Amyloidosen – Klassifikation, Diagnostik und Therapie

Amyloidoses are rare protein-misfolding disorders characterized by the deposition of insoluble fibrillar aggregates, leading to potentially life-threatening organ damage. These deposits can be systemic or localized. Systemic amyloidoses are often associated with monoclonal gammopathies, chronic inflammatory conditions, or have a hereditary origin. Treatment strategies primarily focus on reducing the production of amyloidogenic proteins—depending on the specific protein and its origin—through therapies such as chemotherapy, anti-inflammatory agents, or gene-silencing approaches. Early diagnosis is crucial to prevent progressive organ dysfunction. Over the past 25 years, significant advances in the diagnosis and therapeutic management of amyloidosis have been made.