Global spread of COVID-19 has raised serious concern for patients with transthyretin amyloidosis

Reviewed by Emily Henderson, B.Sc.May 7 2021

The global spread of COVID-19 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 transthyretin amyloidosis (ATTR) (a disease caused by an abnormal misfolded protein that causes buildup of amyloid deposits in the heart, peripheral nervous system including the autonomic nervous system, or other organs) represent a population particularly vulnerable to COVID-19 morbidity due to the multisystem nature of ATTR amyloidosis.

Early on in the pandemic, an expert roundtable of international clinicians and investigators who specialize in ATTR amyloidosis was convened to discuss the impact of the pandemic on the ATTR patient population, ongoing clinical trials, and access to care.

Since ATTR amyloidosis causes multi-system disease, as does COVID-19, they reviewed how the two diseases could overlap in presentation and how ATTR patients could be particularly vulnerable to advanced COVID complications.

In a position statement currently online in the Orphanet Journal of Rare Diseases, this panel concluded that patients with ATTR amyloidosis who develop COVID-19 have a higher risk of mortality, due to age, the organ system dysfunction caused by ATTR amyloid disease, and other comorbidities such as hypertension and diabetes, necessitating additional precautions and specialized management including:

• Older patients with ATTR amyloidosis are at increased risk for developing severe COVID-19, requiring more careful social distancing, use of protective masks, and frequent hand washing
• Many older patients with ATTR amyloidosis share comorbidities known to increase morbidity and mortality risk in COVID-19. Patients with cardiac ATTR amyloidosis should be aware of their predisposition to complications if they develop COVID-19, particularly stroke, congestive heart failure, and other cardiac-related issues
• Differential access to care and the utilization of telehealth may more greatly impact older individuals with ATTR amyloidosis
• Limitations of in-person evaluations and performing diagnostic evaluation during the COVID-19 pandemic limits the ability to diagnose and follow progression of patients with ATTR amyloidosis
• Laboratory test results, such as elevated cardiac biomarkers, may be seen in both ATTR amyloidosis and COVID-19, confusing interpretation

Patients with ATTR amyloidosis are at increased risk for COVID complications, so recognition of ATTR in undiagnosed patients as well as specialized care in diagnosed ATTR patients who contract COVID is warranted."

Frederick Ruberg, MD, Study Corresponding Author and Associate Professor, Medicine and Radiology, Boston University School of Medicine

The report was coordinated by Drs. Ruberg and Thomas Brannagan III, MD, professor of neurology, Columbia University.

According to the panel, because of overwhelmed resources as well as safety of patients and healthcare personnel, new approaches to clinical research including remote assessments need to be considered. "More research is needed to fill remaining gaps in knowledge to better understand the real-world clinical impact of COVID-19 on patients with ATTR amyloidosis," added Ruberg, who specializes in cardiac amyloidosis at the BU/Boston Medical Center Amyloidosis Center.