Updates on Interstitial Lung Disease

Dear Colleagues,

Interstitial lung disease is an important cause of end-stage lung disease and is the most common indication for lung transplantation. Accurate, early diagnosis and the initiation of appropiate treatment remain essential. Recent advances in the diagnosis and treatment of ILD are promising. For the diagnosis of ILD, lung biopsy techniques, radiology, and biomarkers have aided in a less invasive and earlier diagnosis. Treatment for ILD remains focused on slowing the progression of fibrotic disease and immune modulation for inflammatory ILD. Studies targeting several pathways and ongoing clinical trials have yielded promising early results. Updated guidelines for the treatment of fibrotic ILD and CTD-ILD have been recently published. Thus, in this Special Issue, we would like to focus on recent updates in the diagnosis and management of ILD, with invited review articles from leaders in the field.

Prof. Dr. Atul C. Mehta
Dr. Sameep Sehgal
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

• interstitial lung disease
• idiopathic pulmonary fibrosis
• lung transplantation
• connective tissue disease
• bronchoscopy

Title: Review of the Diagnosis and Management of PH-ILD
Authors: /
Affiliation: /
Abstract: Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is characterized by the presence of precapillary pulmonary hypertension (PH) in patients with underlying ILD. PH-ILD is suggested by the presence of symptoms out of proportion to the extent of the ILD. There are other clues to the presence of PH in the context of ILD including the need for supplemental oxygen, a reduced DLCO especially if accompanied by an elevated FVC%/DLCO% predicted ratio, radiology demonstrating an enlarged pulmonary artery or a dilated right ventricle, a reduced six minute walk distance, or cardiopulmonary exercise testing with evidence of an exhausted circulatory reserve. Echocardiography is the best screening tool but right heart catheterization remains the gold standard for the diagnosis of PH. When appropriate, treatment with inhaled treprostinil, or possibly pulmonary vasodilators such as phosphodiesterase 5 inhibitors and/or intravenous or subcutaneous prostanoids may be indicated.

Title: What's on the Horizon for Treatments in Idiopathic Pulmonary Fibrosis
Authors: Teng Moua; Misbah Baqir; Jay H Ryu
Affiliation: Mayo Clinic
Abstract: Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal lung disease most commonly encountered in older individuals. Several decades of research have contributed to a better understanding of its pathogenesis though only two drugs thus far have shown treatment efficacy, i.e., slowing of lung function decline. The pathogenesis of IPF remains incompletely understood and involves multiple complex interactions and mechanisms working in tandem or separately to result in unchecked deposition of extracellular matrix components and collagen characteristic of the disease. These mechanisms include aberrant response to injury in the alveolar epithelium, inappropriate communication between epithelial cells and mesenchymal cells, imbalances between oxidative injury and tissue repair, recruitment of inflammatory pathways that induce fibrosis, and cell senescence leading to sustained activation and proliferation of fibroblasts and myofibroblasts. Targeted approaches to each of these mechanistic pathways have led to recent clinical studies evaluating the safety and efficacy of several agents. This review highlights selected concepts in the pathogenesis of IPF as a rationale for understanding current or future therapeutic approaches, followed by a review of several selected agents and their recent or active clinical studies.

Title: Management of Interstitial Lung Disease in the ICU: A Comprehensive Review
Authors: Zehra Dhanani; Rohit Gupta
Affiliation: Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine, Temple University Hospital, Philadelphia, PA
Abstract: Interstitial lung disease encompasses a diverse group of parenchymal lung diseases characterized by varying degrees of inflammation and/or fibrosis. Patients with ILD frequently require hospitalization, with many needing intensive care unit (ICU) admission, most often due to respiratory failure. The diagnosis and management of ILD in ICU present unique challenges. Diagnosis primarily relies on chest CT imaging to identify fibrosis and inflammation. Acute exacerbations, whether in idiopathic pulmonary fibrosis (IPF) or non-IPF ILD, require careful evaluation of potential triggers and differential diagnoses. Bronchoalveolar lavage may provide valuable information, such as identification of infections, but carries risks of complications. Biopsies, whether transbronchial or surgical, can also be informative but pose significant procedural risks. Corticosteroids are the cornerstone of treatment for acute exacerbations of IPF, with higher doses potentially benefiting non-IPF ILD. Additional immunosuppressive agents may be used in cases with evidence of inflammation. Oxygen supplementation, particularly with high-flow nasal cannula, is often employed to manage severe hypoxemia, while noninvasive ventilation can be useful for worsening hypoxemia and/or hypercapnia. When mechanical ventilation is used, it is recommended to target low tidal volumes to minimize lung injury; high PEEP may be less effective and even associated with increased mortality. Prone positioning can improve oxygenation in severely hypoxemic patients. In addition to ventilatory strategies, careful fluid management and addressing concomitant pulmonary hypertension are essential components of care. Extracorporeal membrane oxygenation is a high-risk intervention reserved for the most severe cases. Lung transplantation may be considered for end-stage ILD patients in the ICU, with outcomes dependent on urgency of transplant and patient’s overall condition. Managing ILD in the ICU requires a multidisciplinary approach, and despite recent advances, mortality remains high, emphasizing the need for continued research and individualized treatment strategies.

Title: Interstitial lung abnormalities
Authors: /
Affiliation: /
Abstract: /