ATTR amyloidosis during the COVID-19 pandemic: insights from a global medical roundtable

Orphanet Journal of Rare Diseases

Kemi Olugemo¹  Andrew M. Rosen¹  David R. Hurwitz¹  Jacqueline Shehata¹  John L. Berk²  Teresa Coelho³  Mazen Hanna⁴  Mathew S. Maurer⁵  Thomas H. Brannagan⁶  Marcia Waddington-Cruz⁷  Luis F. Quintana⁸  Esther Gonzalez⁹  Hanna K. Gaggin^1,10  Angela Dispenzieri^1,11  Morie Gertz^1,11  Carol Whelan^1,12  Julian D. Gillmore^1,12  Brian M. Drachman^1,13  Thibaud Damy^1,14  Frederick L. Ruberg^1,15  Vera Bril^1,16  Hartmut H. Schmidt^1,17  Nowell Fine^1,18  Chiara Briani^1,19  Sami L. Khella^2,20  Jose Nativi-Nicolau^2,21  Michaela Auer-Grumbach^2,22 

[1] Akcea Therapeutics, Boston, MA, USA;Boston University, Boston, MA, USA;Centro Hospitalar Universitário do Porto, Porto, Portugal;Cleveland Clinic, Cleveland, OH, USA;Columbia University, New York, NY, USA;Department of Neurology, Columbia University, New York, NY, USA;Federal University of Rio de Janeiro, Rio de Janeiro, Brazil;Hospital Clinic, University of Barcelona, Barcelona, Spain;Hospital Puerta de Hierro of Madrid, Madrid, Spain;Massachusetts General Hospital, Boston, MA, USA;Mayo Clinic, Rochester, MN, USA;National Amyloidosis Centre, Royal Free Hospital, London, UK;Penn Presbyterian Medical Center, Philadelphia, PA, USA;Referral Center for Cardiac Amyloidosis, Cardiology Department, APHP-Henri Mondor Hospital, Creteil, France;Section of Cardiovascular Medicine, Department of Medicine and Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA;University Health Network, Toronto, ON, Canada;University Hospital of Münster, Münster, Germany;University of Calgary, Calgary, AB, Canada;University of Padova, Padova, Italy;University of Pennsylvania, Philadelphia, PA, USA;University of Utah, Salt Lake City, UT, USA;Vienna General Hospital, Vienna, Austria;

关键词: COVID-19;    SARS-CoV-2;    Amyloidosis;    Rare disease;    ATTR;   

DOI  :  10.1186/s13023-021-01834-0

来源: Springer

【 摘 要 】

BackgroundThe global spread of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection causing the ongoing coronavirus disease 2019 (COVID-19) pandemic has raised serious concern for patients with chronic disease. A correlation has been identified between the severity of COVID-19 and a patient’s preexisting comorbidities. Although COVID-19 primarily involves the respiratory system, dysfunction in multiple organ systems is common, particularly in the cardiovascular, gastrointestinal, immune, renal, and nervous systems. Patients with amyloid transthyretin (ATTR) amyloidosis represent a population particularly vulnerable to COVID-19 morbidity due to the multisystem nature of ATTR amyloidosis.Main bodyATTR amyloidosis is a clinically heterogeneous progressive disease, resulting from the accumulation of amyloid fibrils in various organs and tissues. Amyloid deposition causes multisystem clinical manifestations, including cardiomyopathy and polyneuropathy, along with gastrointestinal symptoms and renal dysfunction. Given the potential for exacerbation of organ dysfunction, physicians note possible unique challenges in the management of patients with ATTR amyloidosis who develop multiorgan complications from COVID-19. While the interplay between COVID-19 and ATTR amyloidosis is still being evaluated, physicians should consider that the heightened susceptibility of patients with ATTR amyloidosis to multiorgan complications might increase their risk for poor outcomes with COVID-19.ConclusionPatients with ATTR amyloidosis are suspected to have a higher risk of morbidity and mortality due to age and underlying ATTR amyloidosis-related organ dysfunction. While further research is needed to characterize this risk and management implications, ATTR amyloidosis patients might require specialized management if they develop COVID-19. The risks of delaying diagnosis or interrupting treatment for patients with ATTR amyloidosis should be balanced with the risk of exposure in the health care setting. Both physicians and patients must adapt to a new construct for care during and possibly after the pandemic to ensure optimal health for patients with ATTR amyloidosis, minimizing treatment interruptions.

【 授权许可】

CC BY