Theme |
Designated intractable kidney diseases:The control in non-dialysis CKD and renal replacement therapy |
Title |
Diagnosis and management of systemic AL type amyloidosis |
Publish Date |
2016/04 |
Author |
Nahoko Nishimura |
Division of Nephrology and Rheumatology, Aichi Medical University School of Medicine |
Author |
Hirokazu Imai |
Division of Nephrology and Rheumatology, Aichi Medical University School of Medicine |
[ Summary ] |
The number of systemic amyloidosis patients in Japan is approximately 1800. Half of those patients have amyloid light chain (AL) conditions. The annual number of new dialysis patients is almost 100. The total number of patients on dialysis due to systemic amyloidosis is between 450 and 490. The median survival time after initiation of dialysis is 2 years, up from 9 months in the 1990s. New drugs for multiple myeloma such as proteosome inhibitors and lenalidomide significantly improves prognoses and further progress in the study of systemic amyloidosis is expected. In order to provide good outcomes, it is very important to provide early diagnoses and to initiate adequate chemotherapy. |