Article
Cardiac & Cardiovascular Systems
Dilber Ademhan Tural, Nagehan Emiralioglu, Beste Ozsezen, Birce Sunman, Halime Nayir Buyuksahin, Ismail Guzelkas, Berna Oguz, Yelda Bilginer, Diclehan Orhan, Ebru Yalcin, Deniz Dogru, Ugur Ozcelik, Seza Ozen, Nural Kiper
Summary: This study evaluated 17 ILD patients for IPAF criteria, with 6 patients being diagnosed with IPAF. The incidence of IPAF in children with ILD was 4.5%, and the study reviewed the demographic, clinical features, and treatment of these patients.
RESPIRATORY MEDICINE
(2021)
Article
Rheumatology
J. A. Huapaya, A. Boulougoura, J. Fried, S. Mesdaghinia, B. J. Culotta, S. Carson, P. J. Bergquist, P. Krishnan, H. Wang, C. Reichner, V. Steen
Summary: The long-term pulmonary function, radiological patterns, and survival of IPAF patients were described in this study, and a classification based on CTD-like subgroups using clinical/serologic data was explored. The findings showed that pulmonary function and six-minute walk distance remained stable over 36 months in the IPAF cohort. Although 40% of patients could not be sub-classified, the exploratory subclassification stratified 60% of patients into a CTD-like subgroup.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Santiago Auteri, Maria L. Alberti, Martin E. Fernandez, Guadalupe Blanco, Mercedes Raya, Gabriela Guman, Maria C. Garbarino, Orlando Gabriel Carballo, Fabian Caro
Summary: A significant proportion of patients with occult Sjogren Syndrome were found in the study by performing Minor Salivary Gland Biopsy (MSG) and Dry Eye Tests (DET) on patients with Interstitial Pneumonia with Autoimmune Features (IPAF). Some patients diagnosed with SS did not report dry eyes or dry mouth symptoms.
RESPIRATORY MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
Marco Sebastiani, Paola Faverio, Andreina Manfredi, Giulia Cassone, Caterina Vacchi, Anna Stainer, Maria Rosa Pozzi, Carlo Salvarani, Alberto Pesci, Fabrizio Luppi
Summary: Since 2015, there has been significant attention on research related to IPAF, including studies on prevalence, clinical features, pathological features, and prognosis. Recent studies have found that about 15% of IPAF patients may progress to a defined connective tissue disease, and compared to idiopathic pulmonary fibrosis, IPAF patients show better performance in pulmonary function tests and oxygen delivery requirements. Further prospective studies, potentially derived from multicenter cohorts and randomized control trials, are needed to validate the proposed classification criteria.
Article
Rheumatology
Michael R. Allen, Michail K. Alevizos, David Zhang, Elana J. Bernstein
Summary: This study assessed the ability of two risk prediction models (GAP and ILD-GAP) to predict death or lung transplantation in patients with interstitial pneumonia with autoimmune features (IPAF). The results showed that these models performed well, especially for patients with GAP stage 1 and GAP stage 2 disease.
Article
Medicine, General & Internal
Sheetal Chaurasia, Alamelu Haran, Pavny Chawla, Anish Reddy
Summary: IPAF is a relatively newly described clinical syndrome with low prevalence and nonspecific clinical features, pulmonary function tests, and radiological findings. Further large-scale studies are needed to understand the epidemiology of IPAF and develop effective management options.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Article
Rheumatology
G. Sambataro, C. A. Ferrara, S. E. Torrisi, C. Spadaro, G. Vignigni, A. Vancheri, N. Del Papa, M. Orlandi, M. Colaci, L. Malatino, S. Palmucci, L. Cavagna, D. Sambataro, C. Vancheri
Summary: The study aims to evaluate the rate of progression towards SAD in UIP patients satisfying only one IPAF domain compared to classic IPF, and found that 28.9% of UIPAF patients progressed towards SAD.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2022)
Article
Medicine, General & Internal
Paul Decker, Vincent Sobanski, Thomas Moulinet, David Launay, Eric Hachulla, Victor Valentin, Benoit Godbert, Sabine Revuz, Anne Guillaumot, Emmanuel Gomez, Francois Chabot, Lidwine Wemeau, Roland Jaussaud
Summary: The study aimed to evaluate the incidence of definite connective tissue disease (CTD) diagnosis in interstitial pneumonia with autoimmune features (IPAF) patients during follow-up. It was found that IPAF patients were at higher risk of developing CTD compared to non-IPAF patients, with younger age, higher proportion of females, puffy fingers, capillaroscopy abnormalities, and antisynthetase antibodies at diagnosis being potential predictive factors.
