Materiały źródłowe

• #1 Pathophysiology, Classification, and Epidemiology of Amyloidosis | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-031-51757-0_4

  Once considered a rare disease, it is now well acknowledged that, depending on the amyloid precursors, the prevalence of amyloidosis may be frequent, at least in some specific age ranges or clinical settings. […] Aimo A, Merlo M, Porcari A, Georgiopoulos G, Pagura L, Vergaro G, et al. Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta-analysis of screening studies. Eur J Heart Fail. 2022;24(12):234251. […] Kumar N, Zhang NJ, Cherepanov D, Romanus D, Hughes M, Faller DV. Global epidemiology of amyloid light-chain amyloidosis. Orphanet J Rare Dis. 2022;17(1):278. […] Quock TP, Yan T, Chang E, Guthrie S, Broder MS. Epidemiology of AL amyloidosis: a real-world study using US claims data. Blood Adv. 2018;2(10):104653. […] Tanskanen M, Peuralinna T, Polvikoski T, Notkola IL, Sulkava R, Hardy J, et al. Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy study. Ann Med. 2008;40(3):2329.

• #1 Amyloidosis – Wikipedia

  https://en.wikipedia.org/wiki/Amyloidosis

  Amyloidosis has a combined estimated prevalence of 30 per 100,000 persons with the three most common forms being AL, ATTR, and AA. […] AL has the highest incidence at approximately 12 cases per million persons per year and an estimated prevalence of 30,000 to 45,000 cases in the US and European Union. […] AA amyloidoses is the most common form in developing countries and can complicate longstanding infections with tuberculosis, osteomyelitis, and bronchiectasis. […] Wild-type transthyretin (ATTR) amyloidosis is found in a quarter of elderly at postmortem. […] ATTR is found in 13-19% of people experiencing heart failure with preserved ejection fraction, making it a very common form of systemic amyloidosis.

• #1 Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication | Methodist DeBakey Cardiovascular J

  https://journal.houstonmethodist.org/articles/10.14797/mdcvj.1070

  Primary or light chain (AL) amyloidosis, the most common type of systemic amyloidosis, occurs when the free light chains normally associated with immunoglobulins are produced in excess by clonal or frankly malignant plasma cells. […] It is a relatively rare condition, with a worldwide incidence of 5.1 to 12.8 cases per million person-years. In the United States, between 1,275 and 3,200 new cases are diagnosed annually, yielding an incidence of approximately 9 to 14 cases per million person-years and a prevalence of 40.5 cases per million as of 2015—a significant increase from the prevalence of 15.5 cases per million reported in 2007. It is hypothesized that this rising prevalence is due to a growing awareness of the disease and its manifestations as well as improved treatments that have lowered the mortality rate.

• #1 Amyloidosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470285/

  AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries. In the United States, there are approximately 1275 to 3200 new cases per year. The annual proportion of new cases with AL is 78%. […] Familial transthyretin-associated amyloidosis (ATTR) is a less common systemic type of amyloidosis with unknown incidence, but approximately 10% to 20% of diagnosed cases in tertiary centers are secondary to ATTR amyloidosis. Of these cases, seven percent are hereditary and result from mutated transthyretin (ATTRm) and approximately six percent represent the acquired age-related, wild-type ATTRwt amyloidosis. ATTRwt is seen more commonly in males and was formerly known as senile systemic amyloidosis. Secondary or AA amyloidosis represents 6% of all the amyloidosis cases diagnosed each year. AA amyloidosis is an acquired process and reactive due to chronic inflammation.

• #1 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] The most comprehensive epidemiological study in recent decades was conducted by Kyle et al at the Mayo Clinic with data collected from 1950-1990 from the general population residing in Olmsted County, MN, USA. […] These authors reported an incidence of AL amyloidosis in nine cases per million person-years (95% confidence interval; 5.1-12.8 cases per million person-years).

• #1 Cardiac amyloidosis: epidemiology, diagnosis and therapy

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-19/cardiac-amyloidosis-epidemiology-diagnosis-and-therapy

  Cardiac amyloidosis is an infiltrative cardiomyopathy caused by accumulation in the heart interstitium of amyloid fibrils formed by misfolded proteins. Amyloid cardiomyopathy is an underestimated cause of heart failure and cardiac arrhythmias. Two types of amyloid commonly infiltrate the heart: immunoglobulin light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. […] Advances in cardiac imaging and improved awareness among physicians have facilitated the diagnosis of cardiac amyloidosis over the last decade. Involvement of the heart in the setting of amyloidosis carries an adverse prognosis. Timely diagnosis is of the greatest importance for appropriate treatment modalities that may crucially modify the natural history of the disease. […] AL amyloidosis results from the deposition of immunoglobin light chains produced by a plasma-cell dyscrasia. It is a rare condition with an incidence of approximately 2,500 to 5,000 new cases in the USA per year. A recent study reported a significant increase between 2007 and 2015 in the incidence and prevalence rates of AL amyloidosis among individuals aged 18-64 years; it is estimated that nearly 12,000 patients with AL amyloidosis live in the USA.

• #1 Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03547-0

  This SLR demonstrated heterogeneity in ATTR epidemiology and mortality rates across global regions. Further investigation is needed to address knowledge gaps of the epidemiology and burden of ATTR, which may improve early diagnosis and management. […] Recent data suggest that the prevalence of ATTR amyloidosis may be as high as 15% in patients with cardiovascular comorbidities. […] The prevalence of ATTR amyloidosis notably varies by genotype and phenotype. […] The prevalence of unspecified ATTR-CM in patients with HF was reported in 11 studies, and for most of the studies it ranged from 3.3 to 21%. […] The high prevalence of ATTR amyloidosis in patients with HF and AS suggests that routine screening in these groups may yield a significant number of undiagnosed cases, thereby facilitating earlier intervention and potentially altering the disease course.

• #1 Amyloidosis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Amyloidosis_epidemiology_and_demographics

  The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. […] The incidence of AL amyloidosis in USA ranged from 1 per 100,000 to 1.4 per 100,000 from 2007 to 2015. […] The prevalence of AL in USA amyloidosis increased significantly between 2007 and 2015, from 1.6 per 100,000 in 2007 to 4.0 per 100,000 in 2015. […] The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. […] In amyloidosis, the mean age of presentation is 55-60 years. […] Men are more commonly affected by amyloidosis than women. […] Transthyretin-related hereditary amyloidosis is endemic in Portuguese locations Pvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected people, coming from about 500 families, where 70% of the people develop the illness. In northern Sweden, more specifically Pite, Skellefte and Ume, 1.5% of the population has the mutated gene. There are many other populations in the world who exhibit the illness after having developed it independently. […] Hereditary amyloidosis subtypes include a substitution of an amino acid that is detected in approximately 4% of the black population.

• #1 Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication | Methodist DeBakey Cardiovascular J

  https://journal.houstonmethodist.org/articles/10.14797/mdcvj.1070

  The age-specific incidence rate of AL amyloidosis increases with each decade over age 40 years, with 64 years being the median age at diagnosis. In fact, less than 5% of affected patients are under the age of 40. […] AL amyloidosis has no apparent ethnic or geographic specificity. […] Staging of amyloidosis is best performed at diagnosis to help determine a patient’s prognosis and most suitable course of treatment, and then repeated 3 and 6 months after initiating treatment to determine efficacy and response to therapy.

• #1 Primary hepatic amyloidosis: a mini literature review and five cases report | Annals of Hepatology

  https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-primary-hepatic-amyloidosis-mini-literature-S1665268119314504

  A precise estimation for the incidence rate of systematic amyloidosis can be difficult as many cases are frequently undiagnosed or misdiagnosed. In western countries, the reported incidence rate of systemic amyloidosis was 0.09-0.8% in hospitalized patients and 0.4-0.5% in autopsy cases. In China, systemic amyloidosis has been found in only 0.014-0.03% of hospitalized patients. This relatively lower incidence in Chinese population may reflect the impact of multiple factors, such as genetic background, environment, life style, and insufficient awareness of the physicians. The incidence for primary amyloidosis is even lower, with an estimated (and perhaps an underestimated) age-adjusted incidence of 5.1-12.8 per million per year, and only 0.07% in all hospitalized patients. Primary amyloidosis is two times more common in males than in females and usually affects the patients in their 6th to 7th decades, with a median age of diagnosis being 63-64 years. Although younger patients do get affected. Approximately 9% of patients with amyloidosis have liver involvement, and more than 30% of patients with systemic amyloidosis have clinical evidence of hepatic involvement. Autopsy studies have reported an even higher incidence: 56-95% of patients with systemic amyloidosis exhibited hepatic involvement. Hepatomegaly has been reported in about 81-92% patients with hepatic amyloidosis, but this does not necessarily indicate the presence of PHA, especially when patients are complicated with congestive heart failure. In the past 10 years, we only encountered five patients with PHA confirmed by histopathology (4 males and 1 female), which approximately account for 15 patients per million in contemporaneous total hospitalized patients and 15.6% in the all amyloidosis patients of our hospital.

