Review
Biochemistry & Molecular Biology
Carlo Albera, Giulia Verri, Federico Sciarrone, Elena Sitia, Mauro Mangiapia, Paolo Solidoro
Summary: Interstitial lung diseases (ILDs) encompass a diverse group of rare and chronic respiratory disorders, with idiopathic pulmonary fibrosis (IPF) being the most studied member. Recent development of antifibrotic drugs has shown benefits in ILDs characterized by progressive fibrosis, leading to significant changes in management.
Article
Pharmacology & Pharmacy
Alexandra Nagy, Tamas Nagy, Abigel Margit Kolonics-Farkas, Noemi Eszes, Krisztina Vincze, Eniko Barczi, Adam Domonkos Tarnoki, David Laszlo Tarnoki, Gyorgy Nagy, Emese Kiss, Pal Maurovich-Horvat, Aniko Bohacs, Veronika Muller
Summary: This study evaluated the clinical characteristics and predictors of lung function changes in autoimmune PF-ILD patients. Older age and more severe clinical symptoms were observed in the IPAF group. Antifibrotic therapy had a positive impact on long-term lung function improvement in PF-ILD patients.
FRONTIERS IN PHARMACOLOGY
(2021)
Review
Medicine, General & Internal
Carla R. Copeland, Lisa H. Lancaster
Summary: Progressive fibrosing interstitial lung diseases (PF-ILD) is a diverse group of interstitial lung diseases characterized by accelerated respiratory failure, frequent disease exacerbation, and earlier mortality. Available therapeutic approaches include antifibrotic therapy, immunosuppression, novel therapies, and lung transplantation.
FRONTIERS IN MEDICINE
(2021)
Review
Medicine, General & Internal
Ahmad Abu Qubo, K. M. Capaccione, Elana J. Bernstein, Maria Padilla, Mary Salvatore
Summary: This article describes the role of radiology in the diagnosis and follow-up of PF-ILD. The manuscript reviews the radiographic diagnosis and unique CT characteristics associated with specific types of fibrosis. Radiology has the potential to make an important contribution to the multidisciplinary discussion for this important diagnosis.
FRONTIERS IN MEDICINE
(2022)
Review
Medicine, General & Internal
John N. Shumar, Abhimanyu Chandel, Christopher S. King
Summary: Progressive fibrosing interstitial lung disease (PF-ILD) is a subset of interstitial lung diseases characterized by progressive and intractable lung fibrosis, with similarities to idiopathic pulmonary fibrosis. Recent studies have shown the clinical efficacy of antifibrotic therapy in PF-ILD, changing the treatment landscape for fibrotic lung diseases and opening the door for combined therapies targeting both fibrotic and inflammatory components.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Respiratory System
Byoung Soo Kwon, Jooae Choe, Eun Jin Chae, Hee Sang Hwang, Yong-Gil Kim, Jin Woo Song
Summary: Approximately 34% of non-IPF fibrosing ILD patients showed a progressive PF phenotype with poor prognosis, similar to that of IPF. The PF phenotype was identified as an independent prognostic factor in patients with fibrosing ILD.
RESPIRATORY RESEARCH
(2021)
Review
Medicine, General & Internal
Willis S. Bowman, Gabrielle A. Echt, Justin M. Oldham
Summary: Interstitial lung disease (ILD) is a group of diverse lung disorders that can lead to irreversible pulmonary fibrosis. While idiopathic pulmonary fibrosis (IPF) is a common subtype, other ILD patients may develop a progressive fibrosing phenotype. Biomarker investigation has identified molecular markers that can predict disease endpoints, suggesting potential for biomarker implementation in ILD management.
FRONTIERS IN MEDICINE
(2021)
Article
Medicine, General & Internal
Hertzel C. Gerstein, Naveed Sattar, Julio Rosenstock, Chinthanie Ramasundarahettige, Richard Pratley, Renato D. Lopes, Carolyn S. P. Lam, Nardev S. Khurmi, Laura Heenan, Stefano Del Prato, Leanne Dyal, Kelley Branch
Summary: The trial compared the cardiovascular and renal outcomes of weekly injections of efpeglenatide versus a placebo in participants with type 2 diabetes and a history of cardiovascular disease or current kidney disease. The study found that the risk of cardiovascular events was lower with efpeglenatide treatment, and a composite renal outcome event was also reduced.
