8277 Pregnancy-Induced Cushing Syndrome

Abstract Disclosure: S. Al Allawi: None. C. Okigbo: None. R. Osman: None. Introduction: Pregnancy is uncommon in women with Cushing syndrome (CS), as hypercortisolism typically leads to anovulatory infertility. CS during pregnancy can be challenging due to the physiological changes in the hypothalamic-pituitary-adrenal axis, overlapping features with pregnancy, and the potential for misdiagnosis as gestational diabetes or hypertension. Pregnancy-induced Cushing is rarely reported, and can sometimes completely remit postpartum, although some cases recur with subsequent pregnancies. Here, we describe a case of pregnancy-induced hypercortisolism with postpartum remission. Clinical Case: A 26-year-old primigravida with a reported history of PCOS, presented to the hospital at 25 weeks of gestation due to concerns for pre-eclampsia. Prior to her pregnancy, she had a history of oligomenorrhea and had an intrauterine device (IUD) in place until a month before conception. In the first trimester, she developed severe facial acne and excessive hirsutism. An abdominal MRI obtained due to abdominal pain, revealed an enlarged right ovary with a 4.2 cm dominant cyst and a slight asymmetric prominence of the left adrenal, indicating hyperplasia. At 18 weeks, she was diagnosed with pre-eclampsia and subsequently developed severe hypokalemia and muscle weakness. This prompted a workup for hypercortisolism which revealed elevated morning cortisol level of 55 mcg/dL, elevated 24-hour urinary cortisol 2,282 mcg/24 hr, suppressed ACTH < 5 pg/mL, elevated total testosterone 337 ng/dL and plasma renin activity (8.13 ng/mL/hr) with normal DHEAs and aldosterone levels. Repeat workup showed similar findings. A diagnosis of ACTH-independent Cushing syndrome was made. At 25 weeks, she was admitted for severe pre-eclampsia. Physical exam revealed cushingoid features with facial fullness, plethora, dorsocervical fat pads, generalized violaceous striae, and severe acne. A decision was made to initiate Metyrapone, