Английская Википедия:Autoimmune polyendocrine syndrome

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Шаблон:Infobox medical condition (new) Autoimmune polyendocrine syndromes (APSs), also called polyglandular autoimmune syndromes (PGASs)[1] or polyendocrine autoimmune syndromes (PASs), 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, and there are a number of other diseases which involve endocrine autoimmunity.[3][4][5]

Types

Cause

Each "type" of this condition has a different genetic cause. IPEX syndrome is inherited in males by an X-linked recessive process. The FOXP3 gene, whose cytogenetic location is Xp11.23, is involved in the mechanism of the IPEX condition.[10][11]

Diagnosis

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CT scan

Diagnosis for type 1 of this condition for example, sees that the following methods/tests are available:[3]

Differential diagnosis

For this condition, differential diagnosis sees that the following should be considered:[12]

Management

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Ketoconazole

Immunosuppressive therapy may be used in type I of this condition.[13] Ketoconazole can also be used for type I under certain conditions.[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 Шаблон:Medical citation needed

See also

References

Шаблон:Reflist

Further reading

External links

Шаблон:Medical resources Шаблон:Scholia Шаблон:Disorders involving multiple endocrine glands Шаблон:Hypersensitivity and autoimmune diseases Шаблон:Authority control