Abstract
Background
Data on the epidemiology of amyloidosis are scarce worldwide, making it difficult to understand its true incidence and mortality.
Aim
The aim of this study is to estimate the incidence and mortality rate of systemic amyloidosis in people affiliated to a Medical Care Program in the city of Buenos Aires, Argentina.
Methods
This is a hospital-based prospective study. All affiliates over 17 years to the Medical Care Program of the Hospital Italiano de Buenos Aires, and incident cases and deaths due to amyloidosis from the Institucional Amyloidosis Registry from 1 January 2011 to 31 December 2022 were included. Incidence and mortality rates were reported per million person-year with their respective 95% CIs. Crude, standardised and stratified rates were determined.
Results
During the study period, the crude incidence rate was 63 (95% CI 52 to 76) and the crude mortality rate was 31 (95% CI 23 to 40) cases per million person-year, with the wild-type transthyretin amyloidosis (ATTRwt) subtype having the highest rates. Men were more affected, especially from the age of 70 years onwards. In addition, an increase in the incidence and mortality rate was observed throughout the period studied.
Conclusion
The results of this study indicate that amyloidosis remains a rare disease but with an increase in incidence in recent years, especially in elderly people. This underlines the importance of further research in epidemiology for a better understanding of the pathology and its evolution.
Keywords: Epidemiologic Measurements, Epidemiology, Public Health
WHAT IS ALREADY KNOWN ON THIS TOPIC
The incidence of amyloidosis is well known in various regions, but data for Latin America are lacking.
WHAT THIS STUDY ADDS
The findings reveal an increase in amyloidosis incidence in recent years, particularly the ATTRwt subtype.
HOW THIS STUDY MIGHT AFFECT RESEARCH, PRACTICE OR POLICY
A multidisciplinary approach is crucial for improving the quality of life for individuals affected by this rare disease.
Introduction
Systemic amyloidosis is a heterogeneous group of rare diseases characterised by the extracellular deposition of misfolded proteins called amyloid fibrils.1 This deposition causes tissue infiltration and, in certain types of amyloidosis, the direct toxic effect of the circulating precursor further contributes to cellular dysfunction.2,4
Several subtypes of amyloidosis are classified according to the type of protein deposited,1 the most frequent being amyloidosis produced by immunoglobulin light chains (AL amyloidosis), non-hereditary transthyretin deposition (ATTRwt amyloidosis), and amyloidosis generated by serum amyloid protein A (AA amyloidosis).5
Despite the difficulty in obtaining accurate data due to the rare nature of the disease and the lack of registries and data in the world, several epidemiological studies have provided relevant estimates about the incidence of amyloidosis. In a population-based study conducted by the Mayo Clinic in Olmsted County, Minnesota, USA, the incidence of amyloidosis was estimated at 6–10 cases per million person/year.6 In the UK, a study by the National Amyloidosis Centre estimated an annual incidence of 8 cases per million person/year, with AL amyloidosis being the most frequent.7 In Sweden, an annual incidence rate of 8 cases per million person/year was reported for non-hereditary amyloidosis and 1–2 cases per million person/year for AA amyloidosis.8 In Latin America, an epidemiological study conducted by the Amyloidosis Study Group in Argentina estimated an annual incidence rate of 14 cases per million person/year for ATTRwt amyloidosis, 11 cases per million for AL amyloidosis and 2 cases per million person/year for AA amyloidosis.5
In its evolution, amyloidosis is a potentially fatal entity, and morbidity and mortality are generally related to the dysfunction of the affected organ and the subtype of amyloidosis.9 10 A US study used death certificate information from the Centre for Disease Control and Prevention and National Vital Statistics System epidemiological research database from 1979 to 2015 and reported a mortality rate of 4 deaths per million person/year in the general population, with counties reporting rates as high as 32 deaths per million person/year.11
The limited knowledge of the current epidemiology of amyloidosis represents a significant obstacle to the effective management of this disease. That is why we conducted a cohort study using data from the Medical Care Program (MCP) of the Hospital Italiano de Buenos Aires (HIBA) to estimate the incidence and mortality rates of amyloidosis during the period 2011–2022.
Materials and methods
Design and population
This is a hospital-based prospective study. All HIBA MCP affiliates over 17 years of age during the period corresponding to 1 January 2011 until 31 December 2022 were included. The MCP is a prepaid healthcare provider that offers medical care to its beneficiaries through two hospitals, one of them highly specialised, and 24 peripheral ambulatory care centres located in the Buenos Aires metropolitan area. All healthcare interventions for beneficiaries are centrally recorded in a computerised data repository, with one electronic medical record per person.
Because the MCP population is a dynamic cohort, each individual contributed a certain time at risk from January 2011 or the date of MCP enrolment to the date of diagnosis of amyloidosis or death or loss to follow-up or discharge from MCP or administrative closure of the study.
The manuscript adheres to the Strengthening the Reporting of Observational Studies in Epidemiology guidelines.
Amyloidosis case and incident case detection
Incident cases of amyloidosis were identified from the Institutional Amyloidosis Registry (IAR). An incident case was determined as the confirmation of amyloidosis whose date of diagnosis was after the date of affiliation to the MCP. The date of diagnosis of amyloidosis or the date of death from any cause was used as the date of the incident event. The algorithm previously published by the Amyloidosis Study Group was used to detect cases of amyloidosis.5
Briefly, incident cases of amyloidosis captured in the IAR include patients over 17 years of age with a confirmed diagnosis from any hospital department. Possible cases are detected through an alert system when amyloidosis is listed as a diagnosis or when studies such as kappa and lambda light chain concentrations, kappa ratio, transthoracic Doppler echocardiography, cardiovascular MRI, or bone scintigraphy with pyrophosphate are requested. A prospective review of electronic medical records is then conducted to confirm the diagnosis of amyloidosis.
Amyloid typing was based on clinical criteria derived from available studies in the region, as well as the response to treatment, according to established criteria:
ATTRwt amyloidosis: bone scintigraphy with pyrophosphate or cardiac resonance or echocardiogram compatible, and negative studies for TTR mutations, and normal free light chain ratio.12
AA amyloidosis: immunohistochemical staining positive for AA amyloidosis.
AL amyloidosis: elevated free light chains with impaired kappa/lambda ratio and serum and urine immunofixation and compatible symptoms and signs.13
Source of data
All data were collected from the HIBA and IAR hospital databases of MCP affiliates.
Statistical analysis
To calculate the incidence and mortality rates, the following standard formula was used: the crude incidence rate was calculated by dividing the number of new cases during the study period by the total person-years at risk during the same period, multiplied by 1 000 000. The stratified incidence rate was calculated similarly, but considering different categories of age, gender, and/or amyloidosis subtype, using the total person-years at risk within each stratum. Similarly, the crude mortality rate was calculated by dividing the number of deaths in patients with amyloidosis during the study period by the total person-years at risk during that same period, multiplied by 1 000 000. The stratified mortality rate was calculated similarly, considering age, gender, and/or amyloidosis subtype categories, using the total person-years at risk within each stratum.
In the case of ATTRwt amyloidosis, the people at risk are those over 60 years of age. Therefore, for this subtype, the denominator used was people affiliated to the MCP corresponding to this age group. Both incident and mortality rates were estimated for the entire study period (2011–2022) and by quadrennium (2011–2014; 2015–2018; 2019–2022), and rates stratified by age, sex and amyloidosis subtype were calculated.
Finally, rates were reported per million person/year with their respective 95% CI.
Results
Participants
During the 12-year study period, a total of 247.286 MCP affiliates over 17 years of age were identified, of which 105 were diagnosed with amyloidosis.
Incidence rates
During the period between 2011 and 2022, the crude incidence rate was 63 (95% CI 52 to 76) cases per million person/year, showing an increase throughout the study (incidence rate ratio 0.31 95% CI 0.17 to 0.54 when comparing the first period with the third). Punctually, an incidence (cases per million person/year) of 33 (95% CI 20 to 53) between 2011 and 2014, 47 (95% CI 32 to 68) between 2015 and 2018, and 105 (95% CI 82 to 134) between 2019 and 2022 were estimated (figure 1).
Figure 1. (A) Crude incidence and mortality rates for amyloidosis by quadrennium. (B) Diagnostic and treatment milestones that were incorporated in the institution during the period studied. ANMAT, National Food, Drug and Medical Technology Administration; CMR, cardiac magnetic resonance; IHC, immunohistochemistry; TTR, transthyretin.