• #1 Epidemiological perspectives of amyloidosis in Argentina: a cohort study analysing incidence and mortality patterns among a population affiliated to a medical care programme | BMJ Public Health

  https://bmjpublichealth.bmj.com/content/3/1/e001047

  The limited knowledge of the current epidemiology of amyloidosis represents a significant obstacle to the effective management of this disease. […] 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. […] 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. […] The results indicate that amyloidosis remains a rare disease but with an increase in incidence in recent years.

• #1 Re-Definition of the Epidemiology of Cardiac Amyloidosis

  https://www.mdpi.com/2227-9059/10/7/1566

  The epidemiology of cardiac amyloidosis (CA), traditionally considered a rare and incurable disease, has changed drastically over the last ten years, particularly due to the advances in diagnostic methods and therapeutic options in the field of transthyretin CA (ATTR-CA). […] The most significant innovations concern ATTR-CA, whose diagnostic work-up has been deeply transformed by the possibility of reaching a non-biopsy diagnosis through cardiac scintigraphy with bone tracers. […] Consequently, the diagnoses of ATTR-CA have increased exponentially and patients have been diagnosed earlier in the disease course, also because of an increased awareness of CA among physicians of many specialties. […] A prospective multicentric study (AC-TIVE study) conducted in Italy investigated the prevalence of CA among patients with echocardiographic red flags and evaluated their diagnostic accuracy.

• #1 Re-Definition of the Epidemiology of Cardiac Amyloidosis

  https://www.mdpi.com/2227-9059/10/7/1566

  Based on these results, it appears important to change our approach to patients undergoing an echocardiogram, in order to systematically search for CA red flags, regardless of the exam indication; in this way, the echocardiography laboratory will become essential for obtaining an early diagnosis. […] In the last few years, the following subgroups of patients have been found to have a higher prevalence of CA: elderly subjects > 75 years, patients with LVH hospitalized for heart failure with preserved ejection fraction (HFpEF), subjects operated on for bilateral carpal tunnel syndrome (CTS), patients with cardiac hypertrophy not explained by concomitant factors and individuals with aortic valve stenosis (AS). […] The latest advances in the knowledge of the epidemiology of CA have contributed to increasing the awareness in the scientific community, changing the approach to the disease. […] Specific clinical settings have been recognized as strongly associated with CA but further studies are needed to better characterize these populations, mainly to stratify patient risk and evaluate which patients are ideal candidates for the newly available disease-modifying therapies.

• #1 Epidemiology of Amyloidosis and Genetic Pathways to Diagnosis and Typing

  https://www.mdpi.com/2673-6357/2/3/27

  Data on the incidence of amyloidosis is not abundant, which is typical of diseases for which no exact international classification of disease (ICD) codes have been available. In the case of amyloidosis, this is probably a reflection of the rarity, the diagnostic complexity, and heterogeneity of the condition. In a Swedish nationwide study from 2001 to 2008, we estimated the incidence of secondary systemic amyloidosis (ICD-10 code E85.3, most likely AA amyloidosis) at 1.15/million for combined sexes. The female rate was two times higher than the male rate, probably relating to the higher female prevalence of rheumatoid arthritis. The median survival time was 4 years. The incidence estimate from the UK was similar and the recent survival was estimated at 5.3 years. For the other types of amyloidosis, no specific ICD codes were available. However, the incidence for AL was extrapolated to be 3.2/million, with a median survival time of 3 years.

• #1

  https://link.springer.com/article/10.1007/s10741-021-10162-1

  Cardiac amyloidosis, once considered a rare disease, has garnered significant attention over the last few years due to three key reasons: first, increased recognition of this disease in conjunction with various common cardiac conditions such as heart failure with preserved ejection fraction and aortic stenosis; second, due to the advent of promising new therapies for light chain disease (AL), transthyretin (ATTR) cardiomyopathy, and amyloid neuropathy; finally, the advancements in cardiac imaging including echocardiography, magnetic resonance imaging, and nuclear cardiac scintigraphy aid in non-biopsy diagnosis of ATTR cardiac amyloidosis. […] The hereditary forms of ATTR have further come into importance with the availability of genetic testing and increased prevalence of certain mutations in African Americans.

• #2 Cardiac amyloidosis: a changing epidemiology with open challenges

  https://www.oaepublish.com/articles/2574-1209.2021.106

  Cardiac amyloidosis (CA) is increasingly diagnosed due to the advancements made in diagnostics and therapeutics in the last decades, particularly in the field of transthyretin-related CA. […] Studies that have used bone scintigraphy for screening at-risk conditions have shown that about one out of ten patients with heart failure with preserved ejection fraction (HFpEF), aortic stenosis undergoing valve replacement, or hypertrophic cardiomyopathy (HCM) diagnosed later in life might have an underlying or concomitant CA. […] The epidemiology of these conditions is also rapidly evolving. […] The epidemiological scenario of CA is rapidly evolving and this condition is increasingly diagnosed, particularly because of two recent discoveries: first, the possibility of performing a non-invasive diagnosis of ATTR-CA in a vast majority of cases and, second, the demonstrated efficacy of specific treatments aiming at interrupting the progression of myocardial TTR infiltration.

• #2 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] The most comprehensive epidemiological study in recent decades was conducted by Kyle et al at the Mayo Clinic with data collected from 1950-1990 from the general population residing in Olmsted County, MN, USA. […] These authors reported an incidence of AL amyloidosis in nine cases per million person-years (95% confidence interval; 5.1-12.8 cases per million person-years).

• #2 Amyloidosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470285/

  AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries. In the United States, there are approximately 1275 to 3200 new cases per year. The annual proportion of new cases with AL is 78%. […] Familial transthyretin-associated amyloidosis (ATTR) is a less common systemic type of amyloidosis with unknown incidence, but approximately 10% to 20% of diagnosed cases in tertiary centers are secondary to ATTR amyloidosis. Of these cases, seven percent are hereditary and result from mutated transthyretin (ATTRm) and approximately six percent represent the acquired age-related, wild-type ATTRwt amyloidosis. ATTRwt is seen more commonly in males and was formerly known as senile systemic amyloidosis. Secondary or AA amyloidosis represents 6% of all the amyloidosis cases diagnosed each year. AA amyloidosis is an acquired process and reactive due to chronic inflammation.

• #2 Understanding Amyloidosis: Etiology, Epidemiology, Pathophysiology, Management | oneAMYLOIDOSISvoice

  https://oneamyloidosisvoice.com/rcuratenew/understanding-amyloidosis-etiology-epidemiology-pathophysiology-management

  AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries. […] In the United States, there are approximately 1275 to 3200 new cases per year. […] The annual proportion of new cases with AL is 78%. […] Familial transthyretin-associated amyloidosis (ATTR) is a less common systemic type of amyloidosis with unknown incidence, but approximately 10% to 20% of diagnosed cases in tertiary centers are secondary to ATTR amyloidosis.

• #2 Orphanet: Amyloidosis

  https://www.orpha.net/en/disease/detail/69

  AL amyloidosis’s estimated global incidence of 1/95,800 people, with males preponderance. […] Hereditary ATTR amyloidosis (or ATTRv) mid-global prevalence estimate is of 1/450,000 people (range 1/120,000-830,000). […] In Tuscany, the incidence of ATTR wild type (ATTRwt) and ATTRv is 1/37,500 and 1/370,000, respectively, with male preponderance. […] AA amyloidosis estimated incidence is 1-2/1,000,000, but is decreasing.

• #2 Systemic AA amyloidosis: epidemiology, diagnosis, and management – Document – Gale Academic OneFile

  https://go.gale.com/ps/i.do?id=GALE%7CA400253707&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=11791349&p=AONE&sw=w

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] In this sense, the 123I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in systemic amyloidosis. […] Given the etiologic diversity of AA amyloidosis, common therapeutic strategies are scarce.

• #2 Cardiac amyloidosis: epidemiology, diagnosis and therapy

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-19/cardiac-amyloidosis-epidemiology-diagnosis-and-therapy

  Cardiac amyloidosis is an infiltrative cardiomyopathy caused by accumulation in the heart interstitium of amyloid fibrils formed by misfolded proteins. Amyloid cardiomyopathy is an underestimated cause of heart failure and cardiac arrhythmias. Two types of amyloid commonly infiltrate the heart: immunoglobulin light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. […] Advances in cardiac imaging and improved awareness among physicians have facilitated the diagnosis of cardiac amyloidosis over the last decade. Involvement of the heart in the setting of amyloidosis carries an adverse prognosis. Timely diagnosis is of the greatest importance for appropriate treatment modalities that may crucially modify the natural history of the disease. […] AL amyloidosis results from the deposition of immunoglobin light chains produced by a plasma-cell dyscrasia. It is a rare condition with an incidence of approximately 2,500 to 5,000 new cases in the USA per year. A recent study reported a significant increase between 2007 and 2015 in the incidence and prevalence rates of AL amyloidosis among individuals aged 18-64 years; it is estimated that nearly 12,000 patients with AL amyloidosis live in the USA.