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2022)
Article
Medicine, General & Internal
Theodoros Karampitsakos, Vasilios Tzilas, Ourania Papaioannou, Serafeim Chrysikos, Eirini Vasarmidi, Pierre-Antoine Juge, Styliani Vizirianaki, Eleni Bibaki, Argyro Reppa, Prodromos Sidiropoulos, Matthaios Katsaras, Vasilina Sotiropoulou, Panagiota Tsiri, Electra Koulousousa, Eva Theochari, Georgios Tsirikos, Ioannis Christopoulos, Elli Malakounidou, Eirini Zarkadi, Fotios Sampsonas, Georgios Hillas, Theofanis Karageorgas, Dimitrios Daoussis, Christina Kalogeropoulou, Katerina Dimakou, Nikolaos Tzanakis, Raphael Borie, Philippe Dieude, Katerina Antoniou, Bruno Crestani, Demosthenes Bouros, Argyris Tzouvelekis
Summary: Myositis associated ILD is a commonly under-recognized condition. This multicenter retrospective study identified 75 patients with myositis associated-ILD and found that specific ILD radiographic patterns can indicate underlying inflammatory myopathies. Incorporating these radiological patterns in the diagnostic criteria may improve timely interventions and positively impact patients' survival.
FRONTIERS IN MEDICINE
(2023)
Article
Medicine, General & Internal
Sang-Heon Lee, Hong Ki Min, Se-Hee Kim, Young Whan Kim, Kwang Ha Yoo, Hee Joung Kim, In Ae Kim, Hae-Rim Kim
Summary: This study evaluated the application of nailfold capillaroscopy (NFC) in patients with interstitial pneumonia with autoimmune features (IPAF) and compared it with patients with connective tissue disease-interstitial lung disease (CTD-ILD) and idiopathic interstitial pneumonia (IIP). The results showed that the semi-quantitative score of NFC was higher in the CTD-ILD group compared to the IPAF or IIP group, and giant capillaries and hemorrhages were more frequently present in the CTD-ILD and IPAF groups.
KOREAN JOURNAL OF INTERNAL MEDICINE
(2023)
Article
Medicine, General & Internal
Sara Tomassetti, Claudia Ravaglia, Silvia Puglisi, Athol U. Wells, Jay H. Ryu, Marcello Bosi, Alessandra Dubini, Sara Piciucchi, Francesco Girelli, Paola Parronchi, Federico Lavorini, Elisabetta Rosi, Valentina Luzzi, Marco Matucci Cerinic, Venerino Poletti
Summary: This study aimed to evaluate the differences in clinical characteristics, prognosis, and disease course between IPAF/IPF patients and IPF patients. The results showed that patients who met IPAF criteria were more commonly female, had a higher prevalence of gastroesophageal reflux, and had a higher likelihood of experiencing arthralgias, myalgias, and fever. The presence of IPAF had a positive impact on prognosis, while the isolated presence of circulating autoantibodies did not.
FRONTIERS IN MEDICINE
(2023)
Article
Rheumatology
T. Chen, Q-H Li, Y. Zhang, C-S Yin, D. Weng, Y. Zhou, Y. Hu, J-Y Shi, Y-N Chen, S. Ye, X-D Wang, C-Y Wu, Y. Huang, A-H Zhang, H-P Li
Summary: This study aimed to evaluate the efficacy and safety of pirfenidone for the treatment of interstitial pneumonia with autoimmune features (IPAF). The results showed that pirfenidone could help improve forced vital capacity (FVC%) in IPAF patients with an acceptable safety profile. The therapeutic effects were better in patients with higher drug dosage and longer treatment duration.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2022)
Article
Medicine, General & Internal
Fredeswinda Isabel Romero-Bueno, Maria Jesus Rodriguez-Nieto, Carmelo Palacios Miras, Lina Martinez Estupinan, Maria Jose Martinez-Becerra, Maria Carmen Vegas Sanchez, Oderay Mabel Cedeno Diaz, Olga Sanchez-Pernaute
Summary: This study evaluated the performance of IPAF criteria in clinical practice and described the utility of additional workup in identifying patients with underlying connective tissue diseases. The results showed that the use of IPAF criteria can more accurately identify the presence of connective tissue diseases in patients with autoimmune interstitial pneumonia. In addition, NVC examination can help better identify subtypes of autoimmune interstitial pneumonia with potential clinical significance.
