A Case of Isolated Adrenal Insufficiency with Empty Sella and Pulmonary Hypertension

Presentation Number: FRI 555
Date of Presentation: April 1st, 2016

Jagriti Upadhyay*, Devina Luhur Willard and Philip E Knapp
Boston Medical Center, Boston, MA


INTRODUCTION: Empty sella (ES) is a common entity, with an estimated prevalence of 5-25% in the general population. Current literature suggests that hypopituitarism may be present in up to 25% of cases of ES (1). While abnormalities of GH, FSH, LH and prolactin are commonly reported in ES, ACTH deficiency is less common and isolated ACTH deficiency is very rare. Primary pulmonary hypertension (PPH) is also a rare disease and to our knowledge only one case co-existing with isolated ACTH deficiency in a young patient has previously been reported.   

CASE REPORT: A 21 year old G2P1 woman presented at 17 weeks gestation when she was found unconscious. She had low blood glucose of 14mg/dL, hypotension, tachycardia and a low random cortisol level of 0.8ug/dL. Adrenal insufficiency (AI) was suspected and she was started on stress dose steroids. She reported 3 months of progressive fatigue and shortness of breath. Echocardiography revealed severe right ventricle dilatation with moderately depressed systolic function. A right heart catheterization was consistent with severe pulmonary hypertension. CTPA showed no evidence of PE. She stabilized, underwent medical termination of her pregnancy and was continued on steroids. After discharge she was scheduled to see an endocrinologist to evaluate her adrenal axis but did not follow up.

Three years later she had a similar presentation with shortness of breath, early pregnancy and severe pulmonary hypertension. She was apparently asymptomatic in between these admissions and had stopped taking steroids 3 weeks earlier. Upon admission, prior to receiving steroids she was found to have undetectable am cortisol (<1.0 ug/dL) and ACTH (<5pg/mL) levels. Aldosterone level was 30 ng/dL and renin activity was 12.65 ng/ml/hr ruling out primary AI and suggesting central AI. Evaluation of the other pituitary axes revealed normal serum LH, FSH, TSH, FT4, IGF-1 and prolactin. A pituitary MRI showed an empty sella without evidence of lymphocytic hypophysitis. She was discharged on physiologic steroid doses. To confirm her diagnosis a cosyntropin stimulation test was performed on 2 separate occasions. ACTH was undetectable and cortisol before and after stimulation was undetectable each time.

 CONCLUSION: While hypopituitarism is a known feature of ES, isolated central adrenal insufficiency is rarely observed. A prior case has been reported with the combination of isolated central AI, empty sella and PPH in which the patient had anti-phospholipid antibody syndrome as a unifying diagnosis (2). Our patient had negative ANA and anticardiolipin antibodies and had no other features suggestive of antiphospholipid syndrome. Isolated ACTH deficiency is often seen in lymphocytic hypophysitis but our patient did not have evidence of this on MRI. Empty sella has been described as a late manifestation of lymphocytic hypophysitis (3) which therefore remains on the differential for our patient.   


Nothing to Disclose: JU, DLW, PEK