Kidney amyloidosis. An algorithm for nephrologist

Abstract

Amyloidosis is a heterogeneous group of protein misfolding diseases characterized by deposition of insoluble amyloid proteins in extracellular space that may cause organ dysfunction. At least 30 different amyloidogenic proteins have been identified in humans. Kidneys are affected in 50–80% of amyloidosis AL cases and 97% in amyloidosis AA at the moment of diagnosis. Patients are usually consulted with nephrologist for the evaluation of proteinuria/nephrotic syndrome. The gold standard for diagnosis of renal amyloidosis is kidney biopsy. The subtype of amyloid protein may be differentiated by e.g. mass spectroscopy. The treatment of amyloidosis depends on the underlying disease that cause amyloid formation.