Among the incidence rates for each subtype, ATTRwt amyloidosis was the most frequent, with an incidence of 66 (95% CI 52 to 85) cases per million person/year, while for AL amyloidosis it was 16 (95% CI 11 to 24) and for AA amyloidosis 4 (95% CI 2 to 9) cases per million person/year.
When rates per quadrennium were determined, an increase was observed throughout the study for ATTRwt amyloidosis (IRR 0.14 95% CI 0.05 to 0.33), while the other amyloidosis subtypes maintained a stable incidence (figure 2). Specifically, for ATTRwt amyloidosis, rates of 19 (95% CI 9 to 43) cases per million person/year were recorded in the period 2011–2014; 45 (95% CI 26 to 75) cases in the period 2015–2018, and 139 (95% CI 103 to 189) cases in the period 2019–2022.
Figure 2. Incidence rate (cases per million person/years) by quadrennium for each subtype of amyloidosis. Period 2011–2014: AL amyloidosis=13.4 (95% CI 6.4 to 28.2), ATTRwt amyloidosis=19.4 (95% CI 8.7 to 43.1), ATTRv amyloidosis=no incidents, AA amyloidosis=1.9 (95% CI 0.3 to 13.6). Period 2015–2018: AL amyloidosis=14.3 (95% CI 7.2 to 28.6), ATTRwt amyloidosis=44.5 (95% CI 26.3 to 75.1), ATTRv amyloidosis=no incidents, AA amyloidosis=3.6 (95% CI 0.9 to 14.3). Period 2019–2022: AL amyloidosis=20.2 (95% CI 11.5 to 35.6), ATTRwt amyloidosis=139.4 (95% CI 102.7 to 189.4) ATTRv amyloidosis=1.7 (95% CI 0.2 to 12.0), AA amyloidosis=6.7 (95% CI 2.5 to 18.0). AA, amyloid protein A amyloidosis; AL, immunoglobulin light chain amyloidosis; ATTRwt, wild type transthyretin amyloidosis; ATTRv, variant transthyretin amyloidosis.
About the global incidence rates stratified by age and sex, the incidence was higher among males in the age stratum exceeding 80 years. In addition, the AL amyloidosis subtype showed cases at younger ages in women, while ATTRwt amyloidosis occurred more frequently and at younger ages in men. For its part, ATTRv was diagnosed in a woman between 70 and 79 years of age. Concerning AA amyloidosis, cases were recorded in younger men; however, there were no differences in incidence between sexes (table 1).
Table 1. Incidence rates per million (95% CI) stratified by age, gender and amyloidosis subtype during the study period 2011–2022.
| Age stratum(years) | 50–59 | 60–69 | 70–79 | Over 80 |
|---|---|---|---|---|
| All amyloidosis subtypes | ||||
| Men | – | 77.6 (38.8–155.2) | 126.8 (72.1–223.4) | 728.8 (550.8–964.2) |
| Women | 16.3 (4.1–65.2) | 46.2 (23.1–92.4) | 61.7 (34.2–111.4) | 78.5 (46.5–132.5) |
| IRR | – | 1.68 (0.55–5.13) | 2.06 (0.83–5.14) | 9.29 (5.05–18.22) |
| AL amyloidosis | ||||
| Men | – | 38.8 (14.6–103.4) | 42.2 (15.9–112.6) | 29.8 (7.4–119.0) |
| Women | 16.3 (4.1–65.2) | 34.7 (15.6–77.1) | 22.4 (8.4–59.8) | 280 (11.7–67.3) |
| IRR | – | 1.12 (0.23–4.72) | 1.88 (0.35–10.11) | 1.06 (0.10–6.48) |
| ATTRwt amyloidosis | ||||
| Men | – | 19.0 (4.9–77.8) | 73.9 (35.3–15.5) | 669.3 (499.7–896.5) |
| Women | – | – | 11.2 (2.8–44.8) | 28.0 (11.7–67.3) |
| IRR | – | – | 6.60 (1.26–65.10) | 23.88 (9.51–77.10) |
| ATTRv amyloidosis | ||||
| Men | – | – | – | – |
| Women | – | – | 5.6 (0.8–39.8) | – |
| IRR | – | – | – | – |
| AA amyloidosis | ||||
| Men | – | 9.7 (1.3–6.9) | 10.6 (1.5–7.5) | – |
| Women | – | – | 16.8 (5.42–5.2) | 11.2 (2.8–44.8) |
| IRR | – | – | 0.63 (0.01–7.82) | – |
IRR, incidence rate ratio.