NEW ENGLAND JOURNAL OF MEDICINE
(2021)
Article
Medicine, General & Internal
Anna Denis, Monique Henket, Marie Ernst, Nathalie Maes, Marie Thys, Celine Regnier, Olivier Malaise, Anne-Noelle Frix, Fanny Gester, Colin Desir, Paul Meunier, Renaud Louis, Michel Malaise, Julien Guiot
Summary: Rheumatoid arthritis associated-interstitial lung disease (RA-ILD) is the most common pulmonary manifestation of rheumatoid arthritis (RA) and a significant cause of mortality. A subset of patients with RA-ILD exhibit a progressive fibrotic phenotype, known as progressive fibrosing interstitial lung disease (PF-ILD). This study retrospectively investigated the frequency of RA-ILD and PF-ILD in a cohort of RA patients, comparing their characteristics and outcomes. The results showed that RA-ILD and PF-ILD patients were older, had a higher proportion of men, and a higher prevalence of smoking history. Non-specific interstitial pneumonia pattern was more common in both RA-ILD and PF-ILD groups. The risk of death was twice as high in RA-ILD patients compared to RA patients.
FRONTIERS IN MEDICINE
(2022)
Article
Pharmacology & Pharmacy
Xianqiu Chen, Jian Guo, Dong Yu, Bing Jie, Ying Zhou
Summary: This study assessed clinical characteristics and outcomes of PF-ILD patients in a single-center cohort, finding that extent of fibrotic changes on HRCT and pulmonary hypertension were predictors of mortality in patients with PF-ILD.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Endocrinology & Metabolism
Qinghua Zhang, Yan Wang, Chang Tian, Jinyan Yu, Yanlei Li, Junling Yang
Summary: This study investigated the genetic contributions to the development of type 2 diabetes mellitus (T2DM) complicated with interstitial lung disease (ILD). Whole genome re-sequencing was performed on a pedigree with T2DM complicated with ILD, and the MUC5B SNP rs2943512 (A > C) was identified as a potentially important genetic variant. In vitro experiments showed that up-regulated MUC5B, induced by high glucose, activated ERK1/2 and increased IL-1 beta and IL-6. Silencing MUC5B decreased the levels of p-ERK1/2, IL-1 beta, and IL-6.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Article
Medicine, General & Internal
Masamichi Komatsu, Hiroshi Yamamoto, Yoshiaki Kitaguchi, Satoshi Kawakami, Mina Matsushita, Takeshi Uehara, Takumi Kinjo, Yosuke Wada, Takashi Ichiyama, Kazuhisa Urushihata, Atsuhito Ushiki, Masanori Yasuo, Masayuki Hanaoka
Summary: Progressive fibrosing interstitial lung disease (PF-ILD) is a type of fibrosing ILD with unclear clinical characteristics and poor prognosis. Patients with PF-ILD require more intensive treatment and have a higher mortality rate, similar to that of patients with idiopathic pulmonary fibrosis.
Article
Urology & Nephrology
Yu-Lun Ou, Mei-Yueh Lee, I-Ting Lin, Wei-Lun Wen, Wei-Hao Hsu, Szu-Chia Chen
Summary: This study found significant associations between various obesity-related indices and albuminuria as well as advanced kidney disease in patients with type 2 diabetes mellitus. Screening for these indices may be important for public health programs to prevent complications related to kidney disease.
Article
Rheumatology
Andreina Manfredi, Caterina Vacchi, Giovanni DellaCasa, Stefania Cerri, Giulia Cassone, Giovanna Di Cecco, Fabrizio Luppi, Carlo Salvarani, Marco Sebastiani
Summary: This study investigated the prevalence of fibrosing patterns in pSS patients with ILD and found that over half of the patients had a fibrotic pattern, which tended to occur in younger patients with shorter pSS duration compared to those with nonfibrotic patterns.
Article
Medicine, General & Internal
Stefan Cristian Stanel, Pilar Rivera-Ortega
Summary: Progressive fibrosing interstitial lung diseases (PF-ILDs) are a group of diseases that worsen despite standard treatments, leading to respiratory failure and early mortality. Early diagnosis and monitoring can be improved through standardized multidisciplinary team discussions, machine learning algorithms, blood biomarkers, genetic testing, and home monitoring using wearable devices. Significant changes to clinical practice for PF-ILDs can be expected in the near future.
FRONTIERS IN MEDICINE
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Lena Wucherpfennig, Simon M. F. Triphan, Oliver Weinheimer, Monika Eichinger, Sabine Wege, Ralf Eberhardt, Michael U. Puderbach, Hans-Ulrich Kauczor, Claus P. Heussel, Gudula Heussel, Mark O. Wielpuetz
Summary: The study demonstrates that MRA provides high-quality diagnostic images in patients with COPD and CF, and its image quality and reproducibility are not dependent on disease severity.
