Orphanet Journal of Rare Diseases (May 2021)

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

  • Thomas H. Brannagan,
  • Michaela Auer-Grumbach,
  • John L. Berk,
  • Chiara Briani,
  • Vera Bril,
  • Teresa Coelho,
  • Thibaud Damy,
  • Angela Dispenzieri,
  • Brian M. Drachman,
  • Nowell Fine,
  • Hanna K. Gaggin,
  • Morie Gertz,
  • Julian D. Gillmore,
  • Esther Gonzalez,
  • Mazen Hanna,
  • David R. Hurwitz,
  • Sami L. Khella,
  • Mathew S. Maurer,
  • Jose Nativi-Nicolau,
  • Kemi Olugemo,
  • Luis F. Quintana,
  • Andrew M. Rosen,
  • Hartmut H. Schmidt,
  • Jacqueline Shehata,
  • Marcia Waddington-Cruz,
  • Carol Whelan,
  • Frederick L. Ruberg

DOI
https://doi.org/10.1186/s13023-021-01834-0
Journal volume & issue
Vol. 16, no. 1
pp. 1 – 13

Abstract

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Abstract Background The 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 body ATTR 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. Conclusion Patients 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.

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