• #2 Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03547-0

  This SLR demonstrated heterogeneity in ATTR epidemiology and mortality rates across global regions. Further investigation is needed to address knowledge gaps of the epidemiology and burden of ATTR, which may improve early diagnosis and management. […] Recent data suggest that the prevalence of ATTR amyloidosis may be as high as 15% in patients with cardiovascular comorbidities. […] The prevalence of ATTR amyloidosis notably varies by genotype and phenotype. […] The prevalence of unspecified ATTR-CM in patients with HF was reported in 11 studies, and for most of the studies it ranged from 3.3 to 21%. […] The high prevalence of ATTR amyloidosis in patients with HF and AS suggests that routine screening in these groups may yield a significant number of undiagnosed cases, thereby facilitating earlier intervention and potentially altering the disease course.

• #2 Amyloidosis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Amyloidosis_epidemiology_and_demographics

  The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. […] The incidence of AL amyloidosis in USA ranged from 1 per 100,000 to 1.4 per 100,000 from 2007 to 2015. […] The prevalence of AL in USA amyloidosis increased significantly between 2007 and 2015, from 1.6 per 100,000 in 2007 to 4.0 per 100,000 in 2015. […] The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. […] In amyloidosis, the mean age of presentation is 55-60 years. […] Men are more commonly affected by amyloidosis than women. […] Transthyretin-related hereditary amyloidosis is endemic in Portuguese locations Pvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected people, coming from about 500 families, where 70% of the people develop the illness. In northern Sweden, more specifically Pite, Skellefte and Ume, 1.5% of the population has the mutated gene. There are many other populations in the world who exhibit the illness after having developed it independently. […] Hereditary amyloidosis subtypes include a substitution of an amino acid that is detected in approximately 4% of the black population.

• #2 Global epidemiology of amyloid light-chain amyloidosis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02414-6

  To understand the true burden of AL amyloidosis, increase awareness, inform treatment options, and allow effective healthcare provision, it is essential to determine how many cases of AL amyloidosis exist globally. Few studies have examined the epidemiology of AL amyloidosis, and country-specific registries for AL amyloidosis are limited. The objective of this study was to examine the global epidemiology of AL amyloidosis, reporting annual incidence and period prevalence. […] This analysis indicated that in 2018, the crude incidence of AL amyloidosis in countries in and near Europe, as well as in Brazil, Canada, Japan, Russia, South Korea, Taiwan, and the US was 10.44 PMP, with a prevalence of 51.27 PMP over the preceding 20 years. […] The present study showed small geographic variation in the estimates of AL amyloidosis incidence, which ranged between 8.94 to 13.29 PMP in countries in and near Europe and 6.72 to 14.3 PMP in Brazil, Canada, Japan, Russia, South Korea, Taiwan, and the US.

• #2 AA (Inflammatory) Amyloidosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/335559-overview

  The reported prevalence of amyloidosis in RA ranges from 7%-26%. […] The frequency of renal amyloidosis in some populations with untreated FMF is almost 100%. […] Most available data to approximate the epidemiology of AA amyloidosis are derived from autopsies. The overall autopsy incidence of AA amyloidosis in Western nations ranges from 0.50-0.86%. […] In Japanese people, in whom the SAA 1.5 allele is far more common than in whites (37.4% vs 5.3%), the 1.5 allele is enriched among patients with RA and amyloidosis. […] In the United States, AA amyloidosis is more common in females, reflecting the fact that the major predisposing disease, RA, is predominantly a disorder of younger women and middle-aged men; hence, women are apt to have the disease for a longer period than men. […] Despite the statistical female predominance in terms of overall numbers of AA amyloidosis cases, males seem to have an earlier average age of onset. […] Age at onset of amyloidosis is related to the age at onset of the inflammatory disease, its severity, and the duration of the disease within the constraints imposed by the alleles of SAA carried by the patient.

• #2 Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03547-0

  Our review highlights the limitation in research that offers comprehensive global ATTR-specific epidemiology and mortality data thus hindering our understanding of its true global burden. […] Many studies included in this review originated in Europe and North America, and there was a notable absence of data from regions such as Africa and South America, suggesting geographical disparity in ATTR research. […] The true global prevalence of ATTR-PN remains unclear, though it is estimated to be between 10,000 and 40,000 persons globally, with endemic foci in Portugal, Sweden, and Japan. […] Robust epidemiological ATTR amyloidosis data requires further investigation in a larger sample of patients with diverse phenotypes across multiple countries and world regions.

• #2 Re-Definition of the Epidemiology of Cardiac Amyloidosis

  https://www.mdpi.com/2227-9059/10/7/1566

  The epidemiology of cardiac amyloidosis (CA), traditionally considered a rare and incurable disease, has changed drastically over the last ten years, particularly due to the advances in diagnostic methods and therapeutic options in the field of transthyretin CA (ATTR-CA). […] The most significant innovations concern ATTR-CA, whose diagnostic work-up has been deeply transformed by the possibility of reaching a non-biopsy diagnosis through cardiac scintigraphy with bone tracers. […] Consequently, the diagnoses of ATTR-CA have increased exponentially and patients have been diagnosed earlier in the disease course, also because of an increased awareness of CA among physicians of many specialties. […] A prospective multicentric study (AC-TIVE study) conducted in Italy investigated the prevalence of CA among patients with echocardiographic red flags and evaluated their diagnostic accuracy.

• #2 Re-Definition of the Epidemiology of Cardiac Amyloidosis

  https://www.mdpi.com/2227-9059/10/7/1566

  Based on these results, it appears important to change our approach to patients undergoing an echocardiogram, in order to systematically search for CA red flags, regardless of the exam indication; in this way, the echocardiography laboratory will become essential for obtaining an early diagnosis. […] In the last few years, the following subgroups of patients have been found to have a higher prevalence of CA: elderly subjects > 75 years, patients with LVH hospitalized for heart failure with preserved ejection fraction (HFpEF), subjects operated on for bilateral carpal tunnel syndrome (CTS), patients with cardiac hypertrophy not explained by concomitant factors and individuals with aortic valve stenosis (AS). […] The latest advances in the knowledge of the epidemiology of CA have contributed to increasing the awareness in the scientific community, changing the approach to the disease. […] Specific clinical settings have been recognized as strongly associated with CA but further studies are needed to better characterize these populations, mainly to stratify patient risk and evaluate which patients are ideal candidates for the newly available disease-modifying therapies.

• #2

  http://uu.diva-portal.org/smash/record.jsf?pid=diva2:1831921

  AL (immunoglobulin light chain) amyloidosis is often associated with delayed diagnosis and thereby high early mortality that is not overcome by contemporary treatment. […] However, population-based reports are scarce regarding epidemiology as well as treatment outcomes. […] Paper IV was a population-based epidemiological study in which we could determine the standardized incidence of systemic AL amyloidosis to 12.0 (95% CI 9.3-14.7) per million person-years for Uppsala County, without significant change during the period 2000-2020. […] Prolonged overall survival was observed over time, and there was also a decrease in early mortality, indicating earlier diagnosis of especially patients with cardiac involvement. […] Population-based incidence, prevalence and survival of systemic AL amyloidosis in Sweden.

• #3 Systemic AA amyloidosis: epidemiology, diagnosis, and management – Document – Gale Academic OneFile

  https://go.gale.com/ps/i.do?id=GALE%7CA400253707&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=11791349&p=AONE&sw=w

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] In this sense, the 123I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in systemic amyloidosis. […] Given the etiologic diversity of AA amyloidosis, common therapeutic strategies are scarce.

• #3 Understanding Amyloidosis: Etiology, Epidemiology, Pathophysiology, Management | oneAMYLOIDOSISvoice

  https://oneamyloidosisvoice.com/rcuratenew/understanding-amyloidosis-etiology-epidemiology-pathophysiology-management

  AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries. […] In the United States, there are approximately 1275 to 3200 new cases per year. […] The annual proportion of new cases with AL is 78%. […] Familial transthyretin-associated amyloidosis (ATTR) is a less common systemic type of amyloidosis with unknown incidence, but approximately 10% to 20% of diagnosed cases in tertiary centers are secondary to ATTR amyloidosis.

• #3 Amyloidosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470285/

  AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries. In the United States, there are approximately 1275 to 3200 new cases per year. The annual proportion of new cases with AL is 78%. […] Familial transthyretin-associated amyloidosis (ATTR) is a less common systemic type of amyloidosis with unknown incidence, but approximately 10% to 20% of diagnosed cases in tertiary centers are secondary to ATTR amyloidosis. Of these cases, seven percent are hereditary and result from mutated transthyretin (ATTRm) and approximately six percent represent the acquired age-related, wild-type ATTRwt amyloidosis. ATTRwt is seen more commonly in males and was formerly known as senile systemic amyloidosis. Secondary or AA amyloidosis represents 6% of all the amyloidosis cases diagnosed each year. AA amyloidosis is an acquired process and reactive due to chronic inflammation.

