Article
Critical Care Medicine
Cormac McCarthy, Brenna C. Carey, Bruce C. Trapnell
Summary: Autoimmune pulmonary alveolar proteinosis is a rare disease characterized by dysfunction of myeloid cells, abnormal accumulation of pulmonary surfactant, and innate immune deficiency. Research has brought this disease from obscurity to a paradigm of molecular pathogenesis-based diagnosis and treatment. The low prevalence and nonspecific symptoms make diagnosis challenging and delay accurate treatment.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Review
Immunology
Ali Ataya, Vijaya Knight, Brenna C. Carey, Elinor Lee, Elizabeth J. Tarling, Tisha Wang
Summary: The article reviews the role of GM-CSF in aPAP pathogenesis and pulmonary homeostasis, as well as the increased incidence of infections.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Immunology
Xinmei Huang, Mengshu Cao, Yonglong Xiao
Summary: Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder characterized by surfactant accumulation in the alveoli. Alveolar macrophages (AMs) play a crucial role in PAP pathogenesis, particularly in cholesterol clearance and surfactant homeostasis. Novel therapies targeting GM-CSF signaling, cholesterol metabolism, and immune modulation of AMs are being developed to address this disease.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Respiratory System
Francesca Mariani, Elena Salvaterra, Sara Lettieri, Annalisa De Silvestri, Alessandra Corino, Matteo Bosio, Elia Fraolini, Davide Piloni, Giuseppe Rodi, Angelo Guido Corsico, Ilaria Campo
Summary: This study retrospectively analyzed the efficacy of mini-whole lung lavage (WLL) compared to standard WLL for the treatment of PAP. The results showed that mini-WLL had a higher efficacy in improving lung volumes, while no significant differences were found in other indicators between the two groups.
RESPIRATORY RESEARCH
(2022)
Article
Medicine, General & Internal
Jiu-Wu Bai, Jian-nan Huang, Shen-yun Shi, Ai Ge, Hai-wen Lu, Xiao-li Sun, Shu-yi Gu, Shuo Liang, Ke-bin Cheng, Xin-lun Tian, Yong-long Xiao, Kai-feng Xu, Jin-Fu Xu
Summary: This study aimed to evaluate the effectiveness of HRCT scoring in patients with autoimmune pulmonary alveolar proteinosis (APAP). The study found that the SPSPII scoring system based on HRCT density and extent was better than DSS and SPSP in assessing disease severity and prognosis, and could predict treatment efficacy.
FRONTIERS IN MEDICINE
(2023)
Article
Biochemical Research Methods
Brenna Carey, Claudia Chalk, Jennifer Stock, Andrea Toth, Maria Klingler, Henry Greenberg, Kanji Uchida, Paritha Arumugam, Bruce C. Trapnell
Summary: This study reports the development of a method using dried blood spot cards for the measurement of GMAb concentration in blood and the diagnosis of autoimmune PAP. The method showed high accuracy, precision, reliability, sensitivity, and specificity, and performed well under various conditions.
JOURNAL OF IMMUNOLOGICAL METHODS
(2022)
Article
Medicine, General & Internal
Shixu Liu, Xiangning Cui, Kun Xia, Dandan Wang, Jing Han, Xiaoyan Yao, Xiaohong Liu, Lingjie Bian, Jinzhi Zhang, Guangxi Li
Summary: The study reveals that research on PAP is flourishing, and international cooperation is expected to deepen and strengthen in the future. Future research hotspots include the etiology and pathogenesis of PAP, current and emerging therapies, especially novel pathogenesis-based options.
FRONTIERS IN MEDICINE
(2022)
Article
Multidisciplinary Sciences
Ahmad Basim Melhem, Abdelsamea Mohammed Seif, Omar Husni Omar, Samir Al Bashir, Shaher M. Samrah
Summary: This case report describes a patient who developed severe pulmonary alveolar proteinosis (PAP) after a COVID-19 infection. PAP diagnosis can be easily missed due to the similarity of its presentation and radiological findings with COVID-19. The patient did not respond to multiple courses of antibiotics and prolonged corticosteroid therapy, but showed significant improvement after diagnostic bronchoalveolar lavage and subsequent therapeutic lung lavage.
