Historic characteristics and mortality of patients in the Swiss Amyloidosis Registry.

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.

Immune Monitoring-Guided Versus Fixed Duration of Antiviral Prophylaxis Against Cytomegalovirus in Solid-Organ Transplant Recipients: A Multicenter, Randomized Clinical Trial.

BACKGROUND: The use of assays detecting cytomegalovirus (CMV)-specific T cell-mediated immunity may individualize the duration of antiviral prophylaxis after transplantation. METHODS: In this randomized trial, kidney and liver transplant recipients from 6 centers in Switzerland were enrolled if they were CMV-seronegative with seropositive donors or CMV-seropositive receiving antithymocyte globulins. Patients were randomized to a duration of antiviral prophylaxis based on immune monitoring (intervention) or a fixed duration (control). Patients in the control group were planned to receive 180 days (CMV-seronegative) or 90 days (CMV-seropositive) of valganciclovir. Patients were assessed monthly with a CMV ELISpot assay (T-Track CMV); prophylaxis in the intervention group was stopped if the assay was positive. The co-primary outcomes were the proportion of patients with clinically significant CMV infection and reduction in days of prophylaxis. Between-group differences were adjusted for CMV serostatus. RESULTS: Overall, 193 patients were randomized (92 in the immune-monitoring group and 101 in the control group), of whom 185 had evaluation of the primary outcome (87 and 98 patients). CMV infection occurred in 26 of 87 (adjusted percentage, 30.9%) in the immune-monitoring group and in 32 of 98 (adjusted percentage, 31.1%) in the control group (adjusted risk difference, -0.1; 95% confidence interval [CI], -13.0% to 12.7%; P = .064). The duration of prophylaxis was shorter in the immune-monitoring group (adjusted difference, -26.0 days; 95%, CI, -41.1 to -10.8 days; P < .001). CONCLUSIONS: Immune monitoring resulted in a significant reduction of antiviral prophylaxis, but we were unable to establish noninferiority of this approach on the co-primary outcome of CMV infection. CLINICAL TRIALS REGISTRATION: NCT02538172.

Stillbirth and fulminant postpartum haemolysis: COVID-19 or leptospirosis or both?

We report the case of a female patient with a SARS-CoV-2 infection first diagnosed at 32 2/7 weeks of gestation, resulting in stillbirth at 33 5/7 weeks of gestation. Post partum the patient presented with severe and persistent haemolysis, mild thrombocytopaenia, renal insufficiency and proteinuria as well as elevated liver enzymes and jaundice. Further investigations revealed a positive IgM for Leptospira interrogans and proof of infection by PCR in the urine. The patient was treated with penicillin for 7 days and received a total of 23 units of red blood cells within 11 days. Haemolysis diminished over time and haemoglobin, proteinuria and transaminases normalised within 23 days after delivery. We suppose an acute leptospirosis as underlying cause for the haemolysis, mimicking pregnancy-associated thrombotic microangiopathy. Whether stillbirth was related to leptospirosis or SARS-CoV-2 infection remains unclear.

Selective Bcl-2 inhibition promotes hematopoietic chimerism and allograft tolerance without myelosuppression in nonhuman primates.

Hematopoietic stem cell transplantation (HSCT) has many potential applications beyond current standard indications, including treatment of autoimmune disease, gene therapy, and transplant tolerance induction. However, severe myelosuppression and other toxicities after myeloablative conditioning regimens have hampered wider clinical use. To achieve donor hematopoietic stem cell (HSC) engraftment, it appears essential to establish niches for the donor HSCs by depleting the host HSCs. To date, this has been achievable only by nonselective treatments such as irradiation or chemotherapeutic drugs. An approach that is capable of more selectively depleting host HSCs is needed to widen the clinical application of HSCT. Here, we show in a clinically relevant nonhuman primate model that selective inhibition of B cell lymphoma 2 (Bcl-2) promoted hematopoietic chimerism and renal allograft tolerance after partial deletion of HSCs and effective peripheral lymphocyte deletion while preserving myeloid cells and regulatory T cells. Although Bcl-2 inhibition alone was insufficient to induce hematopoietic chimerism, the addition of a Bcl-2 inhibitor resulted in promotion of hematopoietic chimerism and renal allograft tolerance despite using only half of the dose of total body irradiation previously required. Selective inhibition of Bcl-2 is therefore a promising approach to induce hematopoietic chimerism without myelosuppression and has the potential to render HSCT more feasible for a variety of clinical indications.

Clinical prediction model for prognosis in kidney transplant recipients (KIDMO): study protocol.

