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1. Clinical Assessment of Pulmonary Hypertension

Author

Hon, Stephanie M., Lechartier, Benoit, Humbert, Marc, Farber, Harrison W., Allanore, Yannick, editor, Varga, John, editor, Denton, Christopher P., editor, Kuwana, Masataka, editor, Chung, Lorinda, editor, and Shah, Ami A., editor

Published
2024
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2. Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy: An Official American Thoracic Society Clinical Practice Guideline.

Author

Hayes Jr., Don, Jennerich, Ann L., Coleman, Ryan D., Abston, Eric, Adamson, Gregory T., Berger, John T., Cohen, Sarah P., Cooper, David S., Eghtesady, Pirooz, Fynn-Thompson, Francis, Grady, R. Mark, Hon, Stephanie M., Hoopes, Charles W., Jewell, Teresa, Lewthwaite, Hayley, Liu, Michelle F., McGiffin, David C., Mullen, Mary P., Qureshi, Athar M., and Morales, David L. S.

Subjects
PULMONARY hypertension, EXTRACORPOREAL membrane oxygenation, LUNG transplantation, CHILD patients, MEDICAL personnel
Abstract

Background: Pulmonary hypertension in children is progressive with wide variability in prognosis. This document provides an evidence-based clinical practice guideline for the management of children with progressive pulmonary hypertension despite optimal therapy. Methods: A multidisciplinary panel identified pertinent questions regarding the management of children with pulmonary hypertension that has progressed despite optimal therapy, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development and Evaluation approach to develop clinical recommendations. Results: After reviewing the research evidence, the panel considered the balance of desirable (benefits) and undesirable (harms and burdens) effects of the interventions in each proposed question. Valuation of our main outcomes was also considered, together with resources required, equity, acceptability, and feasibility. Recommendations were developed for or against interventional strategies specific to children with pulmonary hypertension that has progressed despite optimal therapy. Conclusions: Although there is a growing population of children with pulmonary hypertension, there is a striking lack of empirical evidence regarding management of those whose disease has progressed despite optimal pharmacotherapy. The panel formulated and provided the rationale for clinical recommendations for or against interventional strategies on the basis of this limited empirical evidence, coupled with expert opinion, to aid clinicians in the management of these complex pediatric patients. In addition, we identified important areas for future research. [ABSTRACT FROM AUTHOR]

Published
2025
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6. Transbronchial Lung Cryobiopsy in Patients with Interstitial Lung Disease: A Systematic Review

Author

Kheir, Fayez, primary, Uribe Becerra, Juan Pablo, additional, Bissell, Brittany, additional, Ghazipura, Marya, additional, Herman, Derrick, additional, Hon, Stephanie M., additional, Hossain, Tanzib, additional, Khor, Yet H., additional, Knight, Shandra L., additional, Kreuter, Michael, additional, Macrea, Madalina, additional, Mammen, Manoj J., additional, Martinez, Fernando J., additional, Poletti, Venerino, additional, Troy, Lauren, additional, Raghu, Ganesh, additional, and Wilson, Kevin C., additional

Published
2022
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9. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Author

Raghu, Ganesh, Remy-Jardin, Martine, Richeldi, Luca, Thomson, Carey C, Inoue, Yoshikazu, Johkoh, Takeshi, Kreuter, Michael, Lynch, David A, Maher, Toby M, Martinez, Fernando J, Molina-Molina, Maria, Myers, Jeffrey L, Nicholson, Andrew G, Ryerson, Christopher J, Strek, Mary E, Troy, Lauren K, Wijsenbeek, Marlie, Mammen, Manoj J, Hossain, Tanzib, Bissell, Brittany D, Herman, Derrick D, Hon, Stephanie M, Kheir, Fayez, Khor, Yet H, Macrea, Madalina, Antoniou, Katerina M, Bouros, Demosthene, Buendia-Roldan, Ivette, Caro, Fabian, Crestani, Bruno, Ho, Lawrence, Morisset, Julie, Olson, Amy L, Podolanczuk, Anna, Poletti, Venerino, Selman, Moisé, Ewing, Thoma, Jones, Stephen, Knight, Shandra L, Ghazipura, Marya, Wilson, Kevin C, Richeldi, Luca (ORCID:0000-0001-8594-1448), Raghu, Ganesh, Remy-Jardin, Martine, Richeldi, Luca, Thomson, Carey C, Inoue, Yoshikazu, Johkoh, Takeshi, Kreuter, Michael, Lynch, David A, Maher, Toby M, Martinez, Fernando J, Molina-Molina, Maria, Myers, Jeffrey L, Nicholson, Andrew G, Ryerson, Christopher J, Strek, Mary E, Troy, Lauren K, Wijsenbeek, Marlie, Mammen, Manoj J, Hossain, Tanzib, Bissell, Brittany D, Herman, Derrick D, Hon, Stephanie M, Kheir, Fayez, Khor, Yet H, Macrea, Madalina, Antoniou, Katerina M, Bouros, Demosthene, Buendia-Roldan, Ivette, Caro, Fabian, Crestani, Bruno, Ho, Lawrence, Morisset, Julie, Olson, Amy L, Podolanczuk, Anna, Poletti, Venerino, Selman, Moisé, Ewing, Thoma, Jones, Stephen, Knight, Shandra L, Ghazipura, Marya, Wilson, Kevin C, and Richeldi, Luca (ORCID:0000-0001-8594-1448)

