Autoimmune Polyendocrine Syndrome Type II with Empty Sella Syndrome

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 1-16-Adrenal Insufficiency
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-15
Alejandro G. Trepp Carrasco*1, Valentina D. Tarasova1, Sri Harsha Tella1 and J Christopher Gallagher2
1Creighton University, Omaha, NE, 2Creighton University Medical Cen, Omaha, NE
Introduction: Empty Sella and hypopituitarism can occur from autoimmune lymphocytic hypophysitis (ALH). Autoimmune Polyendocrine Syndrome (APS) type II may include primary hypoadrenalism (Addison’s disease), lymphocytic thyroiditis and ALH.

Clinical Case: A 63 year old white lady was admitted with syncope, severe hyponatremia, hyperkalemia and a psychotic state. She had recently undergone knee replacement. She had a 10 year history of hypothyroidism. Serum cortisol was 2.3 ug/dL and 30 mins post ACTH it increased to 6.8 ug/dL. Serum ACTH was not available. A diagnosis of Addison’s disease was made. Mineralocorticoid and glucocorticoid replacement was started. An MRI identified empty sella. A CT scan reported normal adrenals. At the follow up visit other tests were now available. 21-hydroxylase antibodies were 24 U/mL (nl. <1), thyroid peroxidase (TPO) antibodies 431 U/mL (nl. <34), LH 8.1 U/L (nl. 11-58), FSH 8.5 U/L (nl. 16-113), somatomedin C 73 ng/mL (nl. 41-168), TSH 0.1 U/mL (nl. 0.3-5.6), free T4 of 1.5 ng/dL (nl. 0.6–1.7) and normal prolactin.

Five months later she was admitted with a 3 day history of dyspnea, fatigue and diarrhea. She was tachycardic and hypotensive. Laboratories showed serum values of sodium 121 mEq/L, potassium 7.1 mEq/L, creatinine 3.9 mg/dL, AST 2214 U/L (nl. <30) and ALT of 1164 U/L (nl.<36). She had stopped her fludrocortisone because of weight gain and missed one dose of hydrocortisone. Echocardiogram showed an ejection fraction of 20 percent with apical akinesis. She recovered quickly after glucocorticoid and mineralocorticoid therapy for 3 days.

Conclusion: Although this illness was recent, lack of hyperpigmentation despite sun bathing suggests that ACTH was not chronically elevated. High TPO antibodies support primary hypothyroidism. However, 2-years old records showed low serum TSH despite low free T4. Finally, post-menopausal low gonadotropins further support hypopituitarism, presumably due to ALH and empty sella. Interestingly, she has classical features of primary adrenal insufficiency.
In the presence of autoimmune thyroid disease and positive adrenal antibodies, the diagnosis of APS type II is made. In these patients, yearly stimulation tests to identify overt adrenal insufficiency are warranted.

Our case illustrates the value of clarifying the source (e.g. primary vs. secondary) and nature (e.g. autoimmune vs. others) of each hormonal deficiency encountered, in order to timely provide the appropriate intervention.

Nothing to Disclose: AGT, VDT, SHT, JCG

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