BACKGROUND: Many potential prognostic factors for predicting kidney transplantation outcomes have been identified. However, in Switzerland, no widely accepted prognostic model or risk score for transplantation outcomes is being routinely used in clinical practice yet. We aim to develop three prediction models for the prognosis of graft survival, quality of life, and graft function following transplantation in Switzerland. METHODS: The clinical kidney prediction models (KIDMO) are developed with data from a national multi-center cohort study (Swiss Transplant Cohort Study; STCS) and the Swiss Organ Allocation System (SOAS). The primary outcome is the kidney graft survival (with death of recipient as competing risk); the secondary outcomes are the quality of life (patient-reported health status) at 12 months and estimated glomerular filtration rate (eGFR) slope. Organ donor, transplantation, and recipient-related clinical information will be used as predictors at the time of organ allocation. We will use a Fine & Gray subdistribution model and linear mixed-effects models for the primary and the two secondary outcomes, respectively. Model optimism, calibration, discrimination, and heterogeneity between transplant centres will be assessed using bootstrapping, internal-external cross-validation, and methods from meta-analysis. DISCUSSION: Thorough evaluation of the existing risk scores for the kidney graft survival or patient-reported outcomes has been lacking in the Swiss transplant setting. In order to be useful in clinical practice, a prognostic score needs to be valid, reliable, clinically relevant, and preferably integrated into the decision-making process to improve long-term patient outcomes and support informed decisions for clinicians and their patients. The state-of-the-art methodology by taking into account competing risks and variable selection using expert knowledge is applied to data from a nationwide prospective multi-center cohort study. Ideally, healthcare providers together with patients can predetermine the risk they are willing to accept from a deceased-donor kidney, with graft survival, quality of life, and graft function estimates available for their consideration. STUDY REGISTRATION: Open Science Framework ID: z6mvj.

Serious Games and Gamification: Health Care Workers' Experience, Attitudes, and Knowledge.

OBJECTIVE: With the rapid advancement of digital technology due to COVID-19, the health care field is embracing the use of digital technologies for learning, which presents an opportunity for teaching methods such as serious games to be developed and improved. Technology offers more options for these educational approaches. The goal of this study was to assess health care workers' experiences, attitudes, and knowledge regarding serious games in training. METHODS: The convenience sample consisted of 223 participants from the specialties of internal medicine and psychiatry who responded to questions regarding sociodemographic data, experience, attitudes, and knowledge regarding serious games. This study used an ordinal regression model to analyze the relationship between knowledge, attitudes, and experiences and the idea or wish to implement serious games. RESULTS: The majority of healthcare workers were not familiar with serious games or gamification. The results show gender and age differences regarding familiarity and willingness to use serious games. With increasing age, the respondents preferred conventional and traditional learning methods to playful teaching elements; younger generations were significantly more motivated than older generations when envisioning using elements of serious games in the future. CONCLUSIONS: The COVID-19 pandemic has encouraged the use of new technologies and digitalization. This study describes positive attitudes toward serious games, mainly in younger people working in health care. Serious games present an opportunity to develop new approaches for postgraduate medical teachings and continuing medical education.

Mammalian Target of Rapamycin Inhibitors and Kidney Function After Thoracic Transplantation: A Systematic Review and Recommendations for Management of Lung Transplant Recipients.

BACKGROUND: Chronic kidney disease (CKD) after lung transplantation is common and limits the survival of transplant recipients. The calcineurin inhibitors (CNI), cyclosporine A, and tacrolimus being the cornerstone of immunosuppression are key mediators of nephrotoxicity. The mammalian target of rapamycin (mTOR) inhibitors, sirolimus and everolimus, are increasingly used in combination with reduced CNI dosage after lung transplantation. METHODS: This systematic review examined the efficacy and safety of mTOR inhibitors after lung transplantation and explored their effect on kidney function. RESULTS: mTOR inhibitors are often introduced to preserve kidney function. Several clinical trials have demonstrated improved kidney function and efficacy of mTOR inhibitors. The potential for kidney function improvement and preservation increases with early initiation of mTOR inhibitors and low target levels for both mTOR inhibitors and CNI. No defined stage of CKD for mTOR inhibitor initiation exists, nor does severe CKD preclude the improvement of kidney function under mTOR inhibitors. Baseline proteinuria may negatively predict the preservation and improvement of kidney function. Discontinuation rates of mTOR inhibitors due to adverse effects increase with higher target levels. CONCLUSIONS: More evidence is needed to define the optimal immunosuppressive regimen incorporating mTOR inhibitors after lung transplantation. Not only the indication criteria for the introduction of mTOR inhibitors are needed, but also the best timing, target levels, and possibly discontinuation criteria must be defined more clearly. Current evidence supports the notion of nephroprotective potential under certain conditions.

