Zasady klasyfikacji i nazewnictwa amyloidoz

Abstract

Amyloidosis is a heterogeneous group of diseases in which symptoms develop due to deposition of amyloid fibril proteins in the extracellular matrix of tissues and organs, as a result of protein misfolding. Because of the rareness, differential clinical manifestations and limited knowledge concerning the pathogenesis of amyloidoses of particular types, multiple medical terms developed in the professional literature to describe the same disease entity. However, with the increasing understanding of molecular basis of amyloidosis, it has become possible to standardize the terminology according to the structure of their chemical precursors. Currently recommended terminology of amyloid fibril proteins and amyloidosis is based on a regularly updated classification of the International Society of Amyloidosis (ISA). In accordance with the ISA definition, amyloid fibril protein deposits in the extracellular matrix in the form of amyloid, prone to Congo red staining and characterized by biofringence under polarized light. The current work presents the classification of so far identified amyloid fibril proteins and amyloidoses related to these proteins. General use of standardized amyloidosis terminology may be a significant facilitation in clinical practice, as well as in research.