autoimmune polyglandular syndrome ( aps ) is a rare genetic disease that causes multiple endocrine disorders .
the vague presentation of endocrine dysfunction can cause a delay in both the diagnosis and the treatment of life - threatening conditions .
we , herein , present a case of aps in a 21-year - old man whose condition did not respond to initial treatment and in whom a definitive diagnosis was subsequently achieved by workup for hypocortisolism .
a 21-year - old man with a history of hashimoto 's thyroiditis with hypothyroidism presented to the emergency department for evaluation of an altered mental status .
he was very lethargic , stared blankly , and was unable to respond appropriately to questions .
he reported no fever , difficulty swallowing , shortness of breath , muscle or joint pain , headache , vomiting , or excessive thirst or urination .
physical examination revealed a temperature ( t ) 34.3c , heart rate ( hr ) 68/min , respiratory rate ( rr ) 16/min , and blood pressure ( bp ) 87/57 mmhg .
he was very lethargic , confused , and able to answer only yes - or - no questions .
the patient exhibited hyponatremia ( 128 meq / l ) , hypoglycemia ( 29 mg / dl ) , and a high thyroid - stimulating hormone ( tsh ) level ( 14.31 iu / ml ) .
test for other metabolic panel , complete blood count , triiodothyronine and thyroxine levels , head computed tomography , and urine toxicology screening results were all normal .
he was diagnosed with severe hypothyroidism and received 2 l of fluid for resuscitation , 25 g of dextrose , and a single intravenous ( iv ) dose of 100 mcg levothyroxine at the emergency department .
a random serum cortisol level was < 1.0 g / dl . a 250 g adrenocorticotropic hormone ( atch ) stimulation test was < 0.1 g / dl . he was diagnosed with primary adrenal insufficiency and received a treatment with iv hydrocortisone .
his mental status , generalized fatigue , and metabolic parameters including glucose and sodium levels substantially improved .
aps type 2 ( aps2 ) was suspected based on his history of hypothyroidism and primary adrenal insufficiency .
the results of an additional liver function test , anti - gad65 antibody titer , testosterone level , and plasma renin activity were all within normal limits .
the patient had returned to his normal level of activity by the time of the outpatient follow - up and reported feeling much better after treatment with hydrocortisone , fludrocortisone , and levothyroxine .
the present case illustrates an example of aps2 , the clinical presentation of which may overlap that of primary adrenal insufficiency and hypothyroidism due to autoimmune thyroiditis .
the present patient exhibited lethargy , an altered mental status , hypothermia , hypotension , hypoglycemia , an elevated tsh level , and normal thyroxine levels .
he was later found to have primary adrenal insufficiency and his clinical symptoms improved after receiving corticosteroid replacement therapy .
other autoimmune diseases , especially of the skin , stomach , and gonads , may occur with increased frequency in patients with aps2 .
the greatest danger associated with aps2 is treatment of the presenting hypothyroid state without recognition of concomitant hypoadrenalism .
hypothyroidism reduces cortisol clearance , and the addition of thyroid hormone replacement increases cortisol clearance .
additionally , hypothyroidism reduces the metabolic rate , thereby , reducing the need for cortisol . the increased metabolic rate accompanying thyroxine replacement increases the requirement for cortisol .
furthermore , a high serum concentration of tsh in the absence of primary thyroid failure can be a feature of adrenal insufficiency .
the development of an adrenal crisis due to underlying adrenal insufficiency has been reported after the administration of thyroxine replacement therapy to patients initially thought to have only hypothyroidism .
the management of aps2 is based on individualized , lifelong replacement therapy for the affected endocrine organs plus monitoring for development of insufficiency in other organs or associated conditions .
the use of medical alert sensors and other measures should be taken to ensure that adrenal function is considered during illness , surgery , or emergency situations .
in summary , we have reported a case involving a patient with aps2 who presented with lethargy , hypotension , hypoglycemia , and an elevated tsh level .
the vague presentation of this syndrome can cause missed or delayed treatment for adrenal insufficiency resulting in a life - threatening adrenal crisis .
 context : 
 autoimmune polyglandular syndrome is a rare condition that causes a variety of clinical symptoms due to autoimmune processes involving multiple endocrine organs . its vague presentation can cause missed or delayed treatment for adrenal insufficiency , resulting in a life - threatening adrenal crisis.case report : a 21-yr - old man presented with lethargy , hypotension , hyponatremia , hypoglycemia , and an elevated thyroid - stimulating hormone level . 
 he was binge drinking the day before presentation . 
 no significant response to initial treatment with levothyroxine and dextrose occurred . 
 diagnostic workup later revealed primary adrenal insufficiency . 
 all initial symptoms completely resolved following treatment with hydrocortisone , fludrocortisone , and levothyroxine.conclusion:autoimmune polyglandular syndrome causes dysfunction of multiple endocrine organs such as the thyroid gland , adrenal gland , and pancreas . initial diagnosis of aps is crucial and difficult because of its vague , acute presentation , which often involves hypothyroidism and adrenal insufficiency . 
 delayed treatment of adrenal insufficiency can result in a life - threatening adrenal crisis . a diagnostic workup for adrenal insufficiency 
 should be performed in patients who do not respond to hypothyroidism treatment . 