Polyglandular endocrine emergency: lessons from a patient, which a book cannot teach

Area of Science:

• Endocrinology
• Cardiology
• Autoimmunology

Background:

• Polyglandular autoimmune syndrome type 2 (PAS-2) involves multiple endocrine gland dysfunctions.
• Patients with PAS-2 have an increased risk of severe metabolic and cardiovascular events.
• Management of co-occurring autoimmune conditions like Addison's disease and hypothyroidism is critical.

Observation:

• A 30-year-old woman with PAS-2 presented with cardiac arrest, hypothermia, hypotension, and bradycardia.
• Biochemical tests indicated acute kidney injury, metabolic acidosis, and possible sepsis.
• Elevated thyroid-stimulating hormone levels suggested a myxedema crisis, alongside Addisonian crisis.

Findings:

• The patient exhibited severe left ventricular impairment with apical ballooning, diagnosed as stress-induced cardiomyopathy.
• Treatment with intravenous liothyronine and hydrocortisone led to gradual recovery.
• Non-adherence to a gluten-free diet was identified as a potential trigger for the cardiac arrest.

Implications:

• This case highlights the critical cardiovascular risks associated with untreated or poorly managed autoimmune endocrine crises.
• It underscores the importance of comprehensive management of all autoimmune conditions in patients with PAS-2.
• Strict adherence to dietary management, such as a gluten-free diet for celiac disease, is vital for preventing severe complications.