FRONTIERS IN MEDICINE
(2023)
Article
Rheumatology
Ziyi Zhang, Xiaoqian Ma, Junye Bai, Shu Xia, Qian Han, Qun Luo
Summary: This study aimed to investigate the potential of cytokines as biomarkers to predict the occurrence of progressive fibrosis (PF) phenotype in patients with interstitial pneumonia with autoimmune features (IPAF). The results showed that 81.25% of PF-IPAF patients had overlapping bronchoalveolar lavage fluid (BALF) cytokine profiles with idiopathic pulmonary fibrosis (IPF). Cluster analysis revealed that IPAF subtype 2 patients had a higher risk of developing the PF phenotype within 1 year, characterized by elevated levels of CCL2 and CXCL12, and lower lymphocyte proportion in BALF.
Article
Rheumatology
Elena K. Joerns, Traci N. Adams, Chad A. Newton, Bonnie Bermas, David Karp, Kiran Batra, Jose Torrealba, Lesley Davila, Joan Reisch, Craig Glazer, Una E. Makris
Summary: This study aimed to characterize the features associated with response to immunosuppression in patients with interstitial pneumonia with autoimmune features (IPAF). The results showed that baseline clinical assessment did not identify which IPAF patients will respond to immunosuppressive therapy. However, combination therapy with mycophenolate mofetil and prednisone was associated with less disease progression in IPAF patients.
JCR-JOURNAL OF CLINICAL RHEUMATOLOGY
(2022)
Article
Critical Care Medicine
Janelle Vu Pugashetti, Ayodeji Adegunsoye, Zhe Wu, Cathryn T. Lee, Anand Srikrishnan, Sahand Ghodrati, Vivian Vo, Elisabetta A. Renzoni, Athol U. Wells, Christine Kim Garcia, Felix Chua, Chad A. Newton, Philip L. Molyneaux, Justin M. Oldham
Summary: This study found that a decline in FVC of >= 10% and six additional criteria can predict transplant-free survival in patients with non-IPF ILD.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Rheumatology
Robert Lafyatis, Eleanor Valenzi
Summary: The assessment of disease activity in systemic sclerosis (SSc) is challenging, and improved outcome measures are needed. Biomarker studies focused on skin disease may provide opportunities for earlier detection of therapeutic effect. By incorporating high-throughput sequencing and biomarkers, we can gain better understanding of the disease mechanisms in systemic sclerosis.
NATURE REVIEWS RHEUMATOLOGY
(2022)
Article
Respiratory System
Richard J. Allen, Amy Stockwell, Justin M. Oldham, Beatriz Guillen-Guio, David A. Schwartz, Toby M. Maher, Carlos Flores, Imre Noth, Brian L. Yaspan, R. Gisli Jenkins, Louise Wain
Summary: This article describes a genome-wide meta-analysis study on the risk of idiopathic pulmonary fibrosis (IPF), identifying five robust novel genetic association signals related to IPF risk.
Article
Critical Care Medicine
Richard J. Allen, Justin M. Oldham, David A. Jenkins, Olivia C. Leavy, Beatriz Guillen-Guio, Carl A. Melbourne, Shwu-Fan Ma, Jonathan Jou, John S. Kim, William A. Fahy, Eunice Oballa, Richard B. Hubbard, Vidya Navaratnam, Rebecca Braybrooke, Gauri Saini, Katy M. Roach, Martin D. Tobin, Nik Hirani, Moira K. B. Whyte, Naftali Kaminski, Yingze Zhang, Fernando J. Martinez, Angela L. Linderholm, Ayodeji Adegunsoye, Mary E. Strek, Toby M. Maher, Philip L. Molyneaux, Carlos Flores, Imre Noth, R. Gisli Jenkins, Louise Wain
Summary: In this study, a genetic variant associated with disease progression in idiopathic pulmonary fibrosis (IPF) was identified, and the PKN2 gene was proposed as a potential target for novel therapeutic approaches. This finding provides insights into the biological mechanisms underlying IPF and offers potential implications for its treatment.