• #3 Epidemiology of Amyloidosis and Genetic Pathways to Diagnosis and Typing

  https://www.mdpi.com/2673-6357/2/3/27

  Data on the incidence of amyloidosis is not abundant, which is typical of diseases for which no exact international classification of disease (ICD) codes have been available. In the case of amyloidosis, this is probably a reflection of the rarity, the diagnostic complexity, and heterogeneity of the condition. In a Swedish nationwide study from 2001 to 2008, we estimated the incidence of secondary systemic amyloidosis (ICD-10 code E85.3, most likely AA amyloidosis) at 1.15/million for combined sexes. The female rate was two times higher than the male rate, probably relating to the higher female prevalence of rheumatoid arthritis. The median survival time was 4 years. The incidence estimate from the UK was similar and the recent survival was estimated at 5.3 years. For the other types of amyloidosis, no specific ICD codes were available. However, the incidence for AL was extrapolated to be 3.2/million, with a median survival time of 3 years.

• #3 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  Recently, similar studies have been carried out in two European regions. […] Pinney et al from the National Health Service National Amyloidosis Centre in the UK, estimated a global incidence of amyloidosis in England of five cases per million person-years. […] Of these, close to three cases per million person-years would have the AL type, and one case, the AA type. […] The percent increase in the frequency of AL amyloidosis with respect to the AA type can be explained by earlier diagnosis of many predisposing conditions for AA amyloidosis and widespread access to more effective treatments that better control many disease processes. […] Better access to antimicrobial agents and a stricter control of tuberculosis have led to a significant decrease in the frequency of chronic infectious diseases in developed countries, where autoimmune diseases such as RA, ankylosing spondylitis, chronic juvenile arthritis, and inflammatory bowel disease, or hereditary inflammatory diseases, such as FMF, TNF receptor-associated periodic syndrome, or Muckle-Wells syndrome, can account for up to 90% of recent cases of amyloidosis.

• #3 Cardiac amyloidosis: Epidemiology, clinical manifestations, and diagnosis – UpToDate

  https://www.uptodate.com/contents/cardiac-amyloidosis-epidemiology-clinical-manifestations-and-diagnosis

  Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. It can present with cardiac signs or symptoms or may be diagnosed as the result of screening in patients who manifest extracardiac signs of amyloidosis. […] This topic will review the epidemiology, clinical manifestations, and diagnosis of cardiac amyloidosis. […] The clinical syndrome associated with cardiac amyloid infiltration in the heart is referred to as „cardiac amyloidosis.” […] Two of the most common types of cardiac amyloidosis are transthyretin amyloidosis (ATTR amyloidosis) and light chain amyloidosis (AL amyloidosis), which are named after the precursor protein of the amyloid deposit.

• #3 Amyloidosis – Wikipedia

  https://en.wikipedia.org/wiki/Amyloidosis

  Amyloidosis has a combined estimated prevalence of 30 per 100,000 persons with the three most common forms being AL, ATTR, and AA. […] AL has the highest incidence at approximately 12 cases per million persons per year and an estimated prevalence of 30,000 to 45,000 cases in the US and European Union. […] AA amyloidoses is the most common form in developing countries and can complicate longstanding infections with tuberculosis, osteomyelitis, and bronchiectasis. […] Wild-type transthyretin (ATTR) amyloidosis is found in a quarter of elderly at postmortem. […] ATTR is found in 13-19% of people experiencing heart failure with preserved ejection fraction, making it a very common form of systemic amyloidosis.

• #3 Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication | Methodist DeBakey Cardiovascular J

  https://journal.houstonmethodist.org/articles/10.14797/mdcvj.1070

  The age-specific incidence rate of AL amyloidosis increases with each decade over age 40 years, with 64 years being the median age at diagnosis. In fact, less than 5% of affected patients are under the age of 40. […] AL amyloidosis has no apparent ethnic or geographic specificity. […] Staging of amyloidosis is best performed at diagnosis to help determine a patient’s prognosis and most suitable course of treatment, and then repeated 3 and 6 months after initiating treatment to determine efficacy and response to therapy.

• #3 Amyloidosis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Amyloidosis_epidemiology_and_demographics

  The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. […] The incidence of AL amyloidosis in USA ranged from 1 per 100,000 to 1.4 per 100,000 from 2007 to 2015. […] The prevalence of AL in USA amyloidosis increased significantly between 2007 and 2015, from 1.6 per 100,000 in 2007 to 4.0 per 100,000 in 2015. […] The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. […] In amyloidosis, the mean age of presentation is 55-60 years. […] Men are more commonly affected by amyloidosis than women. […] Transthyretin-related hereditary amyloidosis is endemic in Portuguese locations Pvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected people, coming from about 500 families, where 70% of the people develop the illness. In northern Sweden, more specifically Pite, Skellefte and Ume, 1.5% of the population has the mutated gene. There are many other populations in the world who exhibit the illness after having developed it independently. […] Hereditary amyloidosis subtypes include a substitution of an amino acid that is detected in approximately 4% of the black population.

• #3 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  Nevertheless, tuberculosis and other chronic infections are still relevant causes of AA amyloidosis in other European countries, as well as in developing countries. […] It is difficult to establish the approximate prevalence of AA amyloidosis among patients with inflammatory diseases, since these estimations vary substantially with the diagnostic method used (autopsy, affected organ biopsy, or indirect biopsy, such as abdominal fat, rectal mucosa, or minor salivary glands), clinical status (preclinical or symptomatic amyloidosis), or study type (case series or population-based). […] The prevalence of AA amyloidosis in patients with RA has been estimated to be 5%-78%; whereas, it is 10%-13% in patients with FMF. […] However, most of these studies were conducted before the generalized use of biologic therapies and current, more intensive, treatment protocols, that enable prompt control of the inflammatory process.

• #3 Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03547-0

  Our review highlights the limitation in research that offers comprehensive global ATTR-specific epidemiology and mortality data thus hindering our understanding of its true global burden. […] Many studies included in this review originated in Europe and North America, and there was a notable absence of data from regions such as Africa and South America, suggesting geographical disparity in ATTR research. […] The true global prevalence of ATTR-PN remains unclear, though it is estimated to be between 10,000 and 40,000 persons globally, with endemic foci in Portugal, Sweden, and Japan. […] Robust epidemiological ATTR amyloidosis data requires further investigation in a larger sample of patients with diverse phenotypes across multiple countries and world regions.

• #3 Re-Definition of the Epidemiology of Cardiac Amyloidosis

  https://www.mdpi.com/2227-9059/10/7/1566

  The epidemiology of cardiac amyloidosis (CA), traditionally considered a rare and incurable disease, has changed drastically over the last ten years, particularly due to the advances in diagnostic methods and therapeutic options in the field of transthyretin CA (ATTR-CA). […] The most significant innovations concern ATTR-CA, whose diagnostic work-up has been deeply transformed by the possibility of reaching a non-biopsy diagnosis through cardiac scintigraphy with bone tracers. […] Consequently, the diagnoses of ATTR-CA have increased exponentially and patients have been diagnosed earlier in the disease course, also because of an increased awareness of CA among physicians of many specialties. […] A prospective multicentric study (AC-TIVE study) conducted in Italy investigated the prevalence of CA among patients with echocardiographic red flags and evaluated their diagnostic accuracy.

• #3 Global epidemiology of amyloid light-chain amyloidosis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02414-6

  In the present study, the prevalence of AL amyloidosis increased over the 20-year period of analysis from 8.17 PMP at 1 year following diagnosis to 51.27 PMP at year 20. Annual prevalence estimates reported previously were also found to increase over time. […] This apparent rise in the prevalence of AL amyloidosis may reflect an actual increase in disease incidence, improved survival resulting from recent advances in therapeutic options, increased disease awareness, and more appropriate diagnosis. […] This study provides up-to-date global estimates of the incidence and 1-year, 5-year, 10-year, and 20-year period prevalence of AL amyloidosis. The epidemiological estimates for AL amyloidosis in this study highlight the rarity of condition across the globe and aid in understanding the true burden of the disease, which is vital for increasing awareness and furthering research and innovation of treatment options.

• #3

  https://smw.ch/index.php/smw/article/view/3485

  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.

• #3 Expert Analysis and Opinion—Understanding Cardiac Amyloidosis

  https://www.acc.org/Latest-in-Cardiology/Articles/2021/04/14/17/11/Understanding-Cardiac-Amyloidosis

  Cardiac amyloidosis (CA) is a protein-folding disorder nearly exclusively caused by misfolded amyloid transthyretin (ATTR) and amyloid light chain (AL) proteins. […] AL amyloidosis is a rare disease, ATTR may not be. […] AL incidence is 1:50,000-100,000, with 5,000-7,000 new cases identified annually in the United States. The prevalence is increasing, as survival has improved dramatically. […] ATTR demonstrates an age-dependent clinical penetrance. […] The clinical presentation and course of AL and ATTR are variable, but early diagnosis remains essential with specific populations that merit consideration of screening. […] Populations that merit enhanced consideration of ATTR amyloidosis include: severe aortic stenosis, particularly the low-flow, low-gradient phenotype; older patients with HFpEF, particularly those with bilateral carpal tunnel syndrome, spinal stenosis, or tendon rupture; autonomic or peripheral neuropathy and HF; presumed new diagnosis of hypertrophic cardiomyopathy (HCM) in an older patient.

