Identifying clinical and research priorities in sickle cell lung disease an official American Thoracic Society workshop report


A. Parker Ruhl, National Institute of Allergy and Infectious Diseases (NIAID)
S. Christy Sadreameli, Johns Hopkins School of Medicine
Julian L. Allen, The Children's Hospital of Philadelphia
Debra P. Bennett, Patient Advocate
Andrew D. Campbell, Childrens National Health System
Thomas D. Coates, Children's Hospital Los Angeles
Dapa A. Diallo, University of Sciences
Joshua J. Field, Medical College of Wisconsin
Elizabeth K. Fiorino, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell
Mark T. Gladwin, University of Pittsburgh Medical Center
Jeffrey A. Glassberg, Icahn School of Medicine at Mount Sinai
Victor R. Gordeuk, University of Illinois at Chicago
Leroy M. Graham, Atlanta Medical Center
Anne Greenough, King's College London
Jo Howard, Children's Hospital Los Angeles
Gregory J. Kato, University of Pittsburgh Medical Center
Jennifer Knight-Madden, Caribbean Institute for Health Research
Benjamin T. Kopp, The Ohio State University College of Medicine
Anastassios C. Koumbourlis, The George Washington University
Sophie M. Lanzkron, Johns Hopkins School of Medicine
Robert I. Liem, Northwestern University Feinberg School of Medicine
Roberto F. Machado, Indiana University School of Medicine
Alem Mehari, Howard University College of Medicine
Claudia R. Morris, Emory University School of Medicine
Folasade O. Ogunlesi, The George Washington University
Carol L. Rosen, University Hospitals Rainbow Babies & Children's Hospital
Kim Smith-Whitley, University of Pennsylvania Perelman School of Medicine
Danna Tauber, The Children's Hospital of Philadelphia
Nancy Terry, National Institutes of Health (NIH)
Swee Lay Thein, National Heart, Lung, and Blood Institute (NHLBI)
Elliott Vichinsky, Children's Hospital Oakland Research Institute
Nargues A. Weir, National Institutes of Health (NIH)
Robyn T. Cohen, Boston University School of Medicine
Elizabeth S. Klings, Boston University School of Medicine

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Background: Pulmonary complications of sickle cell disease (SCD) are diverse and encompass acute and chronic disease. The understanding of the natural history of pulmonary complications of SCD is limited, no specific therapies exist, and these complications are a primary cause of morbidity and mortality. Methods: We gathered a multidisciplinary group of pediatric and adult hematologists, pulmonologists, and emergency medicine physicians with expertise in SCD-related lung disease along with an SCD patient advocate for an American Thoracic Society–sponsored workshop to review the literature and identify key unanswered clinical and research questions. Participants were divided into four subcommittees on the basis of expertise: 1) acute chest syndrome, 2) lower airways disease and pulmonary function, 3) sleep-disordered breathing and hypoxia, and 4) pulmonary vascular complications of SCD. Before the workshop, a comprehensive literature review of each subtopic was conducted. Clinically important questions were developed after literature review and were finalized by group discussion and consensus. Results: Current knowledge is based on small, predominantly observational studies, few multicenter longitudinal studies, and even fewer high-quality interventional trials specifically targeting the pulmonary complications of SCD. Each subcommittee identified the three or four most important unanswered questions in their topic area for researchers to direct the next steps of clinical investigation. Conclusions: Important and clinically relevant questions regarding sickle cell lung disease remain unanswered. High-quality, multicenter, longitudinal studies and randomized clinical trials designed and implemented by teams of multidisciplinary clinician-investigators are needed to improve the care of individuals with SCD.

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