Abstract

Background: This American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociacion Latinoamericana de T orax guideline updates prior idiopathic pulmonary fibrosis (IPF) guidelines and addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases (ILDs) other than IPF. Methods: A committee was composed of multidisciplinary experts in ILD, methodologists, and patient representatives. 1) Update of IPF: Radiological and histopathological criteria for IPF were updated by consensus. Questions about transbronchial lung cryobiopsy, genomic classifier testing, antacid medication, and antireflux surgery were informed by systematic reviews and answered with evidence-based recommendations using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach. 2) Progressive pulmonary fibrosis (PPF): PPF was defined, and then radiological and physiological criteria for PPF were determined by consensus. Questions about pirfenidone and nintedanib were informed by systematic reviews and answered with evidence-based recommendations using the GRADE approach. Results: 1) Update of IPF: A conditional recommendation was made to regard transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy in centers with appropriate expertise. No recommendation was made for or against genomic classifier testing. Conditional recommendations were made against antacid medication and antireflux surgery for the treatment of IPF. 2) PPF: PPF was defined as at least two of three criteria (worsening symptoms, radiological progression, and physiological progression) occurring within the past year with no alternative explanation in a patient with an ILD other than IPF. A conditional recommendation was made for nintedanib, and additional research into pirfenidone was recommended. Conclusions: The conditional recommendations in this guideline are intended to provide the basis for rational

Published
2022

10. Antacid Medication and Antireflux Surgery in Patients with Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis

Author

Khor, Yet H., primary, Bissell, Brittany, additional, Ghazipura, Marya, additional, Herman, Derrick, additional, Hon, Stephanie M., additional, Hossain, Tanzib, additional, Kheir, Fayez, additional, Knight, Shandra L., additional, Kreuter, Michael, additional, Macrea, Madalina, additional, J. Mammen, Manoj, additional, Molina-Molina, Maria, additional, Selman, Moises, additional, Wijsenbeek, Marlies, additional, Raghu, Ganesh, additional, and Wilson, Kevin C., additional

Published
2022
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11. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Author

Raghu, Ganesh, primary, Remy-Jardin, Martine, additional, Richeldi, Luca, additional, Thomson, Carey C., additional, Inoue, Yoshikazu, additional, Johkoh, Takeshi, additional, Kreuter, Michael, additional, Lynch, David A., additional, Maher, Toby M., additional, Martinez, Fernando J., additional, Molina-Molina, Maria, additional, Myers, Jeffrey L., additional, Nicholson, Andrew G., additional, Ryerson, Christopher J., additional, Strek, Mary E., additional, Troy, Lauren K., additional, Wijsenbeek, Marlies, additional, Mammen, Manoj J., additional, Hossain, Tanzib, additional, Bissell, Brittany D., additional, Herman, Derrick D., additional, Hon, Stephanie M., additional, Kheir, Fayez, additional, Khor, Yet H., additional, Macrea, Madalina, additional, Antoniou, Katerina M., additional, Bouros, Demosthenes, additional, Buendia-Roldan, Ivette, additional, Caro, Fabian, additional, Crestani, Bruno, additional, Ho, Lawrence, additional, Morisset, Julie, additional, Olson, Amy L., additional, Podolanczuk, Anna, additional, Poletti, Venerino, additional, Selman, Moisés, additional, Ewing, Thomas, additional, Jones, Stephen, additional, Knight, Shandra L., additional, Ghazipura, Marya, additional, and Wilson, Kevin C., additional