• #4 Global epidemiology of amyloid light-chain amyloidosis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02414-6

  Amyloid light-chain (AL) amyloidosis is an ultra-rare disease associated with significant morbidity and mortality. Few studies have examined the global epidemiology of this condition. […] This study estimated the diagnosed incidence and 1-year, 5-year, 10-year, and 20-year period prevalence of AL amyloidosis in 2018 for countries in and near Europe, and in the United States (US), Canada, Brazil, Japan, South Korea, Taiwan, and Russia. […] In 2018, an estimated 74,000 AL amyloidosis cases worldwide were diagnosed during the preceding 20 years. The estimated incidence and 20-year prevalence rates were 10 and 51 cases per million population, respectively. […] This study provides up-to-date epidemiological patterns of AL amyloidosis, which is vital for understanding the burden of the disease, increasing awareness, and to further research and treatment options.

• #4 Amyloidosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470285/

  AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries. In the United States, there are approximately 1275 to 3200 new cases per year. The annual proportion of new cases with AL is 78%. […] Familial transthyretin-associated amyloidosis (ATTR) is a less common systemic type of amyloidosis with unknown incidence, but approximately 10% to 20% of diagnosed cases in tertiary centers are secondary to ATTR amyloidosis. Of these cases, seven percent are hereditary and result from mutated transthyretin (ATTRm) and approximately six percent represent the acquired age-related, wild-type ATTRwt amyloidosis. ATTRwt is seen more commonly in males and was formerly known as senile systemic amyloidosis. Secondary or AA amyloidosis represents 6% of all the amyloidosis cases diagnosed each year. AA amyloidosis is an acquired process and reactive due to chronic inflammation.

• #4 Orphanet: Amyloidosis

  https://www.orpha.net/en/disease/detail/69

  AL amyloidosis’s estimated global incidence of 1/95,800 people, with males preponderance. […] Hereditary ATTR amyloidosis (or ATTRv) mid-global prevalence estimate is of 1/450,000 people (range 1/120,000-830,000). […] In Tuscany, the incidence of ATTR wild type (ATTRwt) and ATTRv is 1/37,500 and 1/370,000, respectively, with male preponderance. […] AA amyloidosis estimated incidence is 1-2/1,000,000, but is decreasing.

• #4 AA (Inflammatory) Amyloidosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/335559-overview

  AA amyloidosis occurs in the course of chronic inflammatory diseases (infectious and noninfectious), hereditary periodic fevers, and with certain neoplasms, such as Hodgkin lymphoma and renal cell carcinoma. […] The absolute prevalence of AA amyloidosis is difficult to ascertain because it depends on both the occurrence of predisposing inflammatory disorders and the proportion of individuals with those conditions who develop tissue amyloid deposition. […] AA amyloidosis is far less common in the United States than in other countries, even in the setting of the same inflammatory disease. […] Currently, rheumatic diseases such as rheumatoid arthritis (RA), ankylosing spondylitis (AS), psoriatic arthritis, and juvenile idiopathic arthritis are the most frequent causes (70%) of AA amyloidosis.

• #4 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  Nevertheless, tuberculosis and other chronic infections are still relevant causes of AA amyloidosis in other European countries, as well as in developing countries. […] It is difficult to establish the approximate prevalence of AA amyloidosis among patients with inflammatory diseases, since these estimations vary substantially with the diagnostic method used (autopsy, affected organ biopsy, or indirect biopsy, such as abdominal fat, rectal mucosa, or minor salivary glands), clinical status (preclinical or symptomatic amyloidosis), or study type (case series or population-based). […] The prevalence of AA amyloidosis in patients with RA has been estimated to be 5%-78%; whereas, it is 10%-13% in patients with FMF. […] However, most of these studies were conducted before the generalized use of biologic therapies and current, more intensive, treatment protocols, that enable prompt control of the inflammatory process.

• #4 Amyloidosis – Wikipedia

  https://en.wikipedia.org/wiki/Amyloidosis

  Amyloidosis has a combined estimated prevalence of 30 per 100,000 persons with the three most common forms being AL, ATTR, and AA. […] AL has the highest incidence at approximately 12 cases per million persons per year and an estimated prevalence of 30,000 to 45,000 cases in the US and European Union. […] AA amyloidoses is the most common form in developing countries and can complicate longstanding infections with tuberculosis, osteomyelitis, and bronchiectasis. […] Wild-type transthyretin (ATTR) amyloidosis is found in a quarter of elderly at postmortem. […] ATTR is found in 13-19% of people experiencing heart failure with preserved ejection fraction, making it a very common form of systemic amyloidosis.

• #4 Amyloidosis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Amyloidosis_epidemiology_and_demographics

  The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. […] The incidence of AL amyloidosis in USA ranged from 1 per 100,000 to 1.4 per 100,000 from 2007 to 2015. […] The prevalence of AL in USA amyloidosis increased significantly between 2007 and 2015, from 1.6 per 100,000 in 2007 to 4.0 per 100,000 in 2015. […] The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. […] In amyloidosis, the mean age of presentation is 55-60 years. […] Men are more commonly affected by amyloidosis than women. […] Transthyretin-related hereditary amyloidosis is endemic in Portuguese locations Pvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected people, coming from about 500 families, where 70% of the people develop the illness. In northern Sweden, more specifically Pite, Skellefte and Ume, 1.5% of the population has the mutated gene. There are many other populations in the world who exhibit the illness after having developed it independently. […] Hereditary amyloidosis subtypes include a substitution of an amino acid that is detected in approximately 4% of the black population.

• #4 Epidemiology | oneAMYLOIDOSISvoice

  https://www.oneamyloidosisvoice.com/epidemiology

  Amyloidosis is a heterogeneous disease that results from the deposition of toxic insoluble beta-sheet fibrillar protein aggregates in different […] Amyloidosis is a rare complication of inflammatory bowel disease [IBD]; its low prevalence has hindered both descriptive and therapeutic […] Because amyloidosis is rare, the diagnosis is often delayed or the condition is not diagnosed. Therefore, it is difficult to know exactly how many people are

• #4 Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03547-0

  Our review highlights the limitation in research that offers comprehensive global ATTR-specific epidemiology and mortality data thus hindering our understanding of its true global burden. […] Many studies included in this review originated in Europe and North America, and there was a notable absence of data from regions such as Africa and South America, suggesting geographical disparity in ATTR research. […] The true global prevalence of ATTR-PN remains unclear, though it is estimated to be between 10,000 and 40,000 persons globally, with endemic foci in Portugal, Sweden, and Japan. […] Robust epidemiological ATTR amyloidosis data requires further investigation in a larger sample of patients with diverse phenotypes across multiple countries and world regions.

• #4 Cardiac amyloidosis: epidemiology, diagnosis and therapy

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-19/cardiac-amyloidosis-epidemiology-diagnosis-and-therapy

  Cardiac amyloidosis is an infiltrative cardiomyopathy caused by accumulation in the heart interstitium of amyloid fibrils formed by misfolded proteins. Amyloid cardiomyopathy is an underestimated cause of heart failure and cardiac arrhythmias. Two types of amyloid commonly infiltrate the heart: immunoglobulin light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. […] Advances in cardiac imaging and improved awareness among physicians have facilitated the diagnosis of cardiac amyloidosis over the last decade. Involvement of the heart in the setting of amyloidosis carries an adverse prognosis. Timely diagnosis is of the greatest importance for appropriate treatment modalities that may crucially modify the natural history of the disease. […] AL amyloidosis results from the deposition of immunoglobin light chains produced by a plasma-cell dyscrasia. It is a rare condition with an incidence of approximately 2,500 to 5,000 new cases in the USA per year. A recent study reported a significant increase between 2007 and 2015 in the incidence and prevalence rates of AL amyloidosis among individuals aged 18-64 years; it is estimated that nearly 12,000 patients with AL amyloidosis live in the USA.

• #4 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  Recently, similar studies have been carried out in two European regions. […] Pinney et al from the National Health Service National Amyloidosis Centre in the UK, estimated a global incidence of amyloidosis in England of five cases per million person-years. […] Of these, close to three cases per million person-years would have the AL type, and one case, the AA type. […] The percent increase in the frequency of AL amyloidosis with respect to the AA type can be explained by earlier diagnosis of many predisposing conditions for AA amyloidosis and widespread access to more effective treatments that better control many disease processes. […] Better access to antimicrobial agents and a stricter control of tuberculosis have led to a significant decrease in the frequency of chronic infectious diseases in developed countries, where autoimmune diseases such as RA, ankylosing spondylitis, chronic juvenile arthritis, and inflammatory bowel disease, or hereditary inflammatory diseases, such as FMF, TNF receptor-associated periodic syndrome, or Muckle-Wells syndrome, can account for up to 90% of recent cases of amyloidosis.