Mortality rates
Between 2011 and 2022, the crude mortality rate due to amyloidosis was 31 (95% CI 23 to 40) cases per million person/year, showing an increase throughout the study period (IRR 0.23 95% CI 0.08 to 0.56 when comparing the first period with the third). A rate of 12 (95% CI 5 to 26) cases per million person/year between 2011 and 2014, 27 (95% CI 16 to 44) between 2015 and 2018 and 51 (95% CI 35 to 72) between 2019 and 2022 were estimated (figure 1).
When calculating mortality rates by subtype, ATTRwt amyloidosis showed a rate of 15 (95% CI 10 to 22), AL amyloidosis 11 (95% CI 7 to 17) and AA amyloidosis 3 (95% CI 1 to 7) deaths per million person/year.
Regarding mortality rates stratified by age and sex, this was higher in men older than 80 years. The ATTRwt amyloidosis subtype showed a higher rate in men older than 80 years, while in the AL amyloidosis subtype, no differences between sexes were found, except for deaths at younger ages in women. As for AA amyloidosis, deaths were recorded only in women (table 2).
Table 2. Mortality rates per million (95% CI) stratified by age, gender and amyloidosis subtype during the study period 2011–2022.
| Age stratum(years) | 50–59 | 60–69 | 70–79 | Over 80 |
|---|---|---|---|---|
| All amyloidosis subtypes | ||||
| Men | – | 19.4 (4.8–77.5) | 63.3 (28.4–141.0) | 356.8 (239.1–532.3) |
| Women | 8.1 (1.1–57.8) | 11.6 (2.9–46.2) | 44.8 (22.4–89.6) | 44.8 (22.4–89.6) |
| IRR | – | 1.68 (0.12–23.16) | 1.41 (0.40–4.64) | 7.96 (3.46–20.49) |
| AL amyloidosis | ||||
| Men | – | 19.4 (4.8–77.5) | 31.6 (10.2–98.2) | 29.7 (7.4–118.9) |
| Women | 8.1 (1.1–57.8) | 11.6 (2.9–46.2) | 28.0 (11.7–67.3) | 16,8 (5,4–52,1) |
| IRR | – | 1.68 (0.12–23.16) | 1.13 (0.17–5.81) | 1.77 (015–15.44) |
| ATTRwt amyloidosis | ||||
| Men | – | – | 31.7 (102–98.2) | 297.3 (191.8–480.9) |
| Women | – | – | – | 11.2 (2.8–44.8) |
| IRR | – | – | – | 26.53 (6.44–234.12) |
| AA amyloidosis | ||||
| Men | – | – | – | – |
| Women | – | – | 16.8 (5.42–5.2) | 11.2 (2.8–44.8) |
| IRR | – | – | – | – |
IRR, incidence rate ratio.
Discussion
The results of this study show that amyloidosis is an infrequent health problem in the population studied, consistent with the findings of other studies (see table 3).68 14,22 As shown in this table, the incidence rates of different types of amyloidosis vary across various studies. These data highlight the variability in incidence rates, which range widely depending on the type of amyloidosis and the specific study. For instance, AL amyloidosis shows incidences between 6 and 16.7 cases per 1 000 000 person-years, while ATTRwt amyloidosis ranges from 14.4 to 66 cases per 1 000 000 person-years. Less common forms, such as ATTRv amyloidosis, exhibit incidences between 0.8 and 2.7 cases per 1 000 000 person-years, and secondary amyloidosis is reported at 1.18 cases per 1 000 000 person-years. However, it is important to note that these data represent crude incidence rates, which may limit comparability between studies due to variability from differences in study periods, geographical characteristics and population composition.