Article
Respiratory System
Patricia Leutz-Schmidt, Daiva-Elzbieta Optazaite, Olaf Sommerburg, Monika Eichinger, Sabine Wege, Eva Steinke, Simon Y. Graeber, Michael U. Puderbach, Jens-Peter Schenk, Abdulsattar Alrajab, Simon M. F. Triphan, Hans-Ulrich Kauczor, Mirjam Stahl, Marcus A. Mall, Mark O. Wielputz
Summary: This study evaluated the occurrence of bronchial artery dilatation (BAD) in patients with cystic fibrosis (CF) and its association with disease severity using magnetic resonance imaging (MRI). The results showed that the onset of BAD was associated with disease severity, lung function, and chronic Pseudomonas aeruginosa infection. This finding is important for assessing the severity of CF.
Article
Multidisciplinary Sciences
Jonas Albers, Willi L. Wagner, Mascha O. Fiedler, Anne Rothermel, Felix Wuennemann, Francesca Di Lillo, Diego Dreossi, Nicola Sodini, Elisa Baratella, Marco Confalonieri, Fulvia Arfelli, Armin Kalenka, Joachim Lotz, Juergen Biederer, Mark O. Wielpuetz, Hans-Ulrich Kauczor, Frauke Alves, Giuliana Tromba, Christian Dullin
Summary: Clinical computed tomography (CT) is the preferred imaging method for diagnosing lung diseases, but it has limitations in resolving microscopic structures. Propagation-based imaging (PBI), a phase-sensitive X-ray imaging technique, offers high-resolution imaging at lower radiation doses. In this study, technical refinements of PBI were presented for characterizing lung pathologies, demonstrating improved image quality compared to clinical CT. The combination of PBI and iodine k-edge subtraction imaging showed potential for analyzing lung perfusion in detail.
SCIENTIFIC REPORTS
(2023)
Article
Pharmacology & Pharmacy
Lena Wucherpfennig, Felix Wuennemann, Monika Eichinger, Angelika Seitz, Ingo Baumann, Mirjam Stahl, Simon Y. Graeber, Shengkai Zhao, Jaehi Chung, Jens-Peter Schenk, Abdulsattar Alrajab, Hans-Ulrich Kauczor, Marcus A. Mall, Olaf Sommerburg, Mark O. Wielpuetz
Summary: Chronic rhinosinusitis (CRS) in cystic fibrosis (CF) can cause nasal congestion, rhinorrhea, and anosmia, affecting the quality of life. Previous studies using MRI showed early onset and progression of CRS in CF patients from infancy to school age, as well as mid-term improvements in preschool and school-age CF children with lumacaftor/ivacaftor treatment. However, there is a lack of long-term data on the treatment effects on paranasal sinus abnormalities in CF children.
FRONTIERS IN PHARMACOLOGY
(2023)
Editorial Material
Critical Care Medicine
Mark O. Wielpuetz
Letter
Respiratory System
Katie J. Bayfield, Oliver Weinheimer, Christie Boyton, Rachel Fitzpatrick, Anna Middleton, Brendan Kennedy, Anneliese Blaxland, Geshani Jayasuriya, Neil Caplain, Hana Issa, Robert Goetti, Mark O. Wielpuetz, Lifeng Yu, Craig J. Galban, Terry E. Robinson, Brian Bartholmai, Dominic Fitzgerald, Hiran Selvadurai, Paul D. Robinson
EUROPEAN RESPIRATORY JOURNAL
(2023)
Review
Radiology, Nuclear Medicine & Medical Imaging
Simon M. F. Triphan, Grzegorz Bauman, Philip O. Konietzke, Marilisa Konietzke, Mark Wielpuetz
Summary: Lung perfusion, an essential process for oxygenation, is regulated by hypoxic pulmonary vasoconstriction (HPV) in response to obstructive airway diseases. Various MRI techniques have been developed to visualize lung perfusion, and dynamic-contrast enhanced MRI is widely used in clinical assessment. Non-contrast techniques are still being researched and validated. Preliminary data on these techniques in evaluating therapy effects are emerging. Level of Evidence 5, Technical Efficacy Stage 5.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Article
Pharmacology & Pharmacy
Sabine Dettmer, Oliver Weinheimer, Annette Sauer-Heilborn, Oliver Lammers, Mark O. Wielpuetz, Jan Fuge, Tobias Welte, Frank Wacker, Felix C. Ringshausen
Summary: This study assesses the computed tomography (CT) changes under ETI treatment in cystic fibrosis patients and finds that both the visual and quantitative CT parameters can effectively evaluate the treatment response. The CT changes correlate with clinical improvements.
