Kidney transplantation as a treatment of choice for AA amyloidosis due to periodic fever syndrome

Renal AA amyloidosis is the most serious complication of periodic fever syndrome, which, inadequate suppression, due to persistent inflammation, leads to nephrotic syndrome and renal failure over several years. In most cases, periodic fever syndromes begin to manifest clinically in early childhood. Occurrence in adulthood is considered rare and is associated with a poorer clinical course. Kidney transplantation (KT) is an effective and safe treatment for end-stage chronic kidney disease (CKD) based on AA amyloidosis. In this paper, we present cases of two patients after deceased donor KT, who have been diagnosed with adult periodic fever syndrome. In the first one, diagnosis and treatment began in advanced stage of CKD and therefore underwent KT with compensated disease, while in the second patient, the disease manifested and diagnosed in the post-KT period. Timely initiation of treatment ensured protection of the graft from amyloid deposition.

Automated summary

Renal AA amyloidosis is the most serious complication of periodic fever syndrome, with most cases manifesting in early childhood. Occurrence in adults is rare and may have a poorer clinical course. Kidney transplantation is an effective treatment for end-stage chronic kidney disease based on AA amyloidosis, with early diagnosis and treatment crucial for protecting the graft from amyloid deposition.