View source for Autoimmune polyendocrine syndrome medical therapy

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{{Autoimmune polyendocrine syndrome}}
{{CMG}}; {{AE}}{{Akshun}}

==Overview==
Medical [[therapy]] for autoimmune polyendocrine syndrome (APS) depends upon the sub-type and the [[organ system]] involved. In APS, the focus is to treat the presenting [[Disorder (medicine)|disorder]] which can either be [[mucocutaneous]] [[candidiasis]], [[hypoparathyroidism]], [[adrenal insufficiency]] or [[autoimmune thyroiditis]]. The goal of treatment is to correct [[hormone]] deficiencies, prevent [[complications]], and reduce [[morbidity]]. Treatment includes monitoring of [[glandular]] functions for early detection of glandular failure, lifelong [[hormone replacement therapy]] for established [[glandular]] failure, and [[familial]] [[Screening (medicine)|screening]].

==Medical Therapy==
Medical [[therapy]] for autoimmune polyendocrine syndrome (APS) depends upon the sub-type and the [[organ system]] involved.
*In APS the focus is to treat the presenting [[Disorder (medicine)|disorder]] which can either be [[mucocutaneous]] [[candidiasis]], [[hypoparathyroidism]], [[adrenal insufficiency]] or [[autoimmune thyroiditis]].
*[[Patient]] [[education]] is an important part of treatment in APS. Patients with APS who present with [[mucocutaneous]] [[candidiasis]] may take upto 5-10 years to develop [[hypoparathyroidism]] and another 10 years to show manifestations of [[adrenal insufficiency]]. Therefore, [[patients]] with [[family history]] of APS or an early age onset of APS should be advised to undergo regular surveillance.

