ILD, Drs Kawano-Dourado and Lee provide an overview of connective tissue disease–associated ILD, with current data on epidemiology, risk factors, and treatment approaches. Drs Barnes and Johannson summarize the current approach to management of fibrotic hypersensitivity pneumonitis, an entity increasingly recognized as prevalent, and often, progressive. Drs Collins and Luppi provide an overview of the management of patients with non-IPF fibrotic ILD, incorporating data from recent clinical trials supporting new indications for antifibrotic therapy.
The next 4 articles focus on broad approaches to clinical care and scientific discovery in ILD. In their article, Drs Wong and Danoff define patient-centered care, highlight its critical importance, and provide frameworks for its implementation into research and clinical care. Drs Fisher and Cottin discuss care delivery models and the importance of specialized ILD centers to provide accurate diagnoses, gold-standard management, education, and collaborative shared-care models for patients with pulmonary fibrosis. They further explore how technological advancements can be leveraged to improve access to specialty ILD centers. Drs Newton and Herzog present a state-of-the-art overview of omics-based discoveries, presenting a roadmap toward precision-based approaches to disease management. Finally, Drs Lehmann and Königshoff round out this series with the latest developments in our understanding of pulmonary fibrosis, and the hope for a cure through antiaging and regenerative therapeutic approaches.
In the final article, we summarize the current and future state of ILD management with an overview of short-term priorities for action and longer-term areas for discovery and innovation. All together, we hope that this Clinics in Chest Medicine series engages readers with a passion for ILD clinical care and research. The developments seen in recent years are stepping stones for further advancements in the field, toward goals of early disease identification, efficient provision of patient-centered care, and ultimately, curing lung fibrosis.
© 2021 Published by Elsevier Inc.