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

Cardiac amyloidosis in the setting of systemic amyloidosis due to β₂-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 β₂-microglobulin amyloidosis are unclear. The published literature regarding dialysis-related cardiac β₂-microglobulin amyloidosis (Aβ₂M) was reviewed, and a new case of this disorder is reported. The cumulative available data were analyzed for changing patient characteristics over time. Cardiac Aβ₂M 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 β₂-microglobulin levels in chronic dialysis patients. Newer dialysis technologies appear to reduce the risk of developing Aβ₂M compared with traditional low-flow dialysis membranes. New cases of documented dialysis-related cardiac Aβ₂M are uncommon. Analysis of the cases of dialysis-related cardiac Aβ₂M 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 Aβ₂M 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 Aβ₂M to be more common in those countries. Dialysis-related osteoarticular Aβ₂M appears to continue to occur in the setting of chronic dialysis with the use of high-flow membranes. Dialysis-related cardiac Aβ₂M 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. SUMMARY: Dialysis-related cardiac β₂-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 continue to afflict patients receiving chronic dialysis with newer dialysis technologies.