Table 3. Annual incidence of systemic amyloidosis in different regions.
| Authors and year | Country | Period | Annual incidence |
|---|---|---|---|
| Posadas-Martínez et al, current study | Argentina | 2011–2022 | ATTRwt amyloidosis (over 60 years): 66/1 000 000 person–yearsAL amyloidosis: 16/1 000 000 person–yearsAA amyloidosis: 4/1 000 000 person–years |
| Kyle et al, 1992 | USA | 1950–1989 | AL amyloidosis: 6.0 to 10.5/1 000 000 person–years6 |
| Laires et al, 202315 | USA | AL amyloidosis: 16.7/1 000 000 person–years15 | |
| Cappelli et al, 202316 | Italy | 2006–2022 |
|
| Mellqvist et al, 202317 | Swedish | 2011–2019 | AL amyloidosis: 10.5 to 15.1/1 000 000 person-years17 |
| Dammy et al, 202318 | France | 2011–2019 | ATTR-CA: 0.6 to 3.6/100 000 person-years18 |
| Hemminki et al, 20128 | Swedish | 2001–2008 |
|
| Pinney et al, 20137 | UK | 2000–2008 |
|
| Laires et al, 202419 | USA | 2018–2022 | ATTR-CA: 6/1 000 000 person-years19 |
| Gilstrap et al, 201920 | USA | 2000–2012 | ATTR-CA: 1.8 to 5.5/1 000 000 person-years20 |
| Guha et al, 202421 | UK | 2004–2021 | ATTR-CA: 0.7–1.96/100 000 person-years21 |
| Choi et al, 202422 | Korea | 2008–2020 | ATTR-CA: 0.09–0.22/100 000 person-years22 |
| Quock et al, 201814 | USA | 2007–2015 | Amyloidosis AL: 9.7 to 14.0/1 000 000 person-years14 22 |
| Brink et al | Netherlands | 2017–2018 | Amyloidosis AL: 6.0/1 000 000 person-years |
Regarding the incidence by subtypes in our population, the AL amyloidosis rate remained stable, while ATTRwt amyloidosis showed a significant increase in the last quadrennium, which is similar to that reported by other studies.14 23 We believe that this increased incidence of ATTRwt amyloidosis could be due to the higher detection and accurate characterisation of this subtype. Regarding the incidence of ATTRv amyloidosis, it is important to highlight that this result is influenced by the study design, which only includes incident cases of amyloidosis in patients affiliated with the MCP. Currently, only one case of ATTRv amyloidosis has been diagnosed among MCP-affiliated patients. This low number of cases may reflect a low prevalence of the disease in this specific population and not necessarily an underestimation of the total number of cases in the general population of the region. Therefore, these results should be interpreted with caution.
About the mortality rate, it increased throughout the period, concomitant with other studies.11 24 Regarding the mortality rate stratified by age and sex, it was higher in men over 80 years of age, similar to those reported by Harada et al.24
Both the increased incidence and mortality rates probably reflect an increase in the diagnosis of amyloidosis. Knowledge about the disease has increased in recent years, particularly in our centre, with the creation of the Amyloidosis Study Group and the beginning of patient recruitment in the IAR in 2010. In addition, extensive training of healthcare personnel has been carried out, as well as the dissemination of information through publications, congresses, symposia and courses. Also, new diagnostic methods have been introduced, such as measurement of free light chains, bone scintigraphy with pyrophosphate, echocardiography with strain, cardiac MRI with T1 mapping and non-invasive biopsies of lower salivary glands, which have improved the accuracy of amyloidosis diagnosis and subtype characterisation. During the study period, new specific treatments emerged that changed the prognosis of patients, which may be a source of motivation in the active search for the disease by the healthcare team.
Finally, it should be noted that although mortality increased throughout the study period, the increase was greater for incidence than for mortality in the last quadrennium. On one hand, the increase in mortality would be expected to follow the increase in incidence, and on the other hand, it could be because the timeliness of diagnosis and improvements in treatment favour prognosis and therefore survival.
This study has limitations that should be mentioned. First, while we acknowledge that incidence studies are typically based on population-based registries, our study relies on a cohort of patients affiliated with a health insurance programme with healthcare services limited to the Hospital Italiano network. This design allows us to access detailed follow-up data and ensures the validated detection of amyloidosis cases, which is crucial for maintaining data quality. This approach is similar to that used in other incidence studies focused on specific cohorts.20 The decision to conduct an incidence and mortality study in an affiliated population was driven by the lack of access to a geographically defined, at-risk population in a population-based registry. Despite this limitation, we believe that this study provides valuable insights, particularly given the scarcity of data on rare diseases like amyloidosis. Second, due to the characteristics of the study population, which consists of elderly patients affiliated to a MCP of a community hospital, the external validity of the results is limited. Therefore, the obtained results cannot be extrapolated to the general population of Argentina or Buenos Aires city. Finally, a key limitation of this study is the limited availability of advanced typing technologies in Latin America, which poses significant economic and logistical challenges. The high costs associated with sending samples abroad for analysis further constrain access. Despite these barriers, our centre is working to improve accessibility to these technologies while exploring alternative tools that may be more feasible in resource-limited settings.
