First manifestation of a polyglandular autoimmune syndrome as acute adrenal insufficiency in the 2nd trimester of pregnancy

Introduction Unspecific pregnancy-associated symptoms like nausea and emesis can potentially mask life-threatening underlying causes. In this report, we describe a case of acute adrenal insufficiency in a patient presenting with severe hyperemesis, hypotension and hyponatremia at 19 weeks of gestation.

Case A 30-year-old G2 / P1 with a history of celiac disease presented at 19 0 / 7 weeks with intractable vomiting, hyponatremia (119 mmol/l, ref. 135-145), hypotension (89/65 mmHg) and cachexia (BMI 16,3) to our clinic. Fetal ultrasound showed an unremarkable singleton pregnancy. She was previously treated as a severe case of hyperemesis gravidarum with TPN at a local community hospital and discharged home three days prior.

Blood cortisol levels at time of admission were decreased at 35.0 ng/ml. After administration of 250 µg cosyntropin there was no increase in cortisol levels, indicating acute adrenal crisis. Intravenous hydrocortisone therapy was immediately started after which her clinical condition improved rapidly. She was subsequently started on 40 mg oral hydrocortisone daily for the remainder of her pregnancy. MRI of the head revealed no intracranial masses. Anti-adrenal antibodies were highly positive (>1:1000), as well as antibodies against gastric parietal cells, suggesting an unspecific polyglandular autoimmune syndrome. The patient underwent a repeat cesarean section at term, delivering a healthy baby girl.

Discussion Adrenal insufficiency in pregnancy is a rare cause of severe morbidity if left untreated. The diagnosis can be difficult to establish due to pregnancy-induced metabolic and endocrine changes leading to similar symptoms. Increased vigilance and a high index of suspicion is recommended especially in more serious cases.

Interessenkonflikt

Es bestehen keine Interessenkonflikte

Publication History

Article published online:
07 October 2020