Autoimmune lymphoproliferative syndrome epidemiology and demographics: Difference between revisions

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{{Autoimmune lymphoproliferative syndrome}}
{{Autoimmune lymphoproliferative syndrome}}
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==Overview==
==Overview==
[[Autoimmune lymphoproliferative syndrome]] (ALPS) is a [[rare]] [[disease]] that mostly affects [[children]] of early age. The [[incidence]] and [[prevalence]] of ALPS are unknown. [[Male]] predominance has been found.


==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Incidence===
===Incidence===
* The incidence of Autoimmune lymphoproliferative syndrome(ALPS) is unknown as many cases remain undiagnosed or misdiagnosed.<ref name="RaoOliveira2011">{{cite journal|last1=Rao|first1=V. Koneti|last2=Oliveira|first2=João Bosco|title=How I treat autoimmune lymphoproliferative syndrome|journal=Blood|volume=118|issue=22|year=2011|pages=5741–5751|issn=0006-4971|doi=10.1182/blood-2011-07-325217}}</ref>
 
*The [[incidence]] of [[Autoimmune lymphoproliferative syndrome]](ALPS) is unknown as many cases remain undiagnosed or misdiagnosed.<ref name="RaoOliveira2011">{{cite journal|last1=Rao|first1=V. Koneti|last2=Oliveira|first2=João Bosco|title=How I treat autoimmune lymphoproliferative syndrome|journal=Blood|volume=118|issue=22|year=2011|pages=5741–5751|issn=0006-4971|doi=10.1182/blood-2011-07-325217}}</ref>


===Prevalence===
===Prevalence===
*The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
 
*In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
*The [[prevalence]] is not known as many cases are left unidentified. A total of 500 [[patients]] with ALPS have been identified, from more than 300 families.<ref name="ShahWu2014">{{cite journal|last1=Shah|first1=Shaili|last2=Wu|first2=Eveline|last3=Rao|first3=V. Koneti|last4=Tarrant|first4=Teresa K.|title=Autoimmune Lymphoproliferative Syndrome: an Update and Review of the Literature|journal=Current Allergy and Asthma Reports|volume=14|issue=9|year=2014|issn=1529-7322|doi=10.1007/s11882-014-0462-4}}</ref>
*The prevalence of [disease/malignancy] is estimated to be [number] cases annually.


===Case-fatality rate/Mortality rate===
===Case-fatality rate/Mortality rate===
*In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%.
 
*The case-fatality rate/mortality rate of [disease name] is approximately [number range].
* The [[mortality rate]] is 15% in [[patients]] with [[ALPS]]-FAS by [[age]] 50.<ref name="ShahWu2014">{{cite journal|last1=Shah|first1=Shaili|last2=Wu|first2=Eveline|last3=Rao|first3=V. Koneti|last4=Tarrant|first4=Teresa K.|title=Autoimmune Lymphoproliferative Syndrome: an Update and Review of the Literature|journal=Current Allergy and Asthma Reports|volume=14|issue=9|year=2014|issn=1529-7322|doi=10.1007/s11882-014-0462-4}}</ref>


===Age===
===Age===
*Patients of all age groups may develop [disease name].
 
*The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
*[[Patients]] of all [[age]] groups may develop [[Autoimmune lymphoproliferative syndrome]].<ref name="PriceShaw2014">{{cite journal|last1=Price|first1=Susan|last2=Shaw|first2=Pamela A.|last3=Seitz|first3=Amy|last4=Joshi|first4=Gyan|last5=Davis|first5=Joie|last6=Niemela|first6=Julie E.|last7=Perkins|first7=Katie|last8=Hornung|first8=Ronald L.|last9=Folio|first9=Les|last10=Rosenberg|first10=Philip S.|last11=Puck|first11=Jennifer M.|last12=Hsu|first12=Amy P.|last13=Lo|first13=Bernice|last14=Pittaluga|first14=Stefania|last15=Jaffe|first15=Elaine S.|last16=Fleisher|first16=Thomas A.|last17=Rao|first17=V. Koneti|last18=Lenardo|first18=Michael J.|title=Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations|journal=Blood|volume=123|issue=13|year=2014|pages=1989–1999|issn=0006-4971|doi=10.1182/blood-2013-10-535393}}</ref>
*[Disease name] commonly affects individuals younger than/older than [number of years] years of age.
*Typically [[ALPS]] appears in early [[childhood]]. The [[median]] age at [[diagnosis]] is 3 years.<ref name="Rieux-LaucatMagérus-Chatinet2018">{{cite journal|last1=Rieux-Laucat|first1=Frédéric|last2=Magérus-Chatinet|first2=Aude|last3=Neven|first3=Bénédicte|title=The Autoimmune Lymphoproliferative Syndrome with Defective FAS or FAS-Ligand Functions|journal=Journal of Clinical Immunology|volume=38|issue=5|year=2018|pages=558–568|issn=0271-9142|doi=10.1007/s10875-018-0523-x}}</ref>
*[Chronic disease name] is usually first diagnosed among [age group].
*[Acute disease name] commonly affects [age group].


