The pathology and changing epidemiology of dialysis-related cardiac beta-2 microglobulin amyloidosis

AbstractCardiac amyloidosis in the setting of systemic amyloidosis due to β 2-microglobulin can occur in the setting of long-term dialysis. It has been suggested that newer dialysis techniques may prevent or at least reduce the likelihood of this disorder occurring. Currently, the prevalence and incidence of dialysis-related cardiac β 2-microglobulin amyloidosis are unclear. The published literature regarding dialysis-related cardiac β 2-microglobulin amyloidosis (Aβ 2M) was reviewed, and a new case of this disorder is reported. The cumulative available data were analyzed for changing patient characteristics over time. Cardiac Aβ 2M was previously a common condition in patients who had undergone dialysis for 9 or more years with traditional low-flow dialysis membranes. Newer dialysis technologies reduce, but do not normalize, serum β 2-microglobulin levels in chronic dialysis patients. Newer dialysis technologies appear to reduce the risk of developing Aβ 2M compared with traditional low-flow dialysis membranes. New cases of documented dialysis-related cardiac Aβ 2M are uncommon. Analysis of the cases of dialysis-related cardiac Aβ 2M published over the last 3 decades reveals increasing dialysis intervals over time. Thus, new cases of this disorder are typically associated with remote prior dialysis with low-flow membranes. While initially, the majority of cases of dialysis-related cardiac Aβ 2M were reported from Europe and the United States, more recently, the majority of cases were reported from Japan, where there is a relatively large population of patients on very long-term dialysis. In addition, low-flow dialysis membranes continue to be used in many parts of the world, raising the potential for dialysis-related cardiac Aβ 2M to be more common in those countries. Dialysis-related osteoarticular Aβ 2M appears to continue to occur in the setting of chronic dialysis with the use of high-flow membranes. Dialysis-related cardiac Aβ 2M is currently uncommon and typically associated with the use of low-flow dialysis membranes. However, the condition could potentially occur in the setting of long-term dialysis even with the use of high-flow membranes. SummaryDialysis-related cardiac β 2-microglobulin amyloidosis frequently occurred in the past in patients who had undergone dialysis for nine or more years. Currently, the condition is uncommon and typically associated with remote prior dialysis with low-flow membranes. There is potential for this condition to