[Similarities and differences in the pathophysiology and epidemiology of systemic amyloidoses Focus on immunoglobulin light-chain amyloidosis, hereditary transthyretin amyloidosis and wild-type transthyretin amyloidosis].

Authors

Max Rieger, Rahel Schwotzer

Published in

Therapeutische Umschau. Revue therapeutique. Volume 83. Issue 5. Pages 158-161.

Abstract

Systemic amyloidoses are diseases characterised by the misfolding of soluble proteins and their deposition as amyloid fibrils in the extracellular compartment. Despite sharing a common final pathogenetic mechanism, the individual forms of amyloidosis differ in terms of the origin of the precursor protein, organ involvement, epidemiology and treatment modalities. Immunoglobulin light chain (AL) amyloidosis derives from a monoclonal plasma cell or mature B-cell clone. Hereditary transthyretin amyloidosis (ATTRv) is caused by pathogenic variants in the transthyretin gene, whilst wild-type ATTR amyloidosis (ATTRwt) is an age-related condition with no genetic cause. With the introduction of new disease-specific therapies, the early and accurate classification of amyloidosis has become increasingly important. This review article summarises the common pathophysiology as well as the key differences between AL, ATTRv and ATTRwt amyloidosis in terms of pathogenesis and epidemiology.

PMID:
42979800
Bibliographic data and abstract were imported from PubMed on 04 Nov 2026.

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