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A Case Report of AA Amyloidosis Associated with Familial Periodic Fever Syndrome Diagnosed After Kidney Transplantation: Never Say Never

Overview
Journal Transplant Proc
Specialty General Surgery
Date 2013 Sep 17
PMID 24034047
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Abstract

Recurrent or "de novo" AA amyloidosis in the renal allograft is rarely described. We describe a case of severe nephrotic syndrome in a recipient of a kidney graft with a previous diagnosis of polycystic nephropathy caused by AA amyloidosis diagnosed only after the renal transplantation. The disease was possibly a tumor necrosis factor receptor-associated periodic syndrome (TRAPS). TRAPS is a rare hereditary inflammatory disease never reported to the best of our knowledge, as a de novo diagnosis in the transplantation setting. Biopsy of the renal graft, indicated for the onset of heavy proteinuria, and genetic investigation provided the clues for diagnosis.