• #4 Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03547-0

  Significant advances in the treatment of transthyretin (ATTR) amyloidosis has led to an evolving understanding of the epidemiology of this condition. This systematic literature review (SLR) aims to synthesize current evidence on epidemiology and mortality outcomes in ATTR amyloidosis, addressing the need for a comprehensive understanding of its current global impact. […] Of the 1,458 studies identified, 113 met the inclusion criteria. Forty-nine studies reported on epidemiology, while 64 focused on mortality rates in cohorts of patients with ATTR amyloidosis from Europe (n=16), North America (n=26), Asia (n=5), and Australia (n=2). No studies were found that exclusively focused on ATTR amyloidosis in Africa or South America. ATTR prevalence ranged from 6.1/million in the US to 232/million in Portugal with very limited data on ATTR-PN. The 2-year mortality risk ranged from 10 to 30% among wild-type ATTR-CM and from 10 to 50% for variant type of ATTR-CM.

• #4 Global epidemiology of amyloid light-chain amyloidosis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-022-02414-6

  In the present study, the prevalence of AL amyloidosis increased over the 20-year period of analysis from 8.17 PMP at 1 year following diagnosis to 51.27 PMP at year 20. Annual prevalence estimates reported previously were also found to increase over time. […] This apparent rise in the prevalence of AL amyloidosis may reflect an actual increase in disease incidence, improved survival resulting from recent advances in therapeutic options, increased disease awareness, and more appropriate diagnosis. […] This study provides up-to-date global estimates of the incidence and 1-year, 5-year, 10-year, and 20-year period prevalence of AL amyloidosis. The epidemiological estimates for AL amyloidosis in this study highlight the rarity of condition across the globe and aid in understanding the true burden of the disease, which is vital for increasing awareness and furthering research and innovation of treatment options.

• #5 Amyloidosis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Amyloidosis_epidemiology_and_demographics

  The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. […] The incidence of AL amyloidosis in USA ranged from 1 per 100,000 to 1.4 per 100,000 from 2007 to 2015. […] The prevalence of AL in USA amyloidosis increased significantly between 2007 and 2015, from 1.6 per 100,000 in 2007 to 4.0 per 100,000 in 2015. […] The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. […] In amyloidosis, the mean age of presentation is 55-60 years. […] Men are more commonly affected by amyloidosis than women. […] Transthyretin-related hereditary amyloidosis is endemic in Portuguese locations Pvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected people, coming from about 500 families, where 70% of the people develop the illness. In northern Sweden, more specifically Pite, Skellefte and Ume, 1.5% of the population has the mutated gene. There are many other populations in the world who exhibit the illness after having developed it independently. […] Hereditary amyloidosis subtypes include a substitution of an amino acid that is detected in approximately 4% of the black population.

• #5 Understanding Amyloidosis: Etiology, Epidemiology, Pathophysiology, Management | oneAMYLOIDOSISvoice

  https://oneamyloidosisvoice.com/rcuratenew/understanding-amyloidosis-etiology-epidemiology-pathophysiology-management

  AL amyloidosis has an incidence of 1 case per 100,000 person-years in Western countries. […] In the United States, there are approximately 1275 to 3200 new cases per year. […] The annual proportion of new cases with AL is 78%. […] Familial transthyretin-associated amyloidosis (ATTR) is a less common systemic type of amyloidosis with unknown incidence, but approximately 10% to 20% of diagnosed cases in tertiary centers are secondary to ATTR amyloidosis.

• #5 Cardiac amyloidosis: epidemiology, diagnosis and therapy

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-19/cardiac-amyloidosis-epidemiology-diagnosis-and-therapy

  Published data on the epidemiology of cardiac amyloidosis are based mainly on single-centre studies or population registries. A recent study among Medicare beneficiaries aged 65 years in the USA showed an increase in both the incidence and prevalence rates of cardiac amyloidosis from 2000 to 2012. […] Data from the THAOS registry showed that ATTR amyloidosis primarily affects elderly men with a cardiac predominant phenotype. The prevalence of wild type (non-hereditary) ATTR amyloidosis increases with age with nearly all patients being 60 years of age. […] Cardiac amyloidosis is the dominant feature of wild-type ATTR.

• #5 AA (Inflammatory) Amyloidosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/335559-overview

  The reported prevalence of amyloidosis in RA ranges from 7%-26%. […] The frequency of renal amyloidosis in some populations with untreated FMF is almost 100%. […] Most available data to approximate the epidemiology of AA amyloidosis are derived from autopsies. The overall autopsy incidence of AA amyloidosis in Western nations ranges from 0.50-0.86%. […] In Japanese people, in whom the SAA 1.5 allele is far more common than in whites (37.4% vs 5.3%), the 1.5 allele is enriched among patients with RA and amyloidosis. […] In the United States, AA amyloidosis is more common in females, reflecting the fact that the major predisposing disease, RA, is predominantly a disorder of younger women and middle-aged men; hence, women are apt to have the disease for a longer period than men. […] Despite the statistical female predominance in terms of overall numbers of AA amyloidosis cases, males seem to have an earlier average age of onset. […] Age at onset of amyloidosis is related to the age at onset of the inflammatory disease, its severity, and the duration of the disease within the constraints imposed by the alleles of SAA carried by the patient.

• #5 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  The use of biologic therapies and of more intensive treatment protocols have essentially modified the natural history of inflammatory joint disease in developed countries, and it is expected that they may exert a significant influence on the incidence of amyloidosis in the coming decades. […] Finally, new cases of amyloidosis have been detected in groups that were not traditionally associated with amyloidosis.

• #5 Cardiac amyloidosis: a changing epidemiology with open challenges

  https://www.oaepublish.com/articles/2574-1209.2021.106

  Cardiac amyloidosis (CA) is increasingly diagnosed due to the advancements made in diagnostics and therapeutics in the last decades, particularly in the field of transthyretin-related CA. […] Studies that have used bone scintigraphy for screening at-risk conditions have shown that about one out of ten patients with heart failure with preserved ejection fraction (HFpEF), aortic stenosis undergoing valve replacement, or hypertrophic cardiomyopathy (HCM) diagnosed later in life might have an underlying or concomitant CA. […] The epidemiology of these conditions is also rapidly evolving. […] The epidemiological scenario of CA is rapidly evolving and this condition is increasingly diagnosed, particularly because of two recent discoveries: first, the possibility of performing a non-invasive diagnosis of ATTR-CA in a vast majority of cases and, second, the demonstrated efficacy of specific treatments aiming at interrupting the progression of myocardial TTR infiltration.

• #5 Cardiac Amyloidosis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1967220-overview

  Systemic amyloidosis (AL) is a rare disorder, and it is difficult to estimate the exact incidence due to changing diagnostic criteria. In Olmsted County, Minnesota, only 21 cases were diagnosed from Jan 1950 to Dec 1989. In this first population-based study, the incidence of AL was approximately 8.9 per million person-years. In the United States, approximately 2000-2500 cases of AL are diagnosed annually. […] Amyloidosis is reported as a cause of death in 1 in 1000 of the British population. […] AL is uncommon in non-White individuals and persons younger than 40 years, and it affects men and women equally. Senile amyloidosis, in which wild-type transthyretin (wt-TTR) accumulates in tissue and leads to the development of cardiac dysfunction, is 3 times more common in elderly Black patients compared with White patients (8.2% vs 2.7%, respectively) and is more common in males. Hereditary cardiac amyloidosis resulting from a mutation in TTR is more common in Black individuals than White persons; 23% of the patients have this variant. […] AL type is usually seen in persons older than age 50 years. Although unusual, it can occur as early as the third decade of life. Late-onset amyloidosis (senile) is seen in elderly patients and has a better prognosis than primary amyloidosis.

• #5 Epidemiology | oneAMYLOIDOSISvoice

  https://www.oneamyloidosisvoice.com/epidemiology

  Amyloidosis is a heterogeneous disease that results from the deposition of toxic insoluble beta-sheet fibrillar protein aggregates in different […] Amyloidosis is a rare complication of inflammatory bowel disease [IBD]; its low prevalence has hindered both descriptive and therapeutic […] Because amyloidosis is rare, the diagnosis is often delayed or the condition is not diagnosed. Therefore, it is difficult to know exactly how many people are

• #5 Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-025-03547-0

  Our review highlights the limitation in research that offers comprehensive global ATTR-specific epidemiology and mortality data thus hindering our understanding of its true global burden. […] Many studies included in this review originated in Europe and North America, and there was a notable absence of data from regions such as Africa and South America, suggesting geographical disparity in ATTR research. […] The true global prevalence of ATTR-PN remains unclear, though it is estimated to be between 10,000 and 40,000 persons globally, with endemic foci in Portugal, Sweden, and Japan. […] Robust epidemiological ATTR amyloidosis data requires further investigation in a larger sample of patients with diverse phenotypes across multiple countries and world regions.

• #5 Systemic AA amyloidosis: epidemiology, diagnosis, and management – Document – Gale Academic OneFile

  https://go.gale.com/ps/i.do?id=GALE%7CA400253707&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=11791349&p=AONE&sw=w

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] In this sense, the 123I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in systemic amyloidosis. […] Given the etiologic diversity of AA amyloidosis, common therapeutic strategies are scarce.