LANCET RESPIRATORY MEDICINE
(2023)
Article
Critical Care Medicine
Daniel-Costin Marinescu, Ganesh Raghu, Martine Remy-Jardin, William D. Travis, Ayodeji Adegunsoye, Mary Beth Beasley, Jonathan H. Chung, Andrew Churg, Vincent Cottin, Ryoko Egashira, Evans R. Fernandez Perez, Yoshikazu Inoue, Kerri A. Johannson, Ella A. Kazerooni, Yet H. Khor, David A. Lynch, Nestor L. Muller, Jeffrey L. Myers, Andrew G. Nicholson, Sujeet Rajan, Ryoko Saito-Koyama, Lauren Troy, Simon L. F. Walsh, Athol U. Wells, Marlies S. Wijsenbeek, Joanne L. Wright, Christopher J. Ryerson
Summary: This review discusses the diagnostic challenges of distinguishing IPF and fHP, and provides diagnostic algorithms based on existing guidelines to guide consensus diagnosis. The authors also suggest priorities for future research to address uncertainties in the diagnosis of fibrotic interstitial lung diseases.
Review
Radiology, Nuclear Medicine & Medical Imaging
Lydia Chelala, Ayodeji Adegunsoye, Brittany A. Cody, Aliya N. Husain, Jonathan H. Chung
RADIOLOGIC CLINICS OF NORTH AMERICA
(2022)
Article
Respiratory System
John S. Kim, Ani W. Manichaikul, Eric A. Hoffman, Pallavi Balte, Michaela R. Anderson, Elana J. Bernstein, Purnema Madahar, Elizabeth C. Oelsner, Steven M. Kawut, Artur Wysoczanski, Andrew F. Laine, Ayodeji Adegunsoye, Jennie Z. Ma, Margaret A. Taub, Rasika A. Mathias, Stephen S. Rich, Jerome Rotter, Imre Noth, Christine Kim Garcia, R. Graham Barr, Anna J. Podolanczuk
Summary: This study found that the MUC5B promoter variant is associated with pulmonary fibrosis and qualitative assessments of interstitial abnormalities. Additionally, longitudinal increases in high-attenuation areas (HAAs) are associated with the MUC5B risk allele, as well as an increased risk of death and interstitial lung disease (ILD).
Article
Rheumatology
Sebastien Sanges, Lisa Rice, Ly Tu, Eleanor Valenzi, Jean-Luc Cracowski, David Montani, Julio C. Mantero, Camille Ternynck, Guillemette Marot, Andreea M. Bujor, Eric Hachulla, David Launay, Marc Humbert, Christophe Guignabert, Robert Lafyatis
Summary: This study examined the serum proteome of patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) and identified two proteins that were significantly correlated with pulmonary vascular resistance (PVR), potentially providing biomarkers for earlier diagnosis and treatment.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Critical Care Medicine
Deborah Assayag, Ayodeji Adegunsoye, Robert Sheehy, Julie Morisset, Nasreen Khalil, Kerri A. Johannson, Veronica Marcoux, Martin Kolb, Jolene H. Fisher, Helene Manganas, Jeremy Wrobel, Margaret Wilsher, Sally De Boer, John Mackintosh, Daniel C. Chambers, Ian Glaspole, Gregory J. Keir, Cathryn T. Lee, Renea Jablonski, Rekha Vij, Mary E. Strek, Tamera J. Corte, Christopher J. Ryerson
Summary: This study investigates the influence of biological sex, gender, and race on the initiation of treatment for interstitial lung diseases (ILDs). It found that in the Canadian cohort, a higher proportion of men and White patients received treatment compared to women and non-White patients. In contrast, in the Chicago cohort, a lower proportion of men and White patients received treatment compared to women and non-White patients. No differences in treatment initiation were found based on sex or race in the Australasian cohort.
