Baylor University Medical Center Proceedings October 2017 - 427

Adrenal crisis and autoimmune polyglandular syndromes
Zachary K. Freeland, MD, Richard Lueking, BS, Ginger Tsai-Nguyen, MD, Thoris Pan, MD, and Adan Mora Jr., MD

We report a 67-year-old woman who presented with adrenal crisis as
a manifestation of autoimmune polyglandular syndrome 2, a polygenic
disorder characterized by concurrent primary adrenal insufficiency and
either autoimmune thyroid disease or type 1 diabetes mellitus.

CASE REPORT
A 67-year-old woman with primary adrenal insuﬃciency
(AI), hypothyroidism, non-insulin-dependent diabetes
mellitus (DM), and dyslipidemia presented to our emergency
department with symptomatic hypotension and presyncope
associated with a 2-week history of postprandial nausea, vomiting, diarrhea, and abdominal cramping and a 20-pound
unintentional weight loss. Her initial diagnosis of AI was made
by a cosyntropin stimulation test during a hospital admission
for hypotension and volume depletion 6 years earlier. She
was discharged on hydrocortisone, and later ﬂudrocortisone
was added. Three months prior to the current presentation,
hydrocortisone was discontinued in favor of ﬂudrocortisone
monotherapy for unclear reasons. Subsequently, she struggled
with symptomatic hypotension, with her peak systolic blood
pressure rarely exceeding 100 mm Hg; this persisted despite
doubling of the ﬂudrocortisone dose from 0.1 to 0.2 mg/day.
Upon initial evaluation, the patient's blood pressure was
80/50 mm Hg and her heart rate was 60 beats/min. Initial
laboratory studies were significant for a serum potassium
level of 2.3 mmol/L (normal range, 3.5-5.1 mmol/L) and
magnesium of 1.3 mg/dL (normal range, 1.8-2.4 mg/dL); her
serum sodium, chloride, and bicarbonate levels were within
normal limits. Serum cortisol drawn at 4:00 am was 3.4 μg/dL
(normal range, 3.7-19.4 μg/dL). Computed tomography of
the abdomen revealed atrophied adrenal glands but no other
explanation for her gastrointestinal symptoms (Figure 1).
The patient was given 3 L of normal saline and 100 mg
of hydrocortisone intravenously, started on a norepinephrine
infusion, and admitted to the intensive care unit. Subsequent
management included continuation of hydrocortisone at a dose
of 100 mg every 8 hours, resumption of thyroid hormone replacement, aggressive potassium repletion, and rapid weaning of norepinephrine. Her diarrhea resolved and no source of infection was
identiﬁed. Hydrocortisone was tapered to an oral maintenance
Proc (Bayl Univ Med Cent) 2017;30(4):427-428

Figure 1. Computed tomography scan of the patient's abdomen showing atrophied
adrenal glands (arrows) consistent with autoimmune adrenal insufficiency.

dose of 20 mg every morning and 10 mg every afternoon. Upon
discharge, an endocrinology referral was made due to suspicion
for autoimmune polyglandular syndrome-2 (APS-2).
DISCUSSION
Adrenal crisis (AC) is deﬁned as an acute deterioration in a
patient with AI associated with absolute or relative hypotension
that improves following parenteral glucocorticoid administration (1). Other manifestations include fatigue, weakness,
fever, altered sensorium, anorexia, nausea, vomiting, secretory
diarrhea, abdominal pain, hyponatremia, hyperkalemia, metabolic acidosis, and rarely hypoglycemia (2). The combination
of abdominal pain and fever may lead to an incorrect diagnosis of acute abdomen and potentially catastrophic surgical
exploration (3).
From the Department of Internal Medicine (Freeland) and Division of Pulmonary
and Critical Care Medicine (Tsai-Nguyen, Pan, Mora), Baylor University Medical
Center at Dallas; and the Texas A&M Health Science Center College of Medicine
(Lueking).
Corresponding author: Adan Mora Jr., MD, Division of Pulmonary and Critical
Care Medicine, Baylor University Medical Center at Dallas, 3600 Gaston Avenue,
Wadley Tower Suite 960, Dallas, TX 75246 (e-mail: adam.mora@bswhealth.org).
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