Article
Medicine, General & Internal
Cheng-Hao Chuang, Chih-Hung Cheng, Yu-Chen Tsai, Ming-Ju Tsai, Chau-Chyun Sheu, Inn-Wen Chong
Summary: This study investigated the epidemiology of pulmonary alveolar proteinosis (PAP) in Taiwan and identified the comorbidities and prognostic factors of PAP. The study found that the incidence of PAP has been increasing over the years, and whole lung lavage (WLL) can greatly improve the survival rate of patients.
JOURNAL OF THE FORMOSAN MEDICAL ASSOCIATION
(2023)
Article
Allergy
Siyoon Kim, Min Jee Park, Kun Baek Song, Eom Ji Choi, Eun Young Paek, Dakyeong Lee, Soo-Jong Hong
Summary: This case report presents a 17-year-old female diagnosed with PAP who was treated with both GM-CSF inhalation and whole lung lavage. The treatment resulted in significant improvement of symptoms, imaging findings, and pulmonary function tests. Therefore, the combination of whole lung lavage and subsequent GM-CSF inhalation should be considered as a treatment option for children and adolescents.
ALLERGY ASTHMA & RESPIRATORY DISEASE
(2023)
Review
Respiratory System
Maitri Munsif, Duncan Sweeney, Tracy L. Leong, Rob G. Stirling
Summary: Inhaled GM-CSF treatment has shown significant improvements in symptoms, dyspnea scores, lung function, gas exchange, and radiology indices in patients with aPAP. Further research comparing the effectiveness and patient preference between whole lung lavage and nebulized GM-CSF is needed.
EUROPEAN RESPIRATORY REVIEW
(2023)
Article
Respiratory System
Ivan Oterino-Moreira, Maria-Jesus Linares-Asensio, Sira Sanz-Marquez, Montserrat Perez-Encinas
Summary: Autoimmune pulmonary alveolar proteinosis (aPAP) is caused by IgG antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). Whole lung lavage (WLL) is a complex technique to remove the lipid-proteinaceous material accumulated in the lungs. In some refractory cases, multiple WLLs are required.
CLINICAL RESPIRATORY JOURNAL
(2023)
Article
Immunology
Ananda S. Mirchandani, Stephen J. Jenkins, Calum C. Bain, Manuel A. Sanchez-Garcia, Hannah Lawson, Patricia Coelho, Fiona Murphy, David M. Griffith, Ailiang Zhang, Tyler Morrison, Tony Ly, Simone Arienti, Pranvera Sadiku, Emily R. Watts, Rebecca S. Dickinson, Leila Reyes, George Cooper, Sarah Clark, David Lewis, Van Kelly, Christos Spanos, Kathryn M. Musgrave, Liam Delaney, Isla Harper, Jonathan Scott, Nicholas J. Parkinson, Anthony J. Rostron, J. Kenneth Baillie, Sara Clohisey, Clare Pridans, Lara Campana, Philip Starkey Lewis, A. John Simpson, David H. Dockrell, Jurgen Schwarze, Nikhil Hirani, Peter J. Ratcliffe, Christopher W. Pugh, Kamil Kranc, Stuart J. Forbes, Moira K. B. Whyte, Sarah R. Walmsley
Summary: Hypoxemia is common in patients with acute respiratory distress syndrome (ARDS) and mouse models of hypoxic acute lung injury. It leads to monocytopenia and reduced accumulation of monocyte-derived macrophages, exacerbating inflammation in the lung. Intervention to rescue monocytopenia and limit injury is a potential therapeutic strategy for ARDS.