Published
2022
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14. Use of a Genomic Classifier in Patients with Interstitial Lung Disease: A Systematic Review

Author

Kheir, Fayez, primary, Uribe Becerra, Juan Pablo, additional, Bissell, Brittany, additional, Ghazipura, Marya, additional, Herman, Derrick, additional, Hon, Stephanie M, additional, Hossain, Tanzib, additional, Khor, Yet H., additional, Knight, Shandra L., additional, Kreuter, Michael, additional, Macrea, Madalina, additional, Mammen, Manoj J, additional, Martinez, Fernando J., additional, Poletti, Venerino, additional, Troy, Lauren, additional, Raghu, Ganesh, additional, and Wilson, Kevin C, additional

Published
2021
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15. Use of a Genomic Classifier in Patients with Interstitial Lung Disease: A Systematic Review and Meta-Analysis.

Author

Kheir, Fayez, Becerra, Juan Pablo Uribe, Bissell, Brittany, Ghazipura, Marya, Herman, Derrick, Hon, Stephanie M., Hossain, Tanzib, Khor, Yet H., Knight, Shandra L., Kreuter, Michael, Macrea, Madalina, Mammen, Manoj J., Martinez, Fernando J., Poletti, Venerino, Troy, Lauren, Raghu, Ganesh, Wilson, Kevin C., and Uribe Becerra, Juan Pablo

Subjects
RESEARCH, IDIOPATHIC pulmonary fibrosis, META-analysis, LUNGS, RESEARCH methodology, SYSTEMATIC reviews, INTERSTITIAL lung diseases, EVALUATION research, COMPARATIVE studies, GENOMICS, RESEARCH funding, COMPUTED tomography
Abstract

Background: Usual interstitial pneumonia (UIP) is the histopathologic hallmark of idiopathic pulmonary fibrosis (IPF), the prototypical interstitial lung disease (ILD). Diagnosis of IPF requires that a typical UIP pattern be identified by using high-resolution chest computed tomography or lung sampling. A genomic classifier for UIP has been developed to predict histopathologic UIP by using lung samples obtained through bronchoscopy. Objective: To perform a systematic review to evaluate genomic classifier testing in the detection of histopathologic UIP to inform new American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax guidelines. Data Sources: Medline, Embase, and the Cochrane Central Register of Controlled Trials were searched through June 2020. Data Extraction: Data were extracted from studies that enrolled patients with ILD and reported the use of genomic classifier testing. Synthesis: Data were aggregated across studies via meta-analysis. The quality of the evidence was appraised by using the Grading of Recommendations, Assessment, Development, and Evaluation approach. Results: Genomic classifier testing had a sensitivity of 68% (95% confidence interval [CI], 55-73%) and a specificity of 92% (95% CI, 81-95%) in predicting the UIP pattern in ILD. Confidence in an IPF diagnosis increased from 43% to 93% in one cohort and from 59% to 89% in another cohort. Agreement levels in categorical IPF and non-IPF diagnoses measured by using a concordance coefficient were 0.75 and 0.64 in the two cohorts. The quality of evidence was moderate for test characteristics and very low for both confidence and agreement. Conclusions: Genomic classifier testing predicts histopathologic UIP in patients with ILD with a specificity of 92% and improves diagnostic confidence; however, sensitivity is only 68%, and testing is not widely available. [ABSTRACT FROM AUTHOR]

Published
2022
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19. Pulmonary Arterial Hypertension in Patients Infected with the Human Immunodeficiency Virus

Author

Hon, Stephanie M., Alpizar-Rivas, Rodolfo M., and Farber, Harrison W.

Abstract

It is important to recognize and treat Human Immunodeficiency Virus-associated Pulmonary Arterial Hypertension (HIV-PAH) because of the associated morbidity and mortality. With the introduction of antiretroviral therapies (ART), improved survival has changed the focus of treatment management from immunodeficiency-related opportunistic infections to chronic cardiovascular complications, including HIV-PAH. The 2018 6th World Symposium of Pulmonary Hypertension recommended a revised definition of PAH that might result in a greater number of patients with HIV-PAH; however, the implication of this change is not yet clear. Here, we review current literature on the diagnosis, management and outcomes of patients with HIV-PAH.

Published
2021
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