Peripartum cardiomyopathy: a rare cause of acute heart failure.

A woman in her early 30s presented herself with acute dyspnoea and elevated D-dimers 5 weeks after delivery of her second child. Echocardiographic findings showed signs of acute left ventricular failure, and an MRI confirmed a non-ischaemic dilated left heart failure compatible with peripartum cardiomyopathy. The antihormonal therapy with bromocriptine during 6 weeks and an intensive heart failure therapy led to an amelioration of the heart function within 3 years, but full recovery was not yet observed.

[CME: Neuroborreliosis]. / CME: Neuroborreliose CME-Fragen.

CME: Neuroborreliosis Abstract. Lyme disease is the most common infectious disease transmitted by ticks throughout Europe. Bacteria of the Borrelia burgdorferi complex are transmitted via tick bites to humans. The typical initial presentation is a localized infection of the skin (Erythema migrans). If the patient is not treated with antibiotics, a disseminated infection might occur, presenting as neurological Lyme disease, Lyme carditis, Lyme arthritis or Acrodermatitis chronica atrophicans. A neuroborreliosis occurs in 3-15% of the cases. It may present as polyradiculitis, meningitis or - in rare cases - as encephalomyelitis. The antimicrobial therapy of neuroborreliosis is doxycyclin, ceftriaxone or penicillin G. Prevention is defined by exposure prophylaxis. A vaccination is currently under development in Europe.

[CME: Diagnostic Approach and Management of Thrombotic Microangiopathy]. / CME: Notfallmanagement der thrombotischen Mikroangiopathie.

CME: Diagnostic Approach and Management of Thrombotic Microangiopathy Abstract. Thrombotic microangiopathies (TMA) are characterized by organ thrombosis induced by endothelial injury. They present with thrombocytopenia, hemolytic anemia and schistocytes. In case of an underlying disease-causing TMA, the treatment of the underlying disease is essential. Primary TMAs are divided into thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome and atypical hemolytic-uremic syndrome. Differentiation of these entities is essential, as fast initiation of empiric treatment might be life-saving and disease-modifying treatments for the different entities do exist.

First Testing of Literature-Based Models for Predicting Increase in Body Weight and Adipose Tissue Mass After Kidney Transplantation.

Introduction: Weight gain is a risk factor for poor clinical outcomes following kidney transplantation. Research Question: This study's aim was a first testing of 2 models to identify patients early after kidney transplantation who are at risk for weight gain and increase in adipose tissue mass in the first year after kidney transplantation. Design: The literature-based models were evaluated on longitudinal data of 88, respectively 79 kidney transplant recipients via ordinary and Firth regression, using gains ≥ 5% in weight and adipose tissue mass respectively as primary and secondary endpoints. Results: The models included physical activity, smoking cessation at time of kidney transplantation, self-reported health status, depressive symptomatology, gender, age, education, baseline body mass index and baseline trunk fat as predictors. Area under the curve was 0.797 (95%-CI 0.702 to 0.893) for the weight model and 0.767 (95%-CI 0.656 to 0.878) for the adipose tissue mass model-showing good, respectively fair discriminative ability. For weight gain ≥ 5%, main risk factors were smoking cessation at time of transplantation (OR 16.425, 95%-CI 1.737-155.288) and better self-reported baseline health state (OR 1.068 for each 1-unit increase, 95%-CI 1.012-1.128). For the adipose tissue mass gain ≥ 5%, main risk factor was overweight/obesity (BMI ≥ 25) at baseline (odds ratio 7.659, 95%-CI 1.789-32.789). Conclusions: The models have potential to assess patients' risk for weight or adipose tissue mass gain during the year after transplantation, but further testing is needed before implementation in clinical practice.

Impact of the COVID-19 pandemic on emergency outpatient consultations and admissions of non-COVID-19 patients (ECCO)-A cross-sectional study.

