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Incidence and survival in non-hereditary amyloidosis in Sweden

Hemminki, Kari LU ; Li, Xinjun LU ; Försti, Asta LU ; Sundquist, Jan LU and Sundquist, Kristina LU (2012) In BMC Public Health 12(974).
Abstract
Background: Amyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics. Methods: Amyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008. Results: The incidence of non-hereditary amyloidosis in 949 patients was 8.29 per million person-years and the diagnostic age with the highest incidence was over 65 years. Secondary systemic amyloidosis showed an incidence of 1 per million and a female excess and the largest number of subsequent rheumatoid arthritis deaths; the median survival was 4 years.... (More)
Background: Amyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics. Methods: Amyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008. Results: The incidence of non-hereditary amyloidosis in 949 patients was 8.29 per million person-years and the diagnostic age with the highest incidence was over 65 years. Secondary systemic amyloidosis showed an incidence of 1 per million and a female excess and the largest number of subsequent rheumatoid arthritis deaths; the median survival was 4 years. However, as rheumatoid arthritis deaths also occurred in other diagnostic subtypes, the incidence of secondary systemic amyloidosis was likely to be about 2.0 per million. The median survival of patients with organ-limited amyloidosis was 6 years. Most myeloma deaths occurred in patients diagnosed with unspecified or 'other' amyloidosis. These subtypes probably accounted for most of immunoglobulin light chain (AL) amyloidosis cases; the median survival time was 3 years. Conclusions: The present diagnostic categorization cannot single out AL amyloidosis in the Swedish discharge data but, by extrapolation from myeloma cases, an incidence of 3.2 per million could be ascribed to AL amyloidosis. Similarly, based on rheumatoid arthritis death rates, an incidence of 2.0 could be ascribed to secondary systemic amyloidosis. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
BMC Public Health
volume
12
issue
974
publisher
BioMed Central
external identifiers
  • wos:000311618700001
  • scopus:84868641997
ISSN
1471-2458
DOI
10.1186/1471-2458-12-974
language
English
LU publication?
yes
id
83cb167a-fecd-405d-ae1d-6fcfef8bb431 (old id 3388249)
date added to LUP
2013-02-01 07:03:27
date last changed
2017-09-17 05:48:36
@article{83cb167a-fecd-405d-ae1d-6fcfef8bb431,
  abstract     = {Background: Amyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics. Methods: Amyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008. Results: The incidence of non-hereditary amyloidosis in 949 patients was 8.29 per million person-years and the diagnostic age with the highest incidence was over 65 years. Secondary systemic amyloidosis showed an incidence of 1 per million and a female excess and the largest number of subsequent rheumatoid arthritis deaths; the median survival was 4 years. However, as rheumatoid arthritis deaths also occurred in other diagnostic subtypes, the incidence of secondary systemic amyloidosis was likely to be about 2.0 per million. The median survival of patients with organ-limited amyloidosis was 6 years. Most myeloma deaths occurred in patients diagnosed with unspecified or 'other' amyloidosis. These subtypes probably accounted for most of immunoglobulin light chain (AL) amyloidosis cases; the median survival time was 3 years. Conclusions: The present diagnostic categorization cannot single out AL amyloidosis in the Swedish discharge data but, by extrapolation from myeloma cases, an incidence of 3.2 per million could be ascribed to AL amyloidosis. Similarly, based on rheumatoid arthritis death rates, an incidence of 2.0 could be ascribed to secondary systemic amyloidosis.},
  author       = {Hemminki, Kari and Li, Xinjun and Försti, Asta and Sundquist, Jan and Sundquist, Kristina},
  issn         = {1471-2458},
  language     = {eng},
  number       = {974},
  publisher    = {BioMed Central},
  series       = {BMC Public Health},
  title        = {Incidence and survival in non-hereditary amyloidosis in Sweden},
  url          = {http://dx.doi.org/10.1186/1471-2458-12-974},
  volume       = {12},
  year         = {2012},
}