Am J Med. 2022 Jan 24:S0002-9343(22)00024-9. doi: 10.1016/j.amjmed.2022.01.003. Online ahead of print.
More than 35 amyloid precursor proteins have been identified and many have tropism for the kidney. Renal amyloidosis is most commonly seen in AL and AA amyloidosis and the main clinical manifestations are proteinuria and progressive renal dysfunction. On renal pathology, hallmark findings of amyloidosis include Congo red positivity with apple-green birefringence and randomly arranged fibrils measuring 7-12 nm in diameter on ultrastructural examination. Management of renal amyloidosis typically combines therapy targeting the underlying amyloid process and supportive management. Patients with renal amyloidosis who progress to end-stage renal disease can be treated with dialysis, and in selected patients, with renal transplantation.