During the first year of the COVID-19 pandemic, healthcare facilities worldwide struggled to adequately care for the increasing number of COVID-19 patients while maintaining quality of care for all other patients. The aim of this study was to investigate the displacement and underuse of non-COVID-19 patient care in a medical department of a tertiary hospital in Switzerland. In this retrospective cross-sectional study, internal medicine admissions from 2017 to 2020, emergency outpatient visits from 2019 to 2020 and COVID-19 admissions in 2020 were analyzed and compared using a regression model. Internal medicine admissions were also stratified by diagnosis. A questionnaire was used to assess the pandemic experience of local general practitioners, referring hospitals, and nursing homes. The total number of admissions decreased during the 1st and 2nd waves of the pandemic but increased between the two waves. Elective admissions decreased in 2020 compared to pre-pandemic years: they represented 25% of total admissions in 2020 versus 30% of the total admissions during 2017-2019, p <0.001. Admissions for emergency reasons increased: 71% in 2020 versus 65% in 2017-2019, p < 0.001. Emergency outpatient consultations decreased in 2020 compared to 2019, 62.77 (14.70), mean (SD), weekly visits in 2020 versus 74.13 (13.98) in 2019, p<0.001. Most general practitioners and heads of referring hospitals also reported a decrease in consultations, especially during the 1st wave of the pandemic. Mental illnesses, anxiety or burn-out were perceived in both patients and staff in general practices and nursing homes. In conclusion, the COVID-19 pandemic negatively affected the care of non-COVID-19 patients, particularly those with chronic illnesses. A shift of health care resources from non-COVID patients to COVID patients was observed. These findings could help institutions better manage such a situation in the future.

Successful Induction of Specific Immunological Tolerance by Combined Kidney and Hematopoietic Stem Cell Transplantation in HLA-Identical Siblings.

Induction of immunological tolerance has been the holy grail of transplantation immunology for decades. The only successful approach to achieve it in patients has been a combined kidney and hematopoietic stem cell transplantation from an HLA-matched or -mismatched living donor. Here, we report the first three patients in Europe included in a clinical trial aiming at the induction of tolerance by mixed lymphohematopoietic chimerism after kidney transplantation. Two female and one male patient were transplanted with a kidney and peripherally mobilized hematopoietic stem cells from their HLA-identical sibling donor. The protocol followed previous studies at Stanford University: kidney transplantation was performed on day 0 including induction with anti-thymocyte globulin followed by conditioning with 10x 1.2 Gy total lymphoid irradiation and the transfusion of CD34+ cells together with a body weight-adjusted dose of donor T cells on day 11. Immunosuppression consisted of cyclosporine A and steroids for 10 days, cyclosporine A and mycophenolate mofetil for 1 month, and then cyclosporine A monotherapy with tapering over 9-20 months. The 3 patients have been off immunosuppression for 4 years, 19 months and 8 months, respectively. No rejection or graft-versus-host disease occurred. Hematological donor chimerism was stable in the first, but slowly declining in the other two patients. A molecular microscope analysis in patient 2 revealed the genetic profile of a normal kidney. No relevant infections were observed, and the quality of life in all three patients is excellent. During the SARS-CoV-2 pandemic, all three patients were vaccinated with the mRNA vaccine BNT162b2 (Comirnaty®), and they showed excellent humoral and in 2 out 3 patients also cellular SARS-CoV-2-specific immunity. Thus, combined kidney and hematopoietic stem cell transplantation is a feasible and successful approach to induce specific immunological tolerance in the setting of HLA-matched sibling living kidney donation while maintaining immune responsiveness to an mRNA vaccine (ClinicalTrials.gov: NCT00365846).

Management of transthyretin amyloidosis.

Transthyretin amyloidosis (ATTR amyloidosis) is a disease caused by deposition of transthyretin fibrils in organs and tissues, which causes their dysfunction. The clinical heterogeneity of ATTR amyloidosis and the variable presentation of symptoms at early disease stages, historically meant treatment delays. Diagnostic tools and therapy options of ATTR amyloidosis have markedly improved in recent years. The first Swiss Amyloidosis Network (SAN) meeting (Zurich, Switzerland, January 2020) aimed to define a consensus statement regarding the diagnostic work-up and treatment for systemic amyloidosis, tailored to the Swiss healthcare system. A consortium of 45 clinicians and researchers from all Swiss regions and universities was selected by the SAN committee to represent all sub-specialty groups involved in care of patients with amyloidosis. A steering committee conducted the literature search and analysis, wrote the critical synthesis and elaborated a list of statements that were evaluated by all the participants. These recommendations will improve outcomes and quality of life for patients with ATTR amyloidosis. A global review of these guidelines is planned every 3 years with a formal meeting of all the involved experts.

[CME/Answers: Giant Cell Arteritis]. / CME/Antworten: Riesenzellarteriitis.

