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

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.

• Authors: Rahel Schwotzer, Andreas J Flammer, Sabine Gerull, Thomas Pabst, Paolo Arosio, Manuela Averaimo, Vera Ulrike Bacher, Peter Bode, Andrea Cavalli, Adalgisa Concoluci, Stefan Dirnhofer, Nadia Djerbi, Stefan W Dobner, Thomas Fehr, Maura Garofalo, Ariana Gaspert, Raphael Heimgartner, Annemarie Hübers, Hans H Jung, Chiara Kessler, Raphael Knöpfel, Natallia Laptseva, Robert Manka, Luca Mazzucchelli, Martin Meyer, Violeta Mihaylova, Pierre Monney, Alessio Mylonas, René Nkoulou, Aju Pazhenkottil, Otmar Pfister, Axel Rüfer, Adrian Schmidt, Harald Seeger, Simon F Stämpfli, Guido Stirnimann, Thomas Suter, Marie Théaudin, Giorgio Treglia, Alexandar Tzankov, Friederike Vetter, Markus Zweier, Bernhard Gerber
• Journal: Swiss medical weekly (Swiss Med Wkly) Vol. 150 Pg. w20364 (Nov 30 2020) ISSN: 1424-3997 [Electronic] Switzerland
• PMID: 33277911 (Publication Type: Journal Article)

Topics

• Amyloidosis (drug therapy)
• Humans
• Immunoglobulin Light-chain Amyloidosis (diagnosis, drug therapy)
• Multiple Myeloma
• Switzerland