Article
Critical Care Medicine
Justin M. Oldham, Richard J. Allen, Jose M. Lorenzo-Salazar, Philip L. Molyneaux, Shwu-Fan Ma, Chitra Joseph, John S. Kim, Beatriz Guillen-Guio, Tamara Hernandez-Beeftink, Jonathan A. Kropski, Yong Huang, Cathryn T. Lee, Ayodeji Adegunsoye, Janelle Vu Pugashetti, Angela L. Linderholm, Vivian Vo, Mary E. Strek, Jonathan Jou, Adrian Munoz-Barrera, Luis A. Rubio-Rodriguez, Richard Hubbard, Nik Hirani, Moira K. B. Whyte, Simon Hart, Andrew G. Nicholson, Lisa Lancaster, Helen Parfrey, Doris Rassl, William Wallace, Eleanor Valenzi, Yingze Zhang, Josyf Mychaleckyj, Amy Stockwell, Naftali Kaminski, Paul J. Wolters, Maria Molina-Molina, Nicholas E. Banovich, William A. Fahy, Fernando J. Martinez, Ian P. Hall, Martin D. Tobin, Toby M. Maher, Timothy S. Blackwell, Brian L. Yaspan, R. Gisli Jenkins, Carlos Flores, Louise V. Wain, Imre Noth
Summary: This study conducted a staged genome-wide association study to identify and validate molecular determinants of survival in idiopathic pulmonary fibrosis (IPF). Four novel variants associated with IPF survival were identified, including one within the PCSK6 gene that reached genome-wide significance. Downstream analyses suggested that PCSK6 protein may play an important role in IPF progression.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Multidisciplinary Sciences
Ayodeji Adegunsoye, Chad A. Newton, Justin M. Oldham, Brett Ley, Cathryn T. Lee, Angela L. Linderholm, Jonathan H. Chung, Nicole Garcia, Da Zhang, Rekha Vij, Robert Guzy, Renea Jablonski, Remzi Bag, Rebecca S. Voogt, Shwu-Fan Ma, Anne I. Sperling, Ganesh Raghu, Fernando J. Martinez, Mary E. Strek, Paul J. Wolters, Christine Kim Garcia, Brandon L. Pierce, Imre Noth
Summary: This study investigated the association between leukocyte telomere length (LTL) and age and mortality in racially diverse patients with pulmonary fibrosis (PF). The study found that LTL shortened with increasing age, and this association was even stronger in PF patients. In all racial groups, LTL below the median predicted worse mortality.
NATURE COMMUNICATIONS
(2023)
Article
Health Care Sciences & Services
Ayodeji Adegunsoye, Rachel Baccile, Thomas J. Best, Victoria Zaksas, Hui Zhang, Rasika Karnik, Bhakti K. Patel, Anthony E. Solomonides, William F. Parker, Julian Solway
Summary: Recent use of rituximab or chemotherapy in hospitalized COVID-19 patients is associated with an increased risk of post-COVID-19 pulmonary fibrosis, while corticosteroid use is slightly associated with an increased risk, and amiodarone shows no significant association.
LANCET REGIONAL HEALTH-AMERICAS
(2023)
Letter
Health Care Sciences & Services
Ayodeji Adegunsoye, Rachel Baccile, Thomas J. Best, Victoria Zaksas, Hui Zhang, Rasika Karnik, Bhakti K. Patel, Anthony E. Solomonides, William F. Parker, Julian Solway
LANCET REGIONAL HEALTH-AMERICAS
(2023)
Article
Respiratory System
John S. Kim, Sydney B. Montesi, Ayodeji Adegunsoye, Stephen M. Humphries, Margaret L. Salisbury, Lida P. Hariri, Jonathan A. Kropski, Luca Richeldi, Athol U. Wells, Simon Walsh, R. Gisli Jenkins, Ivan Rosas, Imre Noth, Gary M. Hunninghake, Fernando J. Martinez, Anna J. Podolanczuk
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Meeting Abstract
Critical Care Medicine
E. Wang, K. E. Wroblewski, M. K. McClintock, J. M. Pinto, L. J. Witt
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)