SUN-439 - A Challenging Case of Post-Partum Diagnosis of Addison'S Disease

Background: Addison’s disease, a form of primary adrenal insufficiency (PAI), may initially present with nonspecific, subacute features that make initial diagnosis challenging. During pregnancy, symptoms such as nausea, vomiting, fatigue, and dizziness may overlap with those of pregnancy and delay diagnosis.

Case Report: A 33-year-old woman with dermatomyositis, Sjögren’s syndrome, hypothyroidism and recent post-partum hemorrhage from third-degree laceration requiring blood transfusion presented to the emergency department with shortness of breath and palpitations 10 days post-partum. Vital signs were notable for HR 131 bpm, BP 92/61 mmHg. She was anemic (Hgb 10.3 g/dl [11.5-15.5 g/dL]). Workup for peripartum cardiomyopathy and pulmonary embolism (transthoracic echo, pro-BNP and CTA chest) was negative. She reported poor lactation, fatigue and weakness, raising concern for Sheehan syndrome. She also endorsed a 40-pound weight loss during pregnancy. Laboratory evaluation was significant for Na 130 mmol/L (135-145 mmol/L), eosinophilia, and AM cortisol < 0.3 ug/dL (6.0-18.4 ug/dL). Remaining pituitary labs were consistent with a post-partum pattern. CT abdomen/pelvis showed normal adrenal morphology. Further testing revealed ACTH 1748 pg/mL (7.2-63.3 pg/mL), and positive thyroperoxidase antibody. Given her profoundly low cortisol and high ACTH, with autoimmune history, eosinophilia, and intact pituitary function, Addison’s disease was diagnosed. Of note, she had hyperpigmentation of the knuckles, ears and tongue (initially attributed to dermatomyositis) that was consistent with hyperpigmentation due to Addison’s. She was diagnosed with PAI and started on hydrocortisone and fludrocortisone with clinical improvement and discharged with outpatient follow-up. Post-discharge, 21-hydroxylase antibody was found to be positive.

Discussion: PAI often develops insidiously, with nonspecific symptoms that can mimic more common conditions or be dismissed as vague complaints. For instance, this patient presented twice to the emergency department earlier during pregnancy for varied complaints and was hyponatremic each time to as low as 122 mmol/L without receiving cortisol testing at that time. In a patient with fatigue, hypotension, hyponatremia, poor lactation, and low morning cortisol after post-partum hemorrhage, Sheehan syndrome, or post-partum ischemia of the pituitary gland with resultant variable hypopituitarism, is a reasonable suspected diagnosis. However, our patient’s markedly elevated ACTH, positive 21-hydroxylase antibodies and intact pituitary function confirmed the diagnosis of Addison's.

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