FRONTIERS IN PHARMACOLOGY
(2023)
Article
Medicine, General & Internal
Bedair Dewidar, Lucia Mastrototaro, Cornelia Englisch, Claudia Ress, Cesare Granata, Elisabeth Rohbeck, Dominik Pesta, Geronimo Heilmann, Martin Wolkersdorfer, Irene Esposito, Michelle Reina Do Fundo, Fariba Zivehe, Aslihan Yavas, Michael Roden
Summary: This study aimed to investigate the development of non-alcoholic fatty liver (NAFLD) and the mechanisms related to obesity or diabetes. Findings showed that NAFLD exhibited higher steatosis and NAFLD activity at W8 and liver fibrosis at W16. Insulin sensitivity was significantly lower in diabetes, NAFLD, and obesity models. Hepatic mitochondria adapted to metabolic challenges by increasing fatty acid oxidation-driven respiration, which was linked to dysfunctional unfolded protein response, systemic oxidative stress, insulin resistance, and altered lipid metabolism.
Article
Nutrition & Dietetics
Titiaan E. Post, Jan Schmitz, Cayla Denney, Riccardo De Gioannis, Henning Weis, Dominik Pesta, Andreas Peter, Andreas L. Birkenfeld, Sven Haufe, Uwe Tegtbur, Petra Frings-Meuthen, Ann C. Ewald, Daniel Aeschbach, Jens Jordan
Summary: This study aimed to investigate whether ingesting fructose could improve endurance and cognitive performance in humans under hypoxic conditions. The results showed that fructose intake did not acutely enhance exercise and cognitive performance during moderate hypoxia. Therefore, it is difficult for humans to replicate the hypoxia tolerance seen in naked mole rats through fructose metabolism.
FRONTIERS IN NUTRITION
(2023)
Article
Respiratory System
Mirjam Stahl, Jobst Roehmel, Monika Eichinger, Felix Doellinger, Lutz Naehrlich, Matthias V. Kopp, Anna-Maria Dittrich, Christopher Lee, Olaf Sommerburg, Simon Tian, Tu Xu, Pan Wu, Aniket Joshi, Partha Ray, Margaret E. Duncan, Mark O. Wielputz, Marcus A. Mall
Summary: This study investigated the effects of LUM/IVA treatment on disease progression in children aged 2 to 5 years with CF. Results showed potential early disease modification with LUM/IVA, as indicated by improvements in chest MRI and various clinical markers. LUM/IVA treatment also led to improvements in lung function and growth parameters.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Article
Respiratory System
Lena Wucherpfennig, Felix Wuennemann, Monika Eichinger, Niclas Schmitt, Angelika Seitz, Ingo Baumann, Mirjam Stahl, Simon Y. Graeber, Jaehi Chung, Jens-Peter Schenk, Abdulsattar Alrajab, Hans-Ulrich Kauczor, Marcus A. Mall, Olaf Sommerburg, Mark O. Wielputz
Summary: This study longitudinally evaluated chronic rhinosinusitis (CRS) in children with cystic fibrosis (CF) using paranasal sinus magnetic resonance imaging (MRI). The results showed that CRS starts early in infancy and progresses in severity until school age. The study also found that lumacaftor/ivacaftor therapy can improve CRS in children with CF.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Article
Pediatrics
Lena Maria Roewer, Karl Ludger Radke, Janina Hussmann, Halima Malik, Monika Eichinger, Dirk Voit, Mark Oliver Wielpuetz, Jens Frahm, Dirk Klee, Frank Pillekamp
Summary: In pediatric patients with CHD and chronic RV volume overload, real-time cardiac MRI during free breathing reveals increased RV end-diastolic volume and stroke volume during inspiration, with a lower increase in ejection fraction and a reduced Frank-Starling relationship of the RV compared to controls.
PEDIATRIC RADIOLOGY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Qian Li, Lin Zhu, Oyunbileg von Stackelberg, Simon M. F. Triphan, Juergen Biederer, Oliver Weinheimer, Monika Eichinger, Claus F. Vogelmeier, Rudolf A. Joerres, Hans -Ulrich Kauczor, Claus P. Heussel, Bertram J. Jobst, Mark O. Wielpuetz
Summary: This study investigated the use of morphofunctional chest MRI for the detection and management of incidental pulmonary nodules in patients with chronic obstructive pulmonary disease (COPD). The results showed that the sensitivity of this technique in detecting pulmonary nodules was moderate, but it had high agreement with LDCT in terms of Lung-RADS classification.
RADIOLOGY-CARDIOTHORACIC IMAGING
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Alan A. Peters, Oliver Weinheimer, Oyunbileg von Stackelberg, Jonas Kroschke, Lars Piskorski, Manuel Debic, Kai Schlamp, Linn Welzel, Moritz Pohl, Andreas Christe, Lukas Ebner, Hans-Ulrich Kauczor, Claus Peter Heussel, Mark O. Wielpuetz
Summary: Quantitative computed tomography (QCT) of the whole lung and nodule-bearing lobe can be valuable in assessing the malignancy risk of pulmonary nodules. The study shows that nodules in subjects with higher emphysema levels and less fibrosis in the lungs are more likely to be malignant.
EUROPEAN RADIOLOGY
(2023)