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

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Etat: Public
Version: de l'auteur⸱e
Licence: CC BY-NC-SA 4.0
ID Serval
serval:BIB_BFDED8F49F10
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis.
Périodique
Swiss medical weekly
Auteur⸱e⸱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
Statut éditorial
Publié
Date de publication
30/11/2020
Peer-reviewed
Oui
Volume
150
Pages
w20364
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: epublish
Résumé
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.
Mots-clé
General Medicine
Pubmed
Web of science
Open Access
Oui
Création de la notice
17/12/2020 14:39
Dernière modification de la notice
25/07/2022 6:37
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