Article
Medicine, General & Internal
Gokul Paidi, Jugroop S. Brar, Andrea S. Vizcaino Duran, Jose Benero-Fossatti, Anhad Brar, Farrah P. Aziz Greye
Summary: Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterized by the accumulation of surfactant in the alveoli and bronchiolar spaces due to macrophage dysfunction. The underlying mechanisms of PAP are not fully understood, but it is believed to involve impaired clearance of surfactant and abnormal immune responses. Diagnosis of PAP involves imaging studies and bronchoscopy, and treatment options include whole-lung lavage, pharmacotherapy, and lung transplantation. We report a case of PAP in a 56-year-old female who worked in a dental office and had no prior diagnosis of lung disease.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Medicine, General & Internal
Masafumi Shimoda, Haruyuki Ishii, Yoshiaki Tanaka, Kozo Morimoto, Tamiko Takemura, Teruaki Oka, Kozo Yoshimori, Ken Ohta
Summary: A 46-year-old woman with lung cancer developed autoimmune pulmonary alveolar proteinosis (aPAP) after treatment with osimertinib, which was difficult to differentiate from drug-induced lung injury or lymphangitis based on radiological findings alone.
Article
Respiratory System
Lisa Lancaster, Francesco Bonella, Yoshikazu Inoue, Vincent Cottin, James Siddall, Mark Small, Jonathan Langley
Summary: The study evaluated the impact of idiopathic pulmonary fibrosis (IPF) on healthcare resource use, quality of life, and work impairment using real-world data, finding that patients experience delays in diagnosis and treatment, highlighting the need for more effective therapies and management to reduce the burden of the disease.
Article
Medicine, Research & Experimental
Francesco Bonella, Vincent Cottin, Claudia Valenzuela, Marlies Wijsenbeek, Florian Voss, Klaus B. Rohr, Susanne Stowasser, Toby M. Maher
Summary: This study conducted a meta-analysis of four placebo-controlled phase III trials and found that nintedanib significantly slowed down the decline in forced vital capacity (FVC) in patients with various forms of pulmonary fibrosis, with no evidence of heterogeneity in its relative treatment effect across patient populations.
ADVANCES IN THERAPY
(2022)
Article
Critical Care Medicine
Vincent Cottin, Sara Tomassetti, Claudia Valenzuela, Simon L. F. Walsh, Katerina M. Antoniou, Francesco Bonella, Kevin K. Brown, Harold R. Collard, Tamera J. Corte, Kevin R. Flaherty, Kerri A. Johannson, Martin Kolb, Michael Kreuter, Yoshikazu Inoue, R. Gisli Jenkins, Joyce S. Lee, David A. Lynch, Toby M. Maher, Fernando J. Martinez, Maria Molina-Molina, Jeff L. Myers, Steven D. Nathan, Venerino Poletti, Silvia Quadrelli, Ganesh Raghu, Sujeet K. Rajan, Claudia Ravaglia, Martine Remy-Jardin, Elisabetta Renzoni, Luca K. Richeldi, Paolo Spagnolo, Lauren Troy, Marlies Wijsenbeek, Kevin C. Wilson, Wim Wuyts, Athol U. Wells, Christopher J. Ryerson
Summary: This international working group perspective summarizes the clinical features of idiopathic pulmonary fibrosis (IPF) and proposes a probability model to facilitate the diagnosis of IPF.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Editorial Material
Respiratory System
Francesco Bonella, Raphael Borie
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Respiratory System
Michelle Terwiel, Raphael Borie, Bruno Crestani, Liam Galvin, Francesco Bonella, Aurelie Fabre, Antoine Froidure, Matthias Griese, Jan C. Grutters, Kerri Johannson, Caroline Kannengiesser, Leticia Kawano-Dourado, Maria Molina-Molina, Antje Prasse, Elisabetta A. Renzoni, Jasper van Der Smagt, Venerino Poletti, Katerina Antoniou, Coline H. M. van Moorsel
Summary: This study surveyed the experiences and needs of patients, their relatives, and pulmonologists regarding genetic testing in ILD. The results showed that there is widespread support for the implementation of genetic testing in ILD, and there is a high demand for information, guidelines, and access to testing among all groups surveyed. However, a significant number of pulmonologists currently do not offer genetic testing due to limited access to tests.