• #5

  https://smw.ch/index.php/smw/article/view/3485

  AIMS OF THE STUDY: Systemic amyloidoses are rare protein-folding diseases with heterogeneous, often nonspecific clinical presentations. […] 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. […] 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). […] 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] 2.334.87); it was 4.87 years (95% CI 3.147.22) in light-chain amyloidosis patients and 1.85 years (95% CI 1.483.66) in transthyretin amyloidosis patients, respectively.

• #5 AA Amyloidosis: An Evaluation of Epidemiology and Prevalence in the US and EU5 Countries – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/aa-amyloidosis-an-evaluation-of-epidemiology-and-prevalence-in-the-us-and-eu5-countries/

  AA amyloidosis (AAA) is a rare, systemic form of amyloidosis, which mostly occurs secondary to chronic inflammatory conditions, predominantly systemic arthritides. […] Understanding the epidemiology and prevalence of the disease is difficult, but is critical to improving the ability to diagnose the disease in a timely manner such that optimal treatment can be provided to patients. […] This research suggests an AAA prevalence of 9,100-15,500 patients in the US and approximately 6,200 in the EU5. […] Bringing together centers of excellence and thought leaders to develop a global patient registry would help to further define the epidemiology and prevalence of AAA, and increase awareness of this rare but devastating disease.

• #6 Secondary amyloidosis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Secondary_amyloidosis_epidemiology_and_demographics

  The incidence of AA amyloidosis is approximately 0.16 per 100,000 individuals in 2008 in the United kingdom. […] The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. […] Secondary amyloidosis more commonly affects children. […] Men are more commonly affected by amyloidosis than women. […] The prevalence of AA amyloidosis is 5,000 to 10,000 per 100,000 individuals with chronic inflammatory process per year worldwide. […] There is no racial predilection to secondary amyloidosis.

• #6 Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication | Methodist DeBakey Cardiovascular J

  https://journal.houstonmethodist.org/articles/10.14797/mdcvj.1070

  Primary or light chain (AL) amyloidosis, the most common type of systemic amyloidosis, occurs when the free light chains normally associated with immunoglobulins are produced in excess by clonal or frankly malignant plasma cells. […] It is a relatively rare condition, with a worldwide incidence of 5.1 to 12.8 cases per million person-years. In the United States, between 1,275 and 3,200 new cases are diagnosed annually, yielding an incidence of approximately 9 to 14 cases per million person-years and a prevalence of 40.5 cases per million as of 2015—a significant increase from the prevalence of 15.5 cases per million reported in 2007. It is hypothesized that this rising prevalence is due to a growing awareness of the disease and its manifestations as well as improved treatments that have lowered the mortality rate.

• #6 Epidemiology of Amyloidosis and Genetic Pathways to Diagnosis and Typing

  https://www.mdpi.com/2673-6357/2/3/27

  Using Swedish hospital discharge data, we identified 221 patients with ATTRv amyloidosis, giving an incidence of 2/million. The disease is well-known in Sweden, which is, together with Portugal and Japan, an endemic area for this disease. Surprisingly, our geographic survey of the cases showed that the disease was also highly endemic in Sweden, despite large population movements from the rural to urban areas. The incidence in the province with the highest incidence was 100 times higher than in the rest of Sweden. In a separate study, we assessed cancer incidence in the population diagnosed with ATTRv amyloidosis. Risk of non-Hodgkin lymphoma increased five-fold, including the diffuse large B cell type, with a six-fold increase.

• #6 AA (Inflammatory) Amyloidosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/335559-overview

  The reported prevalence of amyloidosis in RA ranges from 7%-26%. […] The frequency of renal amyloidosis in some populations with untreated FMF is almost 100%. […] Most available data to approximate the epidemiology of AA amyloidosis are derived from autopsies. The overall autopsy incidence of AA amyloidosis in Western nations ranges from 0.50-0.86%. […] In Japanese people, in whom the SAA 1.5 allele is far more common than in whites (37.4% vs 5.3%), the 1.5 allele is enriched among patients with RA and amyloidosis. […] In the United States, AA amyloidosis is more common in females, reflecting the fact that the major predisposing disease, RA, is predominantly a disorder of younger women and middle-aged men; hence, women are apt to have the disease for a longer period than men. […] Despite the statistical female predominance in terms of overall numbers of AA amyloidosis cases, males seem to have an earlier average age of onset. […] Age at onset of amyloidosis is related to the age at onset of the inflammatory disease, its severity, and the duration of the disease within the constraints imposed by the alleles of SAA carried by the patient.

• #6 Cardiac amyloidosis: a changing epidemiology with open challenges

  https://www.oaepublish.com/articles/2574-1209.2021.106

  Cardiac amyloidosis (CA) is increasingly diagnosed due to the advancements made in diagnostics and therapeutics in the last decades, particularly in the field of transthyretin-related CA. […] Studies that have used bone scintigraphy for screening at-risk conditions have shown that about one out of ten patients with heart failure with preserved ejection fraction (HFpEF), aortic stenosis undergoing valve replacement, or hypertrophic cardiomyopathy (HCM) diagnosed later in life might have an underlying or concomitant CA. […] The epidemiology of these conditions is also rapidly evolving. […] The epidemiological scenario of CA is rapidly evolving and this condition is increasingly diagnosed, particularly because of two recent discoveries: first, the possibility of performing a non-invasive diagnosis of ATTR-CA in a vast majority of cases and, second, the demonstrated efficacy of specific treatments aiming at interrupting the progression of myocardial TTR infiltration.

• #6 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] The most comprehensive epidemiological study in recent decades was conducted by Kyle et al at the Mayo Clinic with data collected from 1950-1990 from the general population residing in Olmsted County, MN, USA. […] These authors reported an incidence of AL amyloidosis in nine cases per million person-years (95% confidence interval; 5.1-12.8 cases per million person-years).

• #6 Re-Definition of the Epidemiology of Cardiac Amyloidosis

  https://www.mdpi.com/2227-9059/10/7/1566

  Based on these results, it appears important to change our approach to patients undergoing an echocardiogram, in order to systematically search for CA red flags, regardless of the exam indication; in this way, the echocardiography laboratory will become essential for obtaining an early diagnosis. […] In the last few years, the following subgroups of patients have been found to have a higher prevalence of CA: elderly subjects > 75 years, patients with LVH hospitalized for heart failure with preserved ejection fraction (HFpEF), subjects operated on for bilateral carpal tunnel syndrome (CTS), patients with cardiac hypertrophy not explained by concomitant factors and individuals with aortic valve stenosis (AS). […] The latest advances in the knowledge of the epidemiology of CA have contributed to increasing the awareness in the scientific community, changing the approach to the disease. […] Specific clinical settings have been recognized as strongly associated with CA but further studies are needed to better characterize these populations, mainly to stratify patient risk and evaluate which patients are ideal candidates for the newly available disease-modifying therapies.

• #7 Cardiac amyloidosis: epidemiology, diagnosis and therapy

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-19/cardiac-amyloidosis-epidemiology-diagnosis-and-therapy

  Published data on the epidemiology of cardiac amyloidosis are based mainly on single-centre studies or population registries. A recent study among Medicare beneficiaries aged 65 years in the USA showed an increase in both the incidence and prevalence rates of cardiac amyloidosis from 2000 to 2012. […] Data from the THAOS registry showed that ATTR amyloidosis primarily affects elderly men with a cardiac predominant phenotype. The prevalence of wild type (non-hereditary) ATTR amyloidosis increases with age with nearly all patients being 60 years of age. […] Cardiac amyloidosis is the dominant feature of wild-type ATTR.

• #7 DOAJ Logotype

  https://doaj.org/article/19bebe0d4fd144d7a600e783c6da03d9

  Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States. […] The prevalence of AL amyloidosis increased significantly between 2007 and 2015, from 15.5 cases per million in 2007 to 40.5 in 2015, an annual percentage change (APC) of 12% (P .001). […] There was an increase in AL amyloidosis prevalence over a 9-year period coupled with stable incidence rates. […] Although there is no diagnosis code specific to AL amyloidosis and no validated method for identifying this condition using claims data, extrapolating from our data, there are at least 12 000 adults in the United States living with AL amyloidosis, and the number seems likely to rise.

• #7 Cardiac amyloidosis: a changing epidemiology with open challenges

  https://www.oaepublish.com/articles/2574-1209.2021.106

  The description of the evolving epidemiology of CA remains scant in the literature so far. […] A stable prevalence of AL-CA has also been recently reported in Italy, with data from a single-center 20-year study from Florence including 654 CA patients and a multi-center 5-year Italian survey including 642 CA patients both showing an exponential increase in the number of ATTR-CA diagnoses following 2016. […] Data from the National Amyloidosis Center in London, which provides care to a large cohort in the United Kingdom, suggest that the diagnosis of ATTRwt-CA has increased exponentially there as well. […] Overall, the above evidence confirms that at least one out of ten patients with HFpEF, AS, or HCM diagnosed later in life might have an overlooked ATTR-CA, and systematic screening of patients with these conditions using bone scintigraphy has been proposed by experts.

