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09-SEPTEMBER-2008 02:07:44 - polyendocrine syndrome Autoimmune polyendocrine syndrome Classification and external resources ICD-10 E31.0 OMIM 240300 269200 DiseasesDB 29212 29690 eMedicine med/1867 med/1868 In medicine, autoimmune polyendocrine syndromes are a heterogeneous group of rare diseases characterised by autoimmune activity against more than one endocrine organs, although non-endocrine organs can be affected. There are three autoimmune polyendocrine syndromes, and a number of other diseases which have endocrine autoimmunity as one of their features. Contents 1 The syndromes 1.1 Type 1 1.2 Type 2 1.3 XPID 2 Other diseases 3 Management 4 References 5 Sources The syndromes Type 1 Autoimmune polyendocrine syndrome, type 1 is also known as the candidiasis-hypoparathyroidism-Addison's disease-syndrome after its main features: A mild immune deficiency, leading to persistent mucosal and cutaneous infections with candida yeasts. There is also decreased function of the spleen asplenism. Autoimmune dysfunction of the parathyroid gland leading to hypocalcemia and the adrenal gland Addison's disease: hypoglycemia, hypotension and severe reactions in disease. Other disease associations are: hypothyroidism hypogonadism and infertility vitiligo depigmentation of the skin alopecia baldness malabsorption pernicious anemia chronic active autoimmune hepatitis As opposed to type 2, this syndrome inherits in an autosomal recessive fashion and is due to a defect in AIRE autoimmune regulator,1 a gene located on the 21st chromosome. Normal function of AIRE, a transcription factor, appears to be to confer immune tolerance for antigens from endocrine organs. A EU-funded consortium is currently doing translational research on this condition and has established a webpage at EurAPS. Type 2 Autoimmune polyendocrine syndrome, type 2 also known as Schmidt's syndrome2 is more heterogeneous, occurs more often and has not been linked to one gene. Rather, patients are at a higher risk when they carry a particular HLA genotype DQ2, DQ8 and DRB10404. Features of this syndrome are: Addison's disease3 hypothyroidism diabetes mellitus type 1 less common associations: hypogonadism vitiligo Some researchers favour splitting this syndrome into three distinct syndromes numbering 2, 34 and 4, but research evidence for these distinct combinations is not convincing. XPID The most serious but rarest form is the X-linked polyendocrinopathy, immunodeficiency and diarrhea-syndrome, also called IPEX.. This is due to mutation of the FOXP3 gene on the X chromosome.5 Most patients develop diabetes and diarrhea as neonates and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might suffer mild disease. Other diseases Other diseases featuring polyendocrine autoimmunity: Chromosomal abnormalities Down's syndrome increase the risk of endocrine autoimmunity POEMS syndrome - the E is for endocrinopathy; the cause is a paraprotein excreted by a plasmacytoma or multiple myeloma; other features are polyneuropathy, organomegaly hepatomegaly and splenomegaly, M-protein paraprotein and skin changes. Several very rare diseases. Management In principle, the component diseases are managed as usual. The challenge is to detect the possibility of any of the above syndromes, and to anticipate other manifestations. For example, in a patient with known Type 2 autoimmune polyendocrine syndrome but no features of Addison's disease, regular screening for antibodies against 21-hydroxylase a feature of Addison's may prompt early intervention and hydrocortisone replacement to prevent characteristic crises. References ^ Bensing S, Fetissov SO, Mulder J, et al January 2007. Pituitary autoantibodies in autoimmune polyendocrine syndrome type 1. Proc. Natl. Acad. Sci. U.S.A. 104 3: 949-54. doi:10.1073/pnas.0610070104. PMID 17215373. Retrieved on 2008-07-25. ^ Heuss D, Engelhardt A, Göbel H, Neundörfer B June 1995. Myopathological findings in interstitial myositis in type II polyendocrine autoimmune syndrome Schmidt's syndrome. Neurol. Res. 17 3: 233-7. PMID 7643982. Retrieved on 2008-07-25. ^ Betterle C, Zanchetta R April 2003. Update on autoimmune polyendocrine syndromes APS. Acta Biomed 74 1: 9-33. PMID 12817789. Retrieved on 2008-07-25. ^ de Carmo Silva R, Kater CE, Dib SA, et al February 2000. Autoantibodies against recombinant human steroidogenic enzymes 21-hydroxylase, side-chain cleavage and 17alpha-hydroxylase in Addison's disease and autoimmune polyendocrine syndrome type III. Eur. J. Endocrinol. 142 2: 187-94. PMID 10664529. Retrieved on 2008-07-25. ^ Yong PL, Russo P, Sullivan KE May 2008. Use of Sirolimus in IPEX and IPEX-Like Children. J. Clin. Immunol.. doi:10.1007/s10875-008-9196-1. PMID 18481161. Sources Eisenbarth GS, Gottlieb PA 2004. Autoimmune polyendocrine syndromes. N. Engl. J. Med. 350 20: 2068-79. doi:10.1056/NEJMra030158. PMID 15141045. v d e Endocrine pathology: endocrine diseases E00-35, 240-259 Pancreas/ glucose metabolism Diabetes mellitus types: type 1, type 2, MODY, complications: coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy Hypoglycemia - Hyperinsulinism - Zollinger-Ellison syndrome - insulin receptor Rabson-Mendenhall syndrome - Insulin resistance Hypothalamic/ pituitary axes Pituitary Hyperpituitarism Acromegaly, Hyperprolactinaemia, SIADH Hypopituitarism Sheehan's syndrome, Kallmann syndrome, Growth hormone deficiency, Diabetes insipidus Adiposogenital dystrophy - Empty sella syndrome - Pituitary apoplexy - ACTH deficiency Thyroid Hypothyroidism Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre, Myxedema Hyperthyroidism Graves disease, Toxic multinodular goitre, Teratoma with thyroid tissue or Struma ovarii Thyroiditis De Quervain's thyroiditis, Hashimoto's thyroiditis, Riedel's thyroiditis Euthyroid sick syndrome - Thyroid hormone resistance - Thyroid nodule Parathyroid Hypoparathyroidism Pseudohypoparathyroidism - Hyperparathyroidism Primary, Secondary, Tertiary Adrenal Adrenocortical hyperfunction: Cushing's syndrome Nelson's syndrome, Pseudo-Cushing's syndrome - Hyperaldosteronism Conn syndrome, Bartter syndrome CAH Lipoid, 3β, 11β, 17α, 21α Adrenal insufficiency Addison's disease, Waterhouse-Friderichsen syndrome - Hypoaldosteronism Gonads ovarian Polycystic ovary syndrome, Premature ovarian failure testicular 5-alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency general Hypogonadism, Delayed puberty, Precocious puberty Other Androgen insensitivity syndrome - Autoimmune polyendocrine syndrome - Carcinoid syndrome - Gigantism - Short stature Laron syndrome, Psychogenic dwarfism - Multiple endocrine neoplasia 1, 2 - Progeria - Woodhouse-Sakati syndrome - thymus Abscess of thymus, Thymus hyperplasia see also congenital, neoplasia Retrieved from http://en..org/wiki/Autoimmune_polyendocrine_syndrome Categories: Endocrinology | Autoimmune diseases Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch This page was last modified on 1 August 2008, at 22:09
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