* Treatment of APS  includes:<ref name="pmid26938200">{{cite journal |vauthors=Bilezikian JP, Brandi ML, Cusano NE, Mannstadt M, Rejnmark L, Rizzoli R, Rubin MR, Winer KK, Liberman UA, Potts JT |title=Management of Hypoparathyroidism: Present and Future |journal=J. Clin. Endocrinol. Metab. |volume=101 |issue=6 |pages=2313–24 |year=2016 |pmid=26938200 |pmc=5393596 |doi=10.1210/jc.2015-3910 |url=}}</ref><ref name="pmid27810905">{{cite journal |vauthors=Gan EH, Pearce SH |title=MANAGEMENT OF ENDOCRINE DISEASE: Regenerative therapies in autoimmune Addison's disease |journal=Eur. J. Endocrinol. |volume=176 |issue=3 |pages=R123–R135 |year=2017 |pmid=27810905 |doi=10.1530/EJE-16-0581 |url=}}</ref><ref name="pmid25138826">{{cite journal |vauthors=Inder WJ, Meyer C, Hunt PJ |title=Management of hypertension and heart failure in patients with Addison's disease |journal=Clin. Endocrinol. (Oxf) |volume=82 |issue=6 |pages=789–92 |year=2015 |pmid=25138826 |doi=10.1111/cen.12592 |url=}}</ref><ref name="pmid24766944">{{cite journal |vauthors=Tucci V, Sokari T |title=The clinical manifestations, diagnosis, and treatment of adrenal emergencies |journal=Emerg. Med. Clin. North Am. |volume=32 |issue=2 |pages=465–84 |year=2014 |pmid=24766944 |doi=10.1016/j.emc.2014.01.006 |url=}}</ref><ref name="pmid24755997">{{cite journal |vauthors=Napier C, Pearce SH |title=Current and emerging therapies for Addison's disease |journal=Curr Opin Endocrinol Diabetes Obes |volume=21 |issue=3 |pages=147–53 |year=2014 |pmid=24755997 |doi=10.1097/MED.0000000000000067 |url=}}</ref><ref name="pmid24031090">{{cite journal |vauthors=Grossman A, Johannsson G, Quinkler M, Zelissen P |title=Therapy of endocrine disease: Perspectives on the management of adrenal insufficiency: clinical insights from across Europe |journal=Eur. J. Endocrinol. |volume=169 |issue=6 |pages=R165–75 |year=2013 |pmid=24031090 |pmc=3805018 |doi=10.1530/EJE-13-0450 |url=}}</ref><ref name="pmid23177474">{{cite journal |vauthors=Napier C, Pearce SH |title=Autoimmune Addison's disease |journal=Presse Med |volume=41 |issue=12 P 2 |pages=e626–35 |year=2012 |pmid=23177474 |doi=10.1016/j.lpm.2012.09.010 |url=}}</ref><ref name="pmid22907517">{{cite journal |vauthors=Quinkler M |title=[Addison's disease] |language=German |journal=Med Klin Intensivmed Notfmed |volume=107 |issue=6 |pages=454–9 |year=2012 |pmid=22907517 |doi=10.1007/s00063-012-0112-3 |url=}}</ref><ref name="pmid24434360">{{cite journal |vauthors=Caturegli P, De Remigis A, Rose NR |title=Hashimoto thyroiditis: clinical and diagnostic criteria |journal=Autoimmun Rev |volume=13 |issue=4-5 |pages=391–7 |year=2014 |pmid=24434360 |doi=10.1016/j.autrev.2014.01.007 |url=}}</ref><ref name="pmid25266247">{{cite journal |vauthors=Jonklaas J, Bianco AC, Bauer AJ, Burman KD, Cappola AR, Celi FS, Cooper DS, Kim BW, Peeters RP, Rosenthal MS, Sawka AM |title=Guidelines for the treatment of hypothyroidism: prepared by the american thyroid association task force on thyroid hormone replacement |journal=Thyroid |volume=24 |issue=12 |pages=1670–751 |year=2014 |pmid=25266247 |pmc=4267409 |doi=10.1089/thy.2014.0028 |url=}}</ref>
**'''Mucocutaneous candidiasis''': 
***Preferred regimen (1): [[Fluconazole]] 800 mg (12 mg/kg) on day 1, then 400 mg daily (6 mg/kg/day) for 14 days after first negative [[blood culture]] and resolution of [[Signs and Symptoms|signs/symptoms]]
***Alternative regimen (1): [[Ketoconazole]] 200-400 mg/day [[Oral|PO]] may increase to 400 mg once daily if response is insufficient. Continue until active [[fungal infection]] is resolved; some [[infections]] may require a treatment duration of up to 6 months.
**'''Hypoparathyroidism''': Conventional therapy for [[hypoparathyroidism]]:
*** Oral [[calcium]]: Preferred regimen (1): [[Calcium carbonate]] (40% [[elemental calcium]]) (better absorption with meals) 
***:* Alternative regimen (1): [[Calcium citrate]] (21% [[elemental calcium]]) (more effective in patients with [[achlorhydria]] and [[Proton pump inhibitor|proton pump inhibitors]]' use, worsening [[constipation]])
***[[Vitamin D]] supplementation
***:* Preferred regimen (1): [[Calcitriol]] 0.25 to 2 μg q24h (>.75 μg administered in divided doses)
***:* Preferred regimen (2): [[Cholecalciferol]] (parent vitamin D3)
***:* Preferred regimen (3): [[Ergocalciferol]] (parent vitamin D2)
***:* Alternative regimen (1): 1α-Hydroxyvitamin D ([[alfacalcidol]]) (used outside the United States)
***:* Alternative regimen (2): [[Dihydrotachysterol]] (used outside the United States)
***::Note(1): Serum [[calcium]] ([[Hypoparathyroidism laboratory findings|corrected for albumin]]), [[phosphorus]], and [[creatinine]] concentrations should be measured weekly to monthly during dose adjustments, and twice annually once a stable regimen has been reached. 
***::Note(2): 24 hour [[urinary]] [[calcium]] and [[creatinine]] should be considered during dose adjustments and should be measured twice annually on a stable regimen to evaluate for [[renal]] toxicity.
**'''Adrenal insufficiency (Addison's disease)''':
***[[Glucocorticosteroid]]: Preferred regimen (1): [[Cortisone]] 10 to 37.5 mg q12h [[Orally ingested|orally]] given in 2 divided [[doses]] with two-thirds of the total [[dose]] given in the morning  and one third in the afternoon 
***:*Preferred regimen (2): [[Hydrocortisone]] : 15-30 mg/day orally given in 2 divided [[doses]] with two-thirds of the total [[dose]] given in the morning and one third in the afternoon 
***:*Preferred regimen (3): [[Dexamethasone]] : 0.25 to 0.75 mg orally once daily 
***:*Preferred regimen (4): [[Prednisone]] : 2.5 to 5 mg orally once daily
***[[Mineralocorticoids|Mineralocorticosteroid]]: Preferred regimen (1): [[Fludrocortisone]] : 0.1 to 0.2 mg orally once daily
***Patients of [[Addison's disease]] with mild-to-moderate [[Stress (medicine)|stress]]:
***:*Alternative regimen (1): [[Cortisone]]  50-100 mg/day [[Orally ingested|orally]] given in 2 divided [[doses]] with two-thirds of the total [[dose]] given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) for 3 days
***:*Alternative regimen (2): [[Hydrocortisone]] 30-90 mg/day [[Orally ingested|orally]] given in 2 divided [[doses]] with two-thirds of the total [[dose]] given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) for 3 days
***:*Alternative regimen (3): [[Dexamethasone]] 0.50 to 2.25 mg [[Orally ingested|orally]] once daily for 3 days
***:*Alternative regimen (4): [[Prednisone]]  5-15 mg [[Orally ingested|orally]] once daily for 3 days
***Patients of [[Addison's disease]] with severe [[Stress (medicine)|stress]]:
***:*Alternative regimen (5): [[Hydrocortisone]] sodium succinate 100 mg [[intravenously]] every 6-8 hours
**'''Autoimmune thyroiditis''':
***:*Preferred regimen (1) [[Levothyroxine (oral)|Synthetic levothyroxine]] (L-[[T4]]) 1.6–1.8 μg/kg of body weight per day orally.

For complete [[therapy]] in [[hypopituitarism]] please [[Hypopituitarism medical therapy|click here.]]

For complete [[therapy]] in [[diabetes mellitus type 1]] please [[diabetes mellitus type 1 medical therapy|click here.]]

==References==
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