Swiss Medical Weekly (Dec 2020)
Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis
- Rahel Schwotzer,
- Andreas J. Flammer,
- Sabine Gerull,
- Thomas Pabst,
- Paolo Arosio,
- Manuela Averaimo,
- Ulrike Bacher,
- Peter Bode,
- Andrea Cavalli,
- Adalgisa Condoluci,
- Stefan Dirnhofer,
- Nadia Djerbi,
- Stephan 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 P. 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
Affiliations
- Rahel Schwotzer
- Department of Medical Oncology and Haematology, University Hospital Zurich, Switzerland
- Andreas J. Flammer
- Department of Cardiology, University Hospital Zurich, Switzerland
- Sabine Gerull
- Department of Haematology, University Hospital Basel, Switzerland
- Thomas Pabst
- Department of Oncology, Inselspital, University Hospital and University of Bern, Switzerland
- Paolo Arosio
- Department of Chemistry and Applied Biosciences. ‘ETHZ’, Zurich, Switzerland
- Manuela Averaimo
- Cardiocentro Ticino, Lugano, Switzerland
- Ulrike Bacher
- Department of Haematology, Inselspital, University Hospital and University of Bern, Switzerland
- Peter Bode
- Department of Pathology and Molecular Pathology, University Hospital and University of Zurich, Switzerland
- Andrea Cavalli
- Institute for Research in Biomedicine, Università della Svizzera Italiana, Bellinzona, Switzerland; Swiss Institute of Bioinformatics, Lausanne, Switzerland
- Adalgisa Condoluci
- Division of Haematology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland
- Stefan Dirnhofer
- Department of Medical Genetics and Pathology, University Hospital and University of Basel, Switzerland
- Nadia Djerbi
- Department of Medical Oncology and Haematology, University Hospital Zurich, Switzerland
- Stephan W. Dobner
- Department of Cardiology, Inselspital, University Hospital and University of Bern, Switzerland
- Thomas Fehr
- Department of Internal Medicine, Cantonal Hospital Graubünden, Chur, Switzerland
- Maura Garofalo
- Institute for Research in Biomedicine, Università della Svizzera Italiana, Bellinzona, Switzerland
- Ariana Gaspert
- Department of Pathology and Molecular Pathology, University Hospital and University of Zurich, Switzerland
- Raphael Heimgartner
- Department of Internal Medicine, Cantonal Hospital Graubünden, Chur, Switzerland
- Annemarie Hübers
- Department of Neurology, University Hospital and University of Geneva, Switzerland
- Hans H. Jung
- Department of Neurology, University Hospital and University Zurich, Switzerland
- Chiara Kessler
- Division of Haematology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland
- Raphael Knöpfel
- Department of Internal Medicine, Cantonal Hospital Graubünden, Chur, Switzerland
- Natallia Laptseva
- Department of Cardiology, University Hospital Zurich, Switzerland
- Robert Manka
- Department of Cardiology, University Hospital Zurich, Switzerland; Departement of Nuclear Medicine, University Hospital and University of Zurich, Switzerland
- Luca Mazzucchelli
- Cantonal Institute of Pathology, Locarno, Switzerland
- Martin Meyer
- Department of Cardiology, University Hospital Zurich, Switzerland
- Violeta Mihaylova
- Department of Neurology, University Hospital and University Zurich, Switzerland
- Pierre Monney
- Department of Cardiology, Lausanne University Hospital and University of Lausanne, Switzerland
- Alessio Mylonas
- Institute of Bioengineering, Ecole Polytechnique Fédérale de Lausanne (EPFL), Lausanne, Switzerland
- René Nkoulou
- Department of Cardiology, University Hospital and University of Geneva, Switzerland
- Aju P. Pazhenkottil
- Department of Cardiology, University Hospital Zurich, Switzerland
- Otmar Pfister
- Department of Cardiology, University Hospital and University of Basel, Switzerland
- Axel Rüfer
- Department of Haematology, Luzerner Kantonsspital, Lucerne, Switzerland
- Adrian Schmidt
- Department of Internal Medicine, Clinic for Medical Oncology and Haematology, City Hospital Waid and Triemli, Zurich, Switzerland
- Harald Seeger
- Department of Nephrology, University Hospital and University Zurich, Switzerland
- Simon F. Stämpfli
- Department of Cardiology, Heart Centre Lucerne, Luzerner Kantonsspital, Lucerne, Switzerland
- Guido Stirnimann
- University Clinic for Visceral Surgery and Medicine, University Hospital Inselspital and University of Bern, Switzerland
- Thomas Suter
- Department of Cardiology, Inselspital, University Hospital and University of Bern, Switzerland
- Marie Théaudin
- Department of Neurology, University Hospital and University of Lausanne, Switzerland
- Giorgio Treglia
- Clinic of Nuclear Medicine, Imaging Institute of Southern Switzerland, Ente Ospedaliero Cantonale, Bellinzona, Switzerland; Department of Nuclear Medicine and Molecular Imaging, University Hospital and University of Lausanne, Switzerland
- Alexandar Tzankov
- Department of Medical Genetics and Pathology, University Hospital and University of Basel, Switzerland
- Friederike Vetter
- Department of Medical Oncology and Haematology, University Hospital Zurich, Switzerland
- Markus Zweier
- Institute of Medical Genetics, University of Zurich, Switzerland
- Bernhard Gerber
- Division of Haematology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland
- DOI
- https://doi.org/10.4414/smw.2020.20364
- Journal volume & issue
-
Vol. 150,
no. 4950
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.
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