Pregnancies associated with primary adrenal insufficiency.

OBJECTIVE

To provide a framework for the clinical presentation, evolution, treatment, and outcome of the unusual association between primary adrenal insufficiency (AI) during pregnancy and life-threatening complications for the mother and fetus.

DESIGN

Case reports.

SETTING

Pregnant women with AI treated in the Endocrine and Diabetes Department, Hospital Universitario de Maternidad y Neonatología, Córdoba, Argentina.

PATIENT(S): Three pregnant women with AI.

INTERVENTION(S): Review of hospital records.

MAIN OUTCOME MEASURE(S): Clinical, laboratory features, treatment, and outcome.

RESULT(S): Two women with AI were diagnosed before conception, and one was diagnosed during gestation. Two of the cases were associated with other autoimmune diseases. Two newborns were born with signs of fetal distress, and one passed away hours later. Poor outcome was related to low compliance with treatment.

CONCLUSION(S): AI is often overlooked during pregnancy because of its rarity and pregnancy-like symptoms. Nevertheless, other autoimmune diseases, hyponatremia, metabolic acidosis, nausea and vomiting, and orthostatic hypotension that does not improve with usual treatment or persists after first trimester should evoke a diagnosis of AI. If diagnosis and treatment are properly managed, pregnancy, labor, and delivery may occur without complications. If not, AI is associated with high maternal and fetal morbidity and mortality.