Endocrine Fellow Metropolitan hospital Old bridge, New Jersey, United States
Introduction : Autoimmune Polyglandular Syndrome (APS) Type II ischaracterized by the presence of Addison’s Disease(AD)along with other autoimmune endocrinopathies such as autoimmune thyroid disease and/or Type 1 Diabetes Mellitus(T1DM). Patients may also have other endocrine glands affected and have concurrent primary hypogonadism(as in our patient), myasthenia gravis, pernicious anemia, or celiac disease. While ADis crucial to the diagnosis of APS II, it is not always the presenting illness. We present a case of polyautoimmunitywith the presentation of tachycardia and severe weight loss in uncontrolled hypothyroidism revealing itself asa life -threatening adrenal shock.
Case(s) Description : A 39-year-old female with medical historyofHashimoto’sthyroiditisfor 4 years and premature ovarian failurefor 3 yearspresentedto ED with complaints of dizziness, nausea, vomiting, and fatigue for last 4 months. She also noted a 40lb weight loss, despite being off levothyroxine. She was on no medications,although previously was taking levothyroxine but self-discontinued. Initial vital signsin ED were BP 86/54mmHg and HR 85bpmsupine, BP 105/38mmHg and HR 105bpm on sitting, RR of 18, O2 sat of 97 %. Physical exam showed an ill- appearing woman with dry mucous membranes and hyperpigmentation of the gums, perioral mucosa, and palmar creases. Lab work showed hyponatremia Na 117mmol/L,hyperkalemia K7.2mmol/L, BUN/Cr36/1.5 mg/dL, rhabdomyolysis CK 3491, random cortisol low at 0.6 ug/dL, and TSH 4.7. Record review showed she had been following with the infertility clinic for last few years with estradiol < 32, FSH 26 U/L, LH 29 U/L. Patientwas treated with aggressive hydration for rhabdomyolysis, medically managed for electrolyte disturbancesand started on stress dose steroids. Levothyroxine was resumed after Hydrocortisone was started.Patient was suspected to have primary adrenal insufficiencywith her presentation and confirmed with a positive ACTH stimulation test. CT adrenal glands did not show any adenomas or masses. After stabilization, patient was discharged on replacement doses of Levothyroxine and Hydrocortisone.
Discussion : APS Type II is a rare disorder and all presentations of the disease can present in a life-threatening matter – whether it is AD, T1DM or autoimmune thyroiditis.Fifty percent of patients with Addison’s disease will develop a second autoimmune disease during their lifetimeand the presentation may be decades apart. Our patient presented in full blown adrenal crisis with life threatening electrolyte disturbances, low blood pressure, and rhabdomyolysis. She also presented with manyfew clues that prompted the work-up for another autoimmune condition. Our patient above has been off Levothyroxine replacement for 8 months and despite that had lost 40 lbs during that span of time. Clinicians should be suspicious of APS II when the patient with known autoimmune conditions presents in an unusual manner.