RENAL-TRANSPLANTATION RELIEVES THE SYMPTOMS BUT DOES NOT REVERSE BETA-2-MICROGLOBULIN AMYLOIDOSIS

Renal transplantation is considered to be the treatment of choice of d ialysis-related beta 2-microglobulin amyloidosis (DRA), as it provides near-normal serum levels of beta 2-microglobulin and obviates the nee d for dialysis, However, the long-term outcome of DRA after transplant ation has not been fully assessed, and its evolution after transplant failure has not been reported, This study examined 17 patients with hi stologically confirmed DRA who underwent kidney transplantation and ha d a dialysis-free follow-up period in excess of 1 yr, Immunosuppressiv e treatment included low-dose prednisolone, cyclosporine, and/or azath ioprine. Symptoms related to DRA were sought at every outpatient visit , and bone x-rays were performed at time of transplantation and annual ly thereafter. The number and size of the bone cysts were determined. Most of the DRA symptoms, and particularly shoulder stiffness, disappe ared within the first wk after transplantation and this persisted thro ughout the transplant follow-up period (58.5 +/- 9 months), However, t he number of bone cysts remained remarkably constant even in those pat ients with still-functioning grafts (12 +/- 7.5 and 12.1 +/- 7.7, befo re and at last transplantation follow-up examination, respectively), b eta 2-microglobulin amyloid was found to be present in one patient ope rated on for hip fracture 2 yr after receiving a well-functioning tran splant. Seven patients experienced graft failure and returned to dialy sis after 47 +/- 39 months of transplantation, Severe DRA symptoms rea ppeared strikingly early after resuming hemodialysis, and five out of the seven patients required surgery for carpal tunnel syndrome, three of them within the first yr (mean, 17 +/- 12 months). The number of cy sts significantly increased from 17 +/- 11 to 21 +/- 11 during the sec ond dialysis period, These findings provide further evidence suggestin g that although the clinical expression of DRA is arrested during tran splantation, the anatomical lesions and the pathological processes und erlying it are unlikely to be reversed.