• #7 AA (Inflammatory) Amyloidosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/335559-overview

  AA amyloidosis occurs in the course of chronic inflammatory diseases (infectious and noninfectious), hereditary periodic fevers, and with certain neoplasms, such as Hodgkin lymphoma and renal cell carcinoma. […] The absolute prevalence of AA amyloidosis is difficult to ascertain because it depends on both the occurrence of predisposing inflammatory disorders and the proportion of individuals with those conditions who develop tissue amyloid deposition. […] AA amyloidosis is far less common in the United States than in other countries, even in the setting of the same inflammatory disease. […] Currently, rheumatic diseases such as rheumatoid arthritis (RA), ankylosing spondylitis (AS), psoriatic arthritis, and juvenile idiopathic arthritis are the most frequent causes (70%) of AA amyloidosis.

• #7 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] The most comprehensive epidemiological study in recent decades was conducted by Kyle et al at the Mayo Clinic with data collected from 1950-1990 from the general population residing in Olmsted County, MN, USA. […] These authors reported an incidence of AL amyloidosis in nine cases per million person-years (95% confidence interval; 5.1-12.8 cases per million person-years).

• #8 DOAJ Logotype

  https://doaj.org/article/19bebe0d4fd144d7a600e783c6da03d9

  Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States. […] The prevalence of AL amyloidosis increased significantly between 2007 and 2015, from 15.5 cases per million in 2007 to 40.5 in 2015, an annual percentage change (APC) of 12% (P .001). […] There was an increase in AL amyloidosis prevalence over a 9-year period coupled with stable incidence rates. […] Although there is no diagnosis code specific to AL amyloidosis and no validated method for identifying this condition using claims data, extrapolating from our data, there are at least 12 000 adults in the United States living with AL amyloidosis, and the number seems likely to rise.

• #8 Cardiac amyloidosis: a changing epidemiology with open challenges

  https://www.oaepublish.com/articles/2574-1209.2021.106

  The description of the evolving epidemiology of CA remains scant in the literature so far. […] A stable prevalence of AL-CA has also been recently reported in Italy, with data from a single-center 20-year study from Florence including 654 CA patients and a multi-center 5-year Italian survey including 642 CA patients both showing an exponential increase in the number of ATTR-CA diagnoses following 2016. […] Data from the National Amyloidosis Center in London, which provides care to a large cohort in the United Kingdom, suggest that the diagnosis of ATTRwt-CA has increased exponentially there as well. […] Overall, the above evidence confirms that at least one out of ten patients with HFpEF, AS, or HCM diagnosed later in life might have an overlooked ATTR-CA, and systematic screening of patients with these conditions using bone scintigraphy has been proposed by experts.

• #8 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] The most comprehensive epidemiological study in recent decades was conducted by Kyle et al at the Mayo Clinic with data collected from 1950-1990 from the general population residing in Olmsted County, MN, USA. […] These authors reported an incidence of AL amyloidosis in nine cases per million person-years (95% confidence interval; 5.1-12.8 cases per million person-years).

• #8 Systemic AA amyloidosis: epidemiology, diagnosis, and management | CLEP

  https://www.dovepress.com/systemic-aa-amyloidosis-epidemiology-diagnosis-and-management-peer-reviewed-fulltext-article-CLEP

  Recently, similar studies have been carried out in two European regions. […] Pinney et al from the National Health Service National Amyloidosis Centre in the UK, estimated a global incidence of amyloidosis in England of five cases per million person-years. […] Of these, close to three cases per million person-years would have the AL type, and one case, the AA type. […] The percent increase in the frequency of AL amyloidosis with respect to the AA type can be explained by earlier diagnosis of many predisposing conditions for AA amyloidosis and widespread access to more effective treatments that better control many disease processes. […] Better access to antimicrobial agents and a stricter control of tuberculosis have led to a significant decrease in the frequency of chronic infectious diseases in developed countries, where autoimmune diseases such as RA, ankylosing spondylitis, chronic juvenile arthritis, and inflammatory bowel disease, or hereditary inflammatory diseases, such as FMF, TNF receptor-associated periodic syndrome, or Muckle-Wells syndrome, can account for up to 90% of recent cases of amyloidosis.

• #9

  http://uu.diva-portal.org/smash/record.jsf?pid=diva2:1831921

  AL (immunoglobulin light chain) amyloidosis is often associated with delayed diagnosis and thereby high early mortality that is not overcome by contemporary treatment. […] However, population-based reports are scarce regarding epidemiology as well as treatment outcomes. […] Paper IV was a population-based epidemiological study in which we could determine the standardized incidence of systemic AL amyloidosis to 12.0 (95% CI 9.3-14.7) per million person-years for Uppsala County, without significant change during the period 2000-2020. […] Prolonged overall survival was observed over time, and there was also a decrease in early mortality, indicating earlier diagnosis of especially patients with cardiac involvement. […] Population-based incidence, prevalence and survival of systemic AL amyloidosis in Sweden.

• #9 Systemic AA amyloidosis: epidemiology, diagnosis, and management – Document – Gale Academic OneFile

  https://go.gale.com/ps/i.do?id=GALE%7CA400253707&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=11791349&p=AONE&sw=w

  The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. […] While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. […] The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. […] In this sense, the 123I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in systemic amyloidosis. […] Given the etiologic diversity of AA amyloidosis, common therapeutic strategies are scarce.

• #9 Re-Definition of the Epidemiology of Cardiac Amyloidosis

  https://www.mdpi.com/2227-9059/10/7/1566

  The epidemiology of cardiac amyloidosis (CA), traditionally considered a rare and incurable disease, has changed drastically over the last ten years, particularly due to the advances in diagnostic methods and therapeutic options in the field of transthyretin CA (ATTR-CA). […] The most significant innovations concern ATTR-CA, whose diagnostic work-up has been deeply transformed by the possibility of reaching a non-biopsy diagnosis through cardiac scintigraphy with bone tracers. […] Consequently, the diagnoses of ATTR-CA have increased exponentially and patients have been diagnosed earlier in the disease course, also because of an increased awareness of CA among physicians of many specialties. […] A prospective multicentric study (AC-TIVE study) conducted in Italy investigated the prevalence of CA among patients with echocardiographic red flags and evaluated their diagnostic accuracy.

• #10 A population-based cohort study of the epidemiology of light-chain amyloidosis in Taiwan | Scientific Reports

  https://www.nature.com/articles/s41598-022-18990-3

  The incidence rate of AL (light-chain) amyloidosis is not known in Asia. […] We conducted a retrospective cohort study using the Taiwan National Healthcare Insurance Research database and Death Registry to estimate incidence and all-cause case fatality rates, and characteristics of patients with AL amyloidosis in Taiwan. […] AL amyloidosis age-adjusted annual incidence was 5.73 per million population in 2016 and 5.26 per million population in 2019. […] The incidence of AL amyloidosis in Taiwan appears to be similar to Western countries. […] Our study showed that AL amyloidosis is a rare disease in Taiwan, with age-adjusted incidence rates of 5.26 to 6.55 per million population. […] There was no apparent change in the incidence of AL amyloidosis over the 4 years of the study. […] Our population-based study suggests that the incidence of AL amyloidosis in Taiwan is similar to western countries. […] The lower frequencies of cardiac, renal, and liver co-morbidities in our study could be related to better survival noted in this cohort. […] In conclusion, the incidence of AL amyloidosis in Taiwan appears to be similar to Western countries.

• #10 Amyloidosis – Wikipedia

  https://en.wikipedia.org/wiki/Amyloidosis

  Amyloidosis has a combined estimated prevalence of 30 per 100,000 persons with the three most common forms being AL, ATTR, and AA. […] AL has the highest incidence at approximately 12 cases per million persons per year and an estimated prevalence of 30,000 to 45,000 cases in the US and European Union. […] AA amyloidoses is the most common form in developing countries and can complicate longstanding infections with tuberculosis, osteomyelitis, and bronchiectasis. […] Wild-type transthyretin (ATTR) amyloidosis is found in a quarter of elderly at postmortem. […] ATTR is found in 13-19% of people experiencing heart failure with preserved ejection fraction, making it a very common form of systemic amyloidosis.

• #10 Epidemiology, outcomes, healthcare resource utilisation, and inequities among amyloidosis patients in the UK, an observational study | CPRD

  https://www.cprd.com/approved-studies/epidemiology-outcomes-healthcare-resource-utilisation-and-inequities-among

  Amyloidosis is a rare disease, multisystemic condition in which a protein is deposited in various organs and tissues, leading to their dysfunction and potentially fatal consequences. […] Currently, real-life data on the patient journey, including clinical outcomes, disease progression, treatment patterns, healthcare resource utilisation and management of patients with amyloidosis is limited. […] The overall aim of this study is to assess the distribution of the disease, pre- and post-diagnosis disease journeys, including baseline characteristics, treatment patterns and selected clinical, economic, and humanistic outcomes in patients with amyloidosis in the United Kingdom (UK). […] A recent study using data from the UK National Amyloidosis Centre database, reported that the number of cases of amyloidosis increased by 670% from 1987-1999 to 2010-2019.

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