Autoimmune polyendocrine syndrome: Difference between revisions
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== Management == |
== Management == |
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[[File:Ketoconazole3Dan.gif|thumb|160 px|Ketoconazole]] |
[[File:Ketoconazole3Dan.gif|thumb|160 px|Ketoconazole]] |
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Immunosupressive therapy may also be used in ''type I''.Management in the case of ''type 1'', finds that [[ketoconazole]] may be used to treat the affected individual.<ref name=emed/> The component diseases are managed as usual, the challenge is to detect the possibility of any of the syndromes, and to anticipate other manifestations. For example, in a person with known Type 2 autoimmune polyendocrine syndrome but no features of [[Addison's disease]], regular screening for [[antibody|antibodies]] against [[21-hydroxylase]] may prompt early intervention and [[hydrocortisone]] replacement to prevent characteristic crises. |
Immunosupressive therapy may also be used in ''type I''.Management in the case of ''type 1''<ref>{{Cite journal|last=Weiler|first=Fernanda Guimarães|last2=Dias-da-Silva|first2=Magnus R.|last3=Lazaretti-Castro|first3=Marise|date=2012-02-01|title=Autoimmune polyendocrine syndrome type 1: case report and review of literature|url=http://www.scielo.br/scielo.php?script=sci_abstract&pid=S0004-27302012000100009&lng=en&nrm=iso&tlng=en|journal=Arquivos Brasileiros de Endocrinologia & Metabologia|volume=56|issue=1|pages=54–66|doi=10.1590/S0004-27302012000100009|issn=0004-2730}}</ref>, finds that [[ketoconazole]] may be used to treat the affected individual.<ref name=emed/> The component diseases are managed as usual, the challenge is to detect the possibility of any of the syndromes, and to anticipate other manifestations. For example, in a person with known Type 2 autoimmune polyendocrine syndrome but no features of [[Addison's disease]], regular screening for [[antibody|antibodies]] against [[21-hydroxylase]] may prompt early intervention and [[hydrocortisone]] replacement to prevent characteristic crises. |
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===Prognosis=== |
===Prognosis=== |
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Revision as of 19:37, 6 May 2017
 | This article needs more reliable medical references for verification or relies too heavily on primary sources. (April 2017) |  |
| Autoimmune polyendocrine syndrome | |
|---|---|
| Specialty | Endocrinology  |
Autoimmune polyendocrine syndromes (APSs), also called autoimmune polyglandular syndromes (APSs), polyglandular autoimmune syndromes (PGASs),[1] or polyendocrine autoimmune syndromes, are a heterogeneous group[2] of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can be affected.There are three types of APS or (in terms that mean the same thing) three APSs, and there are a number of other diseases which have endocrine autoimmunity as one of their features.[medical citation needed]
Types
- Autoimmune polyendocrine syndrome type 1 [3]
- Autoimmune polyendocrine syndrome type 2[4]
- The third form is the X-linked polyendocrinopathy, immunodeficiency and diarrhea-syndrome, called IPEX (syndrome). This is due to mutation of the FOXP3 gene on the X chromosome. Most develop diabetes and diarrhea and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might suffer mild disease.[5][6]
Cause
![[icon]](/wiki/File:Wiki_letter_w_cropped.svg){kind=small} | This section is empty. You can help by adding to it. (April 2017) |
Diagnosis
| This section is empty. You can help by adding to it. (April 2017) |
Differential diagnosis
Management
Immunosupressive therapy may also be used in type I.Management in the case of type 1[7], finds that ketoconazole may be used to treat the affected individual.[3] The component diseases are managed as usual, the challenge is to detect the possibility of any of the syndromes, and to anticipate other manifestations. For example, in a person with known Type 2 autoimmune polyendocrine syndrome but no features of Addison's disease, regular screening for antibodies against 21-hydroxylase may prompt early intervention and hydrocortisone replacement to prevent characteristic crises.
Prognosis
See also
References
- ^ "Polyglandular Autoimmune Syndromes: Immunogenetics and Long-Term Follow-Up". Retrieved 1 July 2013.
{{cite journal}}: Cite journal requires|journal=(help); Unknown parameter|deadurl=ignored (|url-status=suggested) (help) - ^ Eisenbarth GS, Gottlieb PA (2004). "Autoimmune polyendocrine syndromes". N. Engl. J. Med. 350 (20): 2068–79. doi:10.1056/NEJMra030158. PMID 15141045.
- ^ a b "Type I Polyglandular Autoimmune Syndrome: Background, Pathophysiology, Epidemiology". 2017-01-06.
{{cite journal}}: Cite journal requires|journal=(help) - ^ "Autoimmune polyglandular syndrome type 2 | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2017-04-20.
- ^ Reference, Genetics Home. "IPEX syndrome". Genetics Home Reference. Retrieved 2017-04-20.
- ^ "Immunodysregulation, polyendocrinopathy and enteropathy X-linked | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2017-04-20.
- ^ Weiler, Fernanda Guimarães; Dias-da-Silva, Magnus R.; Lazaretti-Castro, Marise (2012-02-01). "Autoimmune polyendocrine syndrome type 1: case report and review of literature". Arquivos Brasileiros de Endocrinologia & Metabologia. 56 (1): 54–66. doi:10.1590/S0004-27302012000100009. ISSN 0004-2730.
Further reading
- Improda, Nicola; Capalbo, Donatella; Cirillo, Emilia; Cerbone, Manuela; Esposito, Andrea; Pignata, Claudio; Salerno, Mariacarolina (1 November 2014). "Cutaneous vasculitis in patients with autoimmune polyendocrine syndrome type 1: report of a case and brief review of the literature". BMC pediatrics. 14: 272. doi:10.1186/1471-2431-14-272. ISSN 1471-2431. Retrieved 20 April 2017.
{{cite journal}}: CS1 maint: unflagged free DOI (link) - Shoenfeld, Yehuda; Cervera, Ricard; Gershwin, M. Eric (2008). Diagnostic Criteria in Autoimmune Diseases. Springer Science & Business Media. ISBN 9781603272858.
External links
This template is no longer used; please see Template:Endocrine pathology for a suitable replacement