The American journal of medicine
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Chronic kidney disease affects approximately 10% of the population or 800 million people globally, with diabetes being the leading cause. The presence of chronic kidney disease with impaired kidney function or with albuminuria is associated with an increased risk of a progressive loss of renal function and increased risk of cardiovascular disease and excess mortality. Screening for chronic kidney disease is critically important because during the initial stages patients often have no symptoms and because we now have available recently approved multiple interventions that can reduce the high risks dramatically. ⋯ Clinicians need to perform regular screening and concomitant management of risk factors. Recent therapeutic options must be implemented to improve outcomes. Finally, a reduction in albuminuria after initiation of intervention constitutes a treatment target because it indicates improved prognosis.
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Light chain (AL) amyloidosis is a potentially fatal disease of monoclonal plasma cells that leads to accumulation of light chain amyloid fibrils, organ damage, and the manifestations of clinical disease. Meanwhile, coronavirus disease 2019 (COVID-19) is a disease caused by infection with the severe acute respiratory syndrome coronavirus 2 virus, with the potential to cause severe systemic illness and death. ⋯ This overlap creates unique challenges in caring for patients with AL amyloidosis, which are further compounded by the immunosuppressive nature of anti-plasma cell therapies, the need for frequent clinical assessments, and the exclusion of AL amyloidosis patients from initial COVID-19 vaccine trials. Herein, we highlight many of the relevant concerns related to COVID-19 and the treatment of AL amyloidosis, summarize a general approach for AL amyloidosis management amidst the ongoing COVID-19 pandemic, and discuss current guidance about COVID-19 vaccination of patients with AL amyloidosis.
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The use of statin therapy in atherosclerotic cardiovascular disease (ASCVD) has demonstrated substantial improvement in morbidity and mortality of the aging population. Despite exhaustive studies demonstrating the benefits of statin therapy linking lower cholesterol levels to decreased vascular events, statin guidelines vary greatly with age, and recommendations are unclear regarding initiation and discontinuation of statin therapy in patients 65 years and older. ⋯ Therefore, patients who meet these criteria, regardless of age, should begin statin therapy. There is also some evidence to suggest that statin therapy may be beneficial in primary prevention of major cardiovascular events, although these data are not as well studied as secondary prevention use of statin therapy, and should therefore be individualized for each patient.
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Transthyretin amyloidosis (ATTR) is an under-recognized cause of cardiomyopathy and neuropathy. Until recently, there were limited therapeutic options for ATTR. However, new therapeutics, including tafamidis, patisiran, and inotersen, increase both quality and length of life in patients with ATTR. ⋯ In addition, we discuss emerging ATTR therapies including improvements in drug delivery methods, antibodies to break down deposited amyloid fibrils, and gene editing. ATTR is a prime example of how an understanding of the pathophysiological basis of disease can lead to effective therapies. The future of ATTR therapy is bright, with every reason to believe outcomes will continue to improve.
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Review
Update on the Contemporary Treatment of Light Chain Amyloidosis Including Stem Cell Transplantation.
The management of immunoglobulin light chain (AL) amyloidosis is complex. Emerging data have shown promising results for several novel agents. We review the management of AL amyloidosis, including factors that determine transplant eligibility, treatment options for transplant-ineligible patients, and treatment options for relapsed/refractory AL amyloidosis. ⋯ The latter option is based on a landmark trial that led to the first US Food and Drug Administration-approved therapy for AL amyloidosis. For relapsed/refractory disease, novel therapeutics including proteosome inhibitors, immunomodulatory agents, and monoclonal antibodies have shown promising results. In this review, we summarize data for various therapeutics in different clinical scenarios of AL amyloidosis.