Among the strengths, we consider that it is a study with high internal validity based on data obtained prospectively from the IAR. In this sense, the IAR case capture methodology allows for an exhaustive coverage of incident cases and allows for the validation of mortality data. On the other hand, the interdisciplinary conformation of the Amyloidosis Study Group favours the implementation of actions in clinical practice. The importance of these reported data is emphasised since they can be used as a reference for other research groups, as well as information for management. In this sense, it provides data related to the expected annual cases according to the population covered by the MCP for resource planning and service utilisation, among others.
Conclusion
This study provides detailed information on the incidence and mortality of amyloidosis in a specific population. The results indicate that amyloidosis remains a rare disease but with an increase in incidence in recent years. In addition, the observed increase in the mortality rate, especially in the elderly, emphasises the importance of early diagnosis and appropriate treatment. This study highlights the need for further research into the epidemiology and management of amyloidosis, as well as the need for a multidisciplinary approach to improve the quality of life of patients affected by this rare disease.
Footnotes
Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Data availability free text: The datasets generated and/or analysed during the current study are not publicly available due to our commitment to data confidentiality, but are available from the corresponding author on reasonable request.
Patient consent for publication: Not applicable.
Ethics approval: The conduction of this study was carried out in accordance with ethical principles, in line with the principles of the Declaration of Helsinki and national regulatory standards. Approval was granted by the Ethics Committee of the Hospital Italiano de Buenos Aires (22 September 2022; number of approval 6504). Given the deidentified nature of the data, the use of informed consent was not required.
Provenance and peer review: Not commissioned; externally peer reviewed.
Patient and public involvement: Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Data availability statement
Data are available upon reasonable request.
References
- 1.Benson MD, Buxbaum JN, Eisenberg DS, et al. Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee. Amyloid. 2020;27:217–22. doi: 10.1080/13506129.2020.1835263. [DOI] [PubMed] [Google Scholar]
- 2.Chiti F, Dobson CM. Protein Misfolding, Amyloid Formation, and Human Disease: A Summary of Progress Over the Last Decade. Annu Rev Biochem. 2017;86:27–68. doi: 10.1146/annurev-biochem-061516-045115. [DOI] [PubMed] [Google Scholar]
- 3.Pepys MB. Amyloidosis. Annu Rev Med. 2006;57:223–41. doi: 10.1146/annurev.med.57.121304.131243. [DOI] [PubMed] [Google Scholar]
- 4.Sikkink LA, Ramirez-Alvarado M. Cytotoxicity of amyloidogenic immunoglobulin light chains in cell culture. Cell Death Dis. 2010;1:e98. doi: 10.1038/cddis.2010.75. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.Aguirre MA, Boietti BR, Nucifora E, et al. Incidence rate of amyloidosis in patients from a medical care program in Buenos Aires, Argentina: a prospective cohort. Amyloid. 2016;23:184–7. doi: 10.1080/13506129.2016.1207626. [DOI] [PubMed] [Google Scholar]
- 6.Kyle RA, Linos A, Beard CM, et al. Amyloid and Amyloidosis 1990. 1991. Incidence and epidemiology of primary systemic amyloidosis (al) in olmsted county, minnesota: 1950 through 1989; pp. 211–4. [PubMed] [Google Scholar]
- 7.Pinney JH, Smith CJ, Taube JB, et al. Systemic amyloidosis in England: an epidemiological study. Br J Haematol. 2013;161:525–32. doi: 10.1111/bjh.12286. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Hemminki K, Li X, Försti A, et al. Incidence and survival in non-hereditary amyloidosis in Sweden. BMC Public Health. 2012;12:974. doi: 10.1186/1471-2458-12-974. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Rosenzweig M, Landau H. Light chain (AL) amyloidosis: update on diagnosis and management. J Hematol Oncol. 2011;4:47. doi: 10.1186/1756-8722-4-47. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 10.Dingli D, Tan TS, Kumar SK, et al. Stem cell transplantation in patients with autonomic neuropathy due to primary (AL) amyloidosis. Neurology (ECronicon) 2010;74:913–8. doi: 10.1212/WNL.0b013e3181d55f4d. [DOI] [PubMed] [Google Scholar]
