Evidence map›Paper›PMID 35854312›Full record

SynthesisOrphanet journal of rare diseases2022

Global epidemiology of amyloid light-chain amyloidosis.

Nishant Kumar, Nicole J Zhang, Dasha Cherepanov, Dorothy Romanus, Michael Hughes, Douglas V Faller

Abstract readSystematic Review
In one paragraph

Synthesis in Orphanet journal of rare diseases, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 54 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
54citing papers in PubMed, 2 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

54 citing papers in PubMed, 2 syntheses or guidelines pooled it.

  1. Guideline
  2. Pooled it
  3. Article
  4. Article
  5. Article
  6. Pathogenesis and kidney prognosis of renal amyloidosis.Cellular and molecular life sciences : CMLS · 2026
    Review
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Review
  14. Review
  15. Article
  16. Article
  17. Cardiac Amyloidosis: Tribulations and New Frontiers.Journal of personalized medicine · 2025
    Review
  18. Article
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors.

Nishant KumarRwHealth, Level 39, One Canada Square, Canary Wharf, London, E14 5AB, GB, UK.
Nicole J ZhangRwHealth, Level 39, One Canada Square, Canary Wharf, London, E14 5AB, GB, UK.
Dasha CherepanovTakeda Development Center Americas, Inc., 95 Hayden Ave., Lexington, MA, 02421, USA. Dasha.Cherepanov@Takeda.com.ORCID 0000-0001-6130-2406
Dorothy RomanusTakeda Development Center Americas, Inc., 95 Hayden Ave., Lexington, MA, 02421, USA.
Michael HughesRwHealth, Level 39, One Canada Square, Canary Wharf, London, E14 5AB, GB, UK.
Douglas V FallerTakeda Development Center Americas, Inc., 95 Hayden Ave., Lexington, MA, 02421, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundAmyloid 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.

methodsThis 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. A systematic literature review (SLR) was conducted to identify country-specific, age- and gender-specific diagnosed incidence of AL amyloidosis and observed survival data-point inputs for an incidence-to-prevalence model. Extrapolations were used to estimate incidence and prevalence for countries without registry or published epidemiological data.

resultsOf 171 publications identified in the SLR, 10 records met the criteria for data extraction, and two records were included in the final incidence-to-prevalence model. 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.

conclusionsOrphan medicinal product designation criteria of the European Medicines Agency or Electronic Code of Federal Regulations indicate that a disease must not affect > 5 in 10,000 people across the European Union or affect < 200,000 people in the US. 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.

Indexed as

Immunoglobulin Light-chain AmyloidosisEuropeHumansIncidencePrevalenceRegistriesUnited StatesAmyloid light-chain amyloidosisEpidemiologyGlobalIncidencePrevalenceRare disease

Identifiers

PMID35854312
PMCPMC9295439

What OpenQuestion holds

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LicenceCC BY
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.