Autoimmune lymphoproliferative syndrome epidemiology and demographics: Difference between revisions

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Latest revision as of 19:34, 17 September 2021

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

The incidence of Autoimmune lymphoproliferative syndrome(ALPS) is unknown as many cases remain undiagnosed or misdiagnosed.^[1]

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

The mortality rate is 15% in patients with ALPS-FAS by age 50.^[2]

Age

Patients of all age groups may develop Autoimmune lymphoproliferative syndrome.^[3]
Typically ALPS appears in early childhood. The median age at diagnosis is 3 years.^[4]

Race

There is no racial predilection to Autoimmune Lymphoproliferative Syndrome.

Gender

Males are more commonly affected by Autoimmune lymphoproliferative syndrome than females. The male to female ratio is 14:4.^[3]

Region

There is no information about any specific region where Autoimmune lymphoproliferative syndrome is prevalent.

References

↑ 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.
↑ ^2.0 ^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.
↑ ^3.0 ^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.
↑ 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.

[RaoOliveira2011-1] 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.

[ShahWu2014-2] 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.

[PriceShaw2014-3] 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.

[Rieux-LaucatMagérus-Chatinet2018-4] 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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@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Autoimmune lymphoproliferative syndrome}}
-{{CMG}}
+{{CMG}} {{AE}} {{SharmiB}}
-Please help WikiDoc by adding more content here.  It's easy!  Click  [[Help:How_to_Edit_a_Page|here]]  to learn about editing.
 ==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===
-*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 [[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===
-*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===
-*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===
-*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===
-*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===
-*[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===
-*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==
@@ Line 48: / Line 40: @@
 [[Category:Hematology]]
+[[Category:Up To Date]]
-{{WH}}
-{{WS}}