- 11.Alexander KM, Orav J, Singh A, et al. Geographic Disparities in Reported US Amyloidosis Mortality From 1979 to 2015: Potential Underdetection of Cardiac Amyloidosis. JAMA Cardiol. 2018;3:865–70. doi: 10.1001/jamacardio.2018.2093. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 12.Gillmore JD, Maurer MS, Falk RH, et al. Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis. Circulation. 2016;133:2404–12. doi: 10.1161/CIRCULATIONAHA.116.021612. [DOI] [PubMed] [Google Scholar]
- 13.Palladini G, Russo P, Bosoni T, et al. Identification of amyloidogenic light chains requires the combination of serum-free light chain assay with immunofixation of serum and urine. Clin Chem. 2009;55:499–504. doi: 10.1373/clinchem.2008.117143. [DOI] [PubMed] [Google Scholar]
- 14.Quock TP, Yan T, Chang E, et al. Epidemiology of AL amyloidosis: a real-world study using US claims data. Blood Adv. 2018;2:1046–53. doi: 10.1182/bloodadvances.2018016402. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 15.Laires PA, Evans J, Thompson J, et al. Prevalence, Incidence, and Characterization of LIGHT Chain Amyloidosis in the USA: A Real-World Analysis Utilizing Electronic Health Records (EHR) Blood. 2023;142:6767. doi: 10.1182/blood-2023-181635. [DOI] [Google Scholar]
- 16.Cappelli F, Del Franco A, Vergaro G, et al. Prevalence of transthyretin-related amyloidosis in Tuscany: Data from the regional population-based registry. Int J Cardiol. 2023;382:87–90. doi: 10.1016/j.ijcard.2023.03.063. [DOI] [PubMed] [Google Scholar]
- 17.Mellqvist U-H, Cai Q, Hester LL, et al. Epidemiology and clinical outcomes of light-chain amyloidosis in Sweden: A nationwide population-based study. Eur J Haematol. 2023;111:697–705. doi: 10.1111/ejh.14063. [DOI] [PubMed] [Google Scholar]
- 18.Damy T, Bourel G, Slama M, et al. Incidence and survival of transthyretin amyloid cardiomyopathy from a French nationwide study of in- and out-patient databases. Orphanet J Rare Dis. 2023;18:345. doi: 10.1186/s13023-023-02933-w. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 19.Laires P, Li X, Uday A, et al. PREVALENCE AND INCIDENCE OF ATTR-CM AMYLOIDOSIS IN THE UNITED STATES: INSIGHTS FROM CLAIMS DATABASE AND ELECTRONIC HEALTH RECORDS. J Am Coll Cardiol. 2024;83:615. doi: 10.1016/S0735-1097(24)02605-6. [DOI] [Google Scholar]
- 20.Gilstrap LG, Dominici F, Wang Y, et al. Epidemiology of Cardiac Amyloidosis-Associated Heart Failure Hospitalizations Among Fee-for-Service Medicare Beneficiaries in the United States. Circ Heart Fail. 2019;12:e005407. doi: 10.1161/CIRCHEARTFAILURE.118.005407. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 21.Guha K, Baruah R, Mamza JB, et al. Patient characteristics and incidence of cardiac amyloidosis: an observational study in the UK clinical setting using electronic health records. Eur Heart J. 2024;45 doi: 10.1093/eurheartj/ehae666.1983. [DOI] [Google Scholar]
- 22.Choi Y-J, Choi YJ, Lee JE, et al. Temporal trends in the prevalence, incidence, and mortality of cardiac amyloidosis in South Korea over 12 years. Epidemiol Health. 2024:e2024078. doi: 10.4178/epih.e2024078. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 23.Ravichandran S, Lachmann HJ, Wechalekar AD. Epidemiologic and Survival Trends in Amyloidosis, 1987-2019. N Engl J Med. 2020;382:1567–8. doi: 10.1056/NEJMc1917321. [DOI] [PubMed] [Google Scholar]
- 24.Harada K, Hagiya H, Koyama T, et al. Trends in the amyloidosis mortality rate in Japan: A nationwide observational study from 1998 to 2019. Geriatr Gerontol Int. 2022;22:246–50. doi: 10.1111/ggi.14348. [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Data Availability Statement
Data are available upon reasonable request.