===Race===
===Race===
*There is no racial predilection to [disease name].
 
*[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
*There is no [[racial]] predilection to [[Autoimmune Lymphoproliferative Syndrome]].
 
===Gender===
===Gender===
*[Disease name] affects men and women equally.
 
*[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
*[[Males]] are more commonly affected by [[Autoimmune lymphoproliferative syndrome]] than [[females]]. The [[male]] to [[female]] ratio is 14:4.<ref name="PriceShaw2014">{{cite journal|last1=Price|first1=Susan|last2=Shaw|first2=Pamela A.|last3=Seitz|first3=Amy|last4=Joshi|first4=Gyan|last5=Davis|first5=Joie|last6=Niemela|first6=Julie E.|last7=Perkins|first7=Katie|last8=Hornung|first8=Ronald L.|last9=Folio|first9=Les|last10=Rosenberg|first10=Philip S.|last11=Puck|first11=Jennifer M.|last12=Hsu|first12=Amy P.|last13=Lo|first13=Bernice|last14=Pittaluga|first14=Stefania|last15=Jaffe|first15=Elaine S.|last16=Fleisher|first16=Thomas A.|last17=Rao|first17=V. Koneti|last18=Lenardo|first18=Michael J.|title=Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations|journal=Blood|volume=123|issue=13|year=2014|pages=1989–1999|issn=0006-4971|doi=10.1182/blood-2013-10-535393}}</ref>


===Region===
===Region===
*The majority of [disease name] cases are reported in [geographical region].


*[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
*There is no information about any specific region where [[Autoimmune lymphoproliferative syndrome]] is prevalent.
 
===Developed Countries===
 
===Developing Countries===


==References==
==References==
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[[Category:Hematology]]
[[Category:Hematology]]
 
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Latest revision as of 19:34, 17 September 2021

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S

Overview

Autoimmune lymphoproliferative syndrome (ALPS) is a rare disease that mostly affects children of early age. The incidence and prevalence of ALPS are unknown. Male predominance has been found.

Epidemiology and Demographics

Incidence

Prevalence

  • The prevalence is not known as many cases are left unidentified. A total of 500 patients with ALPS have been identified, from more than 300 families.[2]

Case-fatality rate/Mortality rate

Age

Race

Gender

Region

References

  1. ↑ Rao, V. Koneti; Oliveira, João Bosco (2011). "How I treat autoimmune lymphoproliferative syndrome". Blood. 118 (22): 5741–5751. doi:10.1182/blood-2011-07-325217. ISSN 0006-4971.
  2. ↑ 2.0 2.1 Shah, Shaili; Wu, Eveline; Rao, V. Koneti; Tarrant, Teresa K. (2014). "Autoimmune Lymphoproliferative Syndrome: an Update and Review of the Literature". Current Allergy and Asthma Reports. 14 (9). doi:10.1007/s11882-014-0462-4. ISSN 1529-7322.
  3. ↑ 3.0 3.1 Price, Susan; Shaw, Pamela A.; Seitz, Amy; Joshi, Gyan; Davis, Joie; Niemela, Julie E.; Perkins, Katie; Hornung, Ronald L.; Folio, Les; Rosenberg, Philip S.; Puck, Jennifer M.; Hsu, Amy P.; Lo, Bernice; Pittaluga, Stefania; Jaffe, Elaine S.; Fleisher, Thomas A.; Rao, V. Koneti; Lenardo, Michael J. (2014). "Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations". Blood. 123 (13): 1989–1999. doi:10.1182/blood-2013-10-535393. ISSN 0006-4971.
  4. ↑ Rieux-Laucat, Frédéric; Magérus-Chatinet, Aude; Neven, Bénédicte (2018). "The Autoimmune Lymphoproliferative Syndrome with Defective FAS or FAS-Ligand Functions". Journal of Clinical Immunology. 38 (5): 558–568. doi:10.1007/s10875-018-0523-x. ISSN 0271-9142.
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