CME/Answers: Giant Cell Arteritis Abstract. Giant cell arteritis (GCA) is the most common vasculitis among patients over the age of 50. Mainly large vessels are targeted. GCA can be differentiated into cranial and extra-cranial types; thus the symptoms can range from headache, blurred vision and jaw claudication to non-specific symptoms like fatigue, polymyalgia and fever. Complications such as an irreversible loss of vision are critical, which is why timeous diagnosis and treatment are essential. There are some recommendations for treatment, but no defined guidelines exist. Steroids have been the standard treatment for the past six decades and remain so, but side effects are common. Tocilizumab represents an alternative and more effective and safer treatment.

[CME: Giant Cell Arteritis]. / CME: Riesenzellarteriitis.

CME: Giant Cell Arteritis Abstract. Giant cell arteritis (GCA) is the most common vasculitis among patients over the age of 50. Mainly large vessels are targeted. GCA can be differentiated into cranial and extra-cranial types; thus the symptoms can range from headache, blurred vision and jaw claudication to non-specific symptoms like fatigue, polymyalgia and fever. Complications such as an irreversible loss of vision are critical, which is why timeous diagnosis and treatment are essential. There are some recommendations for treatment, but no defined guidelines exist. Steroids have been the standard treatment for the past six decades and remain so, but side effects are common. Tocilizumab represents an alternative and more effective and safer treatment.

[CME/Answers: Musculoskeletal Manifestations of Lyme Disease]. / CME/Antworten: Borrelien-Manifestationen am Bewegungsapparat.

Lyme disease is the most common vector-borne illness in Switzerland and Lyme arthritis the most prevalent manifestation of late-stage Lyme disease. It presents as a monoarthitis or oligoarthritis in large joints, often involving the knee. Such a clinical presentation, together with positive Lyme serologies or polymerase chain reactions from synovial fluid/tissue, is considered diagnostic. If there is no tick bite or erythema migrans in the patient's history the diagnosis can be challenging due to the many differential diagnoses. The initial treatment is a prolonged course of oral antibiotics.

[CME: Musculoskeletal Manifestations of Lyme Disease]. / CME: Borrelien-Manifestationen am Bewegungsapparat.

CME: Musculoskeletal Manifestations of Lyme Disease Abstract. Lyme disease is the most common vector-borne illness in Switzerland and Lyme arthritis the most prevalent manifestation of late-stage Lyme disease. It presents as a monoarthitis or oligoarthritis in large joints, often involving the knee. Such a clinical presentation, together with positive Lyme serologies or polymerase chain reactions from synovial fluid/tissue, is considered diagnostic. If there is no tick bite or erythema migrans in the patient's history the diagnosis can be challenging due to the many differential diagnoses. The initial treatment is a prolonged course of oral antibiotics.

Granulomatous interstitial nephritis in a patient with SARS-CoV-2 infection.

BACKGROUND: Acute kidney injury (AKI) associated with severe coronavirus disease 19 (COVID-19) is common and is a significant predictor of morbidity and mortality, especially when dialysis is required. Case reports and autopsy series have revealed that most patients with COVID-19 - associated acute kidney injury have evidence of acute tubular injury and necrosis - not unexpected in critically ill patients. Others have been found to have collapsing glomerulopathy, thrombotic microangiopathy and diverse underlying kidney diseases. A primary kidney pathology related to COVID-19 has not yet emerged. Thus far direct infection of the kidney, or its impact on clinical disease remains controversial. The management of AKI is currently supportive. CASE PRESENTATION: The patient presented here was positive for SARS-CoV-2, had severe acute respiratory distress syndrome and multi-organ failure. Within days of admission to the intensive care unit he developed oliguric acute kidney failure requiring dialysis. Acute kidney injury developed in the setting of hemodynamic instability, sepsis and a maculopapular rash. Over the ensuing days the patient also developed transfusion-requiring severe hemolysis which was Coombs negative. Schistocytes were present on the peripheral smear. Given the broad differential diagnoses for acute kidney injury, a kidney biopsy was performed and revealed granulomatous tubulo-interstitial nephritis with some acute tubular injury. Based on the biopsy findings, a decision was taken to adjust medications and initiate corticosteroids for presumed medication-induced interstitial nephritis, hemolysis and maculo-papular rash. The kidney function and hemolysis improved over the subsequent days and the patient was discharged to a rehabilitation facility, no-longer required dialysis. CONCLUSIONS: Acute kidney injury in patients with severe COVID-19 may have multiple causes. We present the first case of granulomatous interstitial nephritis in a patient with COVID-19. Drug-reactions may be more frequent than currently recognized in COVID-19 and are potentially reversible. The kidney biopsy findings in this case led to a change in therapy, which was associated with subsequent patient improvement. Kidney biopsy may therefore have significant value in pulling together a clinical diagnosis, and may impact outcome if a treatable cause is identified.

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

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.