Article
Respiratory System
Kevin K. Brown, Yoshikazu Inoue, Kevin R. Flaherty, Fernando J. Martinez, Vincent Cottin, Francesco Bonella, Stefania Cerri, Sonye K. Danoff, Stephane Jouneau, Rainer-Georg Goeldner, Martin Schmidt, Susanne Stowasser, Rozsa Schlenker-Herceg, Athol U. Wells
Summary: This study analyzed clinical data from subjects with IPF and other progressive fibrosing ILDs and found that decline in forced vital capacity (FVC) is a significant predictor of mortality.
Article
Respiratory System
Theodoros Karampitsakos, Paolo Spagnolo, Nesrin Mogulkoc, Wim A. Wuyts, Sara Tomassetti, Elisabeth Bendstrup, Maria Molina-Molina, Effrosyni D. Manali, Omer Selim Unat, Francesco Bonella, Nicolas Kahn, Lykourgos Kolilekas, Elisabetta Rosi, Leonardo Gori, Claudia Ravaglia, Venerino Poletti, Zoe Daniil, Thomas Skovhus Prior, Ilias C. Papanikolaou, Samantha Aso, Stavros Tryfon, Despoina Papakosta, Vasillios Tzilas, Elisabetta Balestro, Spyridon Papiris, Katerina Antoniou, Demosthenes Bouros, Athol Wells, Michael Kreuter, Argyris Tzouvelekis
Summary: This study aimed to create a large database for patients with idiopathic pulmonary fibrosis (IPF) and lung cancer in Europe. The results showed that 10.2% of IPF patients also had lung cancer, and IPF patients with lung cancer had a higher risk of all-cause mortality. Lung cancer patients who received antifibrotic treatment had lower all-cause mortality, and surgically treated IPF patients with operable lung cancer had lower all-cause mortality.
Article
Respiratory System
Raphael Borie, Caroline Kannengiesser, Katerina Antoniou, Francesco Bonella, Bruno Crestani, Aurelie Fabre, Antoine Froidure, Liam Galvin, Matthias Griese, Jan C. Grutters, Maria Molina-Molina, Venerino Poletti, Antje Prasse, Elisabetta Renzoni, Jasper van der Smagt, Coline H. M. van Moorsel
Summary: The discovery of gene mutations that cause pulmonary fibrosis confirms the genetic predisposition to this condition. While genetic sequencing is already part of routine clinical practice for familial pulmonary fibrosis cases in some countries, it has not been widely incorporated into interstitial lung disease services and lacks international consensus.
EUROPEAN RESPIRATORY JOURNAL
(2023)
Article
Medicine, General & Internal
Lutz B. Jehn, Ulrich Costabel, Eda Boerner, Julia Waelscher, Dirk Theegarten, Christian Taube, Francesco Bonella
Summary: This study aimed to investigate whether baseline serum KL-6, alone or in combination with clinical variables, could improve stratification of ILD patients according to progression risk. The results showed that male gender and serum KL-6 levels were significant predictors of disease progression, and a simple GK score based on these variables can effectively differentiate high and low risk patients.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Pharmacology & Pharmacy
Francesco Bonella, Paolo Spagnolo, Chris Ryerson
Summary: Idiopathic pulmonary fibrosis (IPF) is a disease with poor survival and complex pathogenesis. While current drugs can slow disease progression, there is a need for new treatment strategies. Several new drugs are currently in clinical development, but our understanding of IPF is still limited.
