Autoimmune polyendocrine syndromes denote a heterogeneous group
of rare diseases. The syndrome is featured by autoimmune activity
against more than one endocrine organ, as non-endocrine organs might be
affected.
As it is, there are three conditions, which can be classified under autoimmune polyendocrine syndromes, along with a number of other diseases, which may be characterized by endocrine autoimmunity. The following are the three conditions:
1.Type 1
Autoimmune polyendocrine syndrome, type 1 is characterized by features like mild immune deficiency, resulting in persistent mucosal, as well as cutaneous infections with candida yeasts, along with autoimmune dysfunction of the parathyroid gland, which can cause hypocalcemia. Other features include a reduced functionality of the spleen, dysfunction of the adrenal gland, causing hypotension, hypoglycemia, etc. Other diseases, associated with this condition include Hypothyroidism, hypogonadism and infertility, depigmentation of the skin, baldness, malabsorption, pernicious anemia, as well as chronic active autoimmune hepatitis.
2.Type 2
Autoimmune polyendocrine syndrome, type 2 is by far, one of the most common forms of polyglandular failure syndromes. It has been found to be more heterogeneous and as a matter of fact, it has not been linked to any one particular gene. Instead, patients tend to carry a higher risk, in case they carry a specific HLA genotype. As it is, autoimmune polyendocrine syndrome Type 2 tends to affect women more than men. Some of the major characteristics of this syndrome include Addison's disease, hypothyroidism, diabetes mellitus, hypogonadism, as well as vitiligo.
3.XPID
The XPID or the X-linked Polyendocrinopathy Immunodeficiency and Diarrhea-syndrome, is by far the rarest, as well as the most threatening form of polyendocrine autoimmunity. It is also known as IPEX. A major characteristic of this syndrome is the mutation of the FOXP3 gene, with the X chromosome. The most common diseases or medical conditions associated with it include diabetes and diarrhea. This may happen as neonates, as many die as a result of autoimmune activity against several organs. As it is, men tend to be affected, while girls remain as carriers and may suffer with a mild disease.
As it is, the component diseases are managed in the same way, as usually done. The major aspect is to find the possibility of any of the above syndromes, as well as for the anticipation of other manifestations.
So, a patient suffering from Type 2 autoimmune polyendocrine syndrome, without any characteristics of Addison's disease, would be diagnosed with the help of regular screening for antibodies, as against 21-hydroxylase. This might result in early intervention, as well as hydrocortisone replacement for prevention of characteristic crises.
As it is, there are three conditions, which can be classified under autoimmune polyendocrine syndromes, along with a number of other diseases, which may be characterized by endocrine autoimmunity. The following are the three conditions:
1.Type 1
Autoimmune polyendocrine syndrome, type 1 is characterized by features like mild immune deficiency, resulting in persistent mucosal, as well as cutaneous infections with candida yeasts, along with autoimmune dysfunction of the parathyroid gland, which can cause hypocalcemia. Other features include a reduced functionality of the spleen, dysfunction of the adrenal gland, causing hypotension, hypoglycemia, etc. Other diseases, associated with this condition include Hypothyroidism, hypogonadism and infertility, depigmentation of the skin, baldness, malabsorption, pernicious anemia, as well as chronic active autoimmune hepatitis.
2.Type 2
Autoimmune polyendocrine syndrome, type 2 is by far, one of the most common forms of polyglandular failure syndromes. It has been found to be more heterogeneous and as a matter of fact, it has not been linked to any one particular gene. Instead, patients tend to carry a higher risk, in case they carry a specific HLA genotype. As it is, autoimmune polyendocrine syndrome Type 2 tends to affect women more than men. Some of the major characteristics of this syndrome include Addison's disease, hypothyroidism, diabetes mellitus, hypogonadism, as well as vitiligo.
3.XPID
The XPID or the X-linked Polyendocrinopathy Immunodeficiency and Diarrhea-syndrome, is by far the rarest, as well as the most threatening form of polyendocrine autoimmunity. It is also known as IPEX. A major characteristic of this syndrome is the mutation of the FOXP3 gene, with the X chromosome. The most common diseases or medical conditions associated with it include diabetes and diarrhea. This may happen as neonates, as many die as a result of autoimmune activity against several organs. As it is, men tend to be affected, while girls remain as carriers and may suffer with a mild disease.
As it is, the component diseases are managed in the same way, as usually done. The major aspect is to find the possibility of any of the above syndromes, as well as for the anticipation of other manifestations.
So, a patient suffering from Type 2 autoimmune polyendocrine syndrome, without any characteristics of Addison's disease, would be diagnosed with the help of regular screening for antibodies, as against 21-hydroxylase. This might result in early intervention, as well as hydrocortisone replacement for prevention of characteristic crises.
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