Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis.

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Version: author
License: CC BY-NC-SA 4.0
Serval ID
serval:BIB_BFDED8F49F10
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis.
Journal
Swiss medical weekly
Author(s)
Schwotzer R., Flammer A.J., Gerull S., Pabst T., Arosio P., Averaimo M., Bacher V.U., Bode P., Cavalli A., Concoluci A., Dirnhofer S., Djerbi N., Dobner S.W., Fehr T., Garofalo M., Gaspert A., Heimgartner R., Hübers A., Jung H.H., Kessler C., Knöpfel R., Laptseva N., Manka R., Mazzucchelli L., Meyer M., Mihaylova V., Monney P., Mylonas A., Nkoulou R., Pazhenkottil A., Pfister O., Rüfer A., Schmidt A., Seeger H., Stämpfli S.F., Stirnimann G., Suter T., Théaudin M., Treglia G., Tzankov A., Vetter F., Zweier M., Gerber B.
ISSN
1424-3997 (Electronic)
ISSN-L
0036-7672
Publication state
Published
Issued date
30/11/2020
Peer-reviewed
Oui
Volume
150
Pages
w20364
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Systemic amyloidosis is a heterogeneous group of diseases associated with protein misfolding into insoluble beta-sheet rich structures that deposit extracellularly in different organs, eventually compromising their function. There are more than 30 different proteins, known to be amyloidogenic with “light chain” (AL)-amyloidosis being the most common type, followed by transthyretin (ATTR)-, and amyloid protein A (AA)-amyloidosis. Systemic amyloidosis is a rare disease with an incidence of around 10 patients in 1 million inhabitants. Recently several new therapeutic options have been developed for subgroups of amyloidosis patients, and the introduction of novel therapies for plasma cell myeloma has led to an increase in the therapeutic armamentarium for plasma cell disorders, including AL amyloidosis. Among them, proteasome inhibitors, immunomodulatory agents (-imids), and monoclonal antibodies have been successfully introduced into clinical practice. Still, high-quality data from randomised controlled trials regarding the benefit of these cost-intensive drugs in AL amyloidosis are widely lacking, and due to the rarity of the disease many physicians will not gain routine experience in the management of these frail patients. The diagnosis of AL amyloidosis relies on a close collaboration between clinicians, pathologists, imaging experts, and sometimes geneticists. Diagnosis and treatment options in this complex disorder should be discussed in dedicated multidisciplinary boards. In January 2020, the first meeting of the Swiss Amyloidosis Network took place in Zurich, Switzerland. One aim of this meeting was to establish a consensus guideline regarding the diagnostic work-up and the treatment recommendations for systemic amyloidosis tailored to the Swiss health care system. Forty-five participants from different fields in medicine discussed many aspects of amyloidosis. These are the Swiss Amyloidosis Network recommendations which focus on diagnostic work-up and treatment of AL-amyloidosis.
Keywords
General Medicine
Pubmed
Web of science
Open Access
Yes
Create date
17/12/2020 14:39
Last modification date
25/07/2022 6:37
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