Letter
Respiratory System
Maria Molina-Molina, Jessica Germaine Shull, Vanesa Vicens-Zygmunt, Pilar Rivera-Ortega, Katerina Antoniou, Francesco Bonella, Elisabetta Renzoni, Anne-Marie Russell, Toby M. Maher, Ada Vancheri, Anna Bachs, Victoria Aviles, Josep Palma, Guadalupe Bermudo, Guillermo Suarez-Cuartin, Cristian Tebe, Raul Rigo-Bonnin, Ana Montes-Worboys, Marlies Wijsenbeek, Carlo Vancheri
EUROPEAN RESPIRATORY JOURNAL
(2023)
Article
Medicine, General & Internal
Simone Kattner, Sivagurunathan Sutharsan, Marc Moritz Berger, Andreas Limmer, Lutz-Bernhard Jehn, Frank Herbstreit, Thorsten Brenner, Christian Taube, Francesco Bonella
Summary: Severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) infection has a variable disease course. KL-6 has the potential to be a candidate biomarker for predicting the severity and outcome of SARS-CoV-2 pneumonia.
JOURNAL OF CLINICAL MEDICINE
(2023)
Letter
Respiratory System
Spyros A. Papiris, Ilaria Campo, Francesca Mariani, Maria Kallieri, Lykourgos Kolilekas, Andriana I. Papaioannou, Efsun Gonca Chousein, Erdogan Cetinkaya, Francesco Bonella, Raphael Borie, Maria Kokosi, Thomas Pickworth, Maria Molina-Molina, Merce Gasa, Elzbieta Radzikowska, Justyna Fijolek, Stephane Jouneau, Emmanuel Gomez, Cormac McCarthy, Elisabeth Bendstrup, Wojciech J. Piotrowski, Rishi Pabary, Alice Hadchouel, Nathalie Coolen-Allou, Tiago Alfaro, Carlos Robalo Cordeiro, Elvira-Markela Antonogiannaki, Ioannis P. Tomos, Despoina Papakosta, Theodoros Kontakiotis, Panagiota Panagiotou, Konstantinos Douros, Andrea Schams, Sara Lettieri, Vassiliki Papaevangelou, Christina Kanaka-Gantenbein, Anna Karakatsani, Stelios Loukides, Ulrich Costabel, Bruno Crestani, Cliff Morgan, Ryushi Tazawa, Andrew Bush, Matthias Griese, Effrosyni D. Manali
Meeting Abstract
Respiratory System
S. Papiris, I Campo, F. Mariani, M. Kallieri, L. Kolilekas, A. Papaioannou, E. G. Chousein, E. Cetinkaya, F. Bonella, R. Borie, M. Kokosi, T. Pickworth, M. Molina-Molina, M. Gasa, E. Radzikowska, J. Fijolek, S. Jouneau, E. Gomez, C. Mccarthy, E. Bendstrup, W. J. Piotrowski, R. Pabary, A. Hadchouel, N. Coolen-Allou, T. Alfaro, C. R. Cordeiro, E. Antonogiannaki, I. P. Tomos, D. Papakosta, T. Kontakiotis, P. Panagiotou, K. Douros, A. Schams, S. Lettieri, V Papaevangelou, C. Kanaka-Gantenbein, A. Karakatsani, S. Loukides, U. Costabel, B. Crestani, C. Morgan, R. Tazawa, A. Bush, M. Griese, E. D. Manali
EUROPEAN RESPIRATORY JOURNAL
(2022)
Review
Respiratory System
Marlies S. Wijsenbeek, Francesco Bonella, Leticia Orsatti, Anne-Marie Russell, Claudia Valenzuela, Wim A. Wuyts, Walter F. Baile
Summary: Communicating with patients with idiopathic pulmonary fibrosis (IPF) can be challenging, but clinicians should provide individualized information and support based on empathy and understanding of the patient's needs and concerns. Various tools and protocols are available to assist clinicians in improving their interactions with patients.