Management of transthyretin amyloidosis

Condoluci, Adalgisa; Théaudin, Marie; Schwotzer, Rahel; Pazhenkottil, Aju P.; Arosio, Paolo; Averaimo, Manuela; Bacher, Ulrike; Bode, P.; Cavalli, Andrea; Dirnhofer, Stefan; Djerbi, Nadia; Dobner, Stephan; Fehr, Thomas; Garofalo, Maura; Gaspert, Ariana; Gerull, Sabine; Heimgartner, Raphael; Hübers, Annemarie; Jung, Hans H.; Kessler, Chiara; Knöpfel, Raphael; Laptseva, Natallia; Magini, Giulia; Manka, Robert; Mazzucchelli, Luca; Meyer, Martin; Mihaylova, Violeta; Monney, Pierre; Mylonas, Alessio; Nkoulou, René; Pabst, Thomas; Pfister, Otmar; Rüfer, Axel; Schmidt, Adrian; Seeger, Harald; Stämpfli, Simon F.; Stirnimann, Guido; Suter, Thomas M.; Treglia, Giorgio; Tzankov, Alexandar; Vetter, Friederike; Zweier, Christiane; Flammer, Andreas J.; Gerber, Bernhard

doi:10.4414/smw.2021.w30053

Cited by 11 publications

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“…This is most likely due to increased awareness and better diagnostic pathways [18]. Patients with heart failure and preserved ejection fraction (HFpEF) within our network undergo systematic evaluation with echocardiography, MRI and, in case of absence of MGUS, bone scintigraphy to detect or rule out transthyretin amyloidosis [19]. We suspect that there will be a further shift towards wild-type transthyretin amyloidosis as the approval of new treatments increases awareness of the disease.…”

Section: Original Articlementioning

confidence: 99%

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Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry

Brouwers,

Heimgartner,

Laptseva

et al. 2024

Swiss Med Wkly

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AIMS OF THE STUDY: Systemic amyloidoses are rare protein-folding diseases with heterogeneous, often nonspecific clinical presentations. To better understand systemic amyloidoses and to apply state-of-the-art diagnostic pathways and treatment, the interdisciplinary Amyloidosis Network was founded in 2013 at University Hospital Zurich. In this respect, a registry was implemented to study the characteristics and life expectancy of patients with amyloidosis within the area covered by the network. Patient data were collected retrospectively for the period 2005–2014 and prospectively from 2015 onwards. METHODS: Patients aged 18 years or older diagnosed with any subtype of systemic amyloidosis were eligible for inclusion if they were treated in one of the four referring centres (Zurich, Chur, St Gallen, Bellinzona). Baseline data were captured at the time of diagnosis. Follow-up data were assessed half-yearly for the first two years, then annually. RESULTS: Between January 2005 and March 2020, 247 patients were screened, and 155 patients with confirmed systemic amyloidosis were included in the present analysis. The most common amyloidosis type was light-chain (49.7%, n = 77), followed by transthyretin amyloidosis (40%, n = 62) and amyloid A amyloidosis (5.2%, n = 8). Most patients (61.9%, n = 96) presented with multiorgan involvement. Nevertheless, single organ involvement was seen in all types of amyloidosis, most commonly in amyloid A amyloidosis (75%, n = 6). The median observation time of the surviving patients was calculated by the reverse Kaplan-Meier method and was 3.29 years (95% confidence interval [CI] 2.33–4.87); it was 4.87 years (95% CI 3.14–7.22) in light-chain amyloidosis patients and 1.85 years (95% CI 1.48–3.66) in transthyretin amyloidosis patients, respectively. The 1-, 3- and 5-year survival rates were 87.0% (95% CI 79.4–95.3%), 68.5% (95% CI 57.4–81.7%) and 66.0% (95% CI 54.6–79.9%) respectively for light-chain amyloidosis patients and 91.2% (95% CI 83.2–99.8%), 77.0% (95% CI 63.4–93.7%) and 50.6% (95% CI 31.8–80.3%) respectively for transthyretin amyloidosis patients. There was no significant difference between the two groups (p = 0.81). CONCLUSION: During registry set-up, a more comprehensive work-up of our patients suffering mainly from light-chain amyloidosis and transthyretin amyloidosis was implemented. Survival rates were remarkably high and similar between light-chain amyloidosis and transthyretin amyloidosis, a finding which was noted in similar historic registries of international centres. However, further studies are needed to depict morbidity and mortality as the amyloidosis landscape is changing rapidly.

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Section: Original Articlementioning

confidence: 99%

“…These practice changes within the network further resulted in a national initiative, where experts from all over Switzerland drew up Guidelines for the treatment of lightchain and transthyretin amyloidosis, also published in Swiss Medical Weekly [19,31].…”

Section: Change Of Practicementioning

confidence: 99%

Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry

Brouwers,

Heimgartner,

Laptseva

et al. 2024

Swiss Med Wkly

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“…In 2021, SAN published a review on the management of ATTR Swiss Amyloidosis Network (SAN): Objectives and Challenges healthbook TIMES Oncology Hematology amyloidosis. 8 Over the years, research activity has increased in Switzerland, with several groups working in basic or translational research, and with the creation of single-center and collaborative clinical projects.…”

Section: The Swiss Amyloidosis Network (San)mentioning

confidence: 99%

Swiss Amyloidosis Network (SAN): Objectives and Challenges

2023

healthbook TIMES Onco Hema

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Systemic amyloidoses are chronic diseases with important consequences for patients' quality of life, thus presenting a major medical and socioeconomic burden. This article discusses the current diagnostic challenges and therapy options for systemic amyloidosis and summarizes the activities of the Swiss Amyloidosis Network (SAN) which aims to increase awareness of these rare diseases, include patients in the Swiss Amyloidosis Registry for research purposes and develop interdisciplinary guidelines for diagnosis and therapy of systemic amyloidosis in Switzerland.

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“…There are two main forms of amyloidosis that significantly affect the heart: light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) [ 4 , 5 ]. The two forms have a completely different pathophysiology and their treatment differs considerably [ 6 , 7 ]. AL type is caused by overproduction and misfolding of antibody light chain fragments by an underlying clonal plasma cell in the bone marrow.…”

Section: Introductionmentioning

confidence: 99%

Arrhythmic Manifestations of Cardiac Amyloidosis: Challenges in Risk Stratification and Clinical Management

Laptseva

Rossi

Sudano

et al. 2023

JCM

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Amylodiosis is a systemic disease characterized by extracellular deposits of insoluble amyloid in various tissues and organs. Cardiac amyloidosis is a frequent feature of the disease, causing a progressive, restrictive type of cardiomyopathy, and is associated with adverse clinical outcomes and increased mortality. The typical clinical presentation in patients with cardiac amyloidosis is heart failure (HF) with preserved ejection fraction. Most patients present with typical symptoms and signs of HF, such as exertional dyspnea, pretibial edema, pleural effusions and angina pectoris due to microcirculatory dysfunction. However, patients may also frequently encounter various arrhythmias, such as atrioventricular nodal block, atrial fibrillation and ventricular tachyarrhythmias. The management of arrhythmias in cardiac amyloidosis patients with drugs and devices is often a clinical challenge. Moreover, predictors of life-threatening arrhythmic events are not well defined. This review intends to give a deepened insight into the arrhythmic features of cardiac amyloidosis by discussing the pathogenesis of these arrhythmias, addressing the challenges in risk stratification and strategies for management in these patients.

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Management of transthyretin amyloidosis

Abstract: This article was corrected and republished online on November 4, 2021. Please see Erratum (Swiss Med Wkly. 2021;151:w30104)

Cited by 11 publications

References 0 publications

Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry

Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry

Swiss Amyloidosis Network (SAN): Objectives and Challenges

Arrhythmic Manifestations of Cardiac Amyloidosis: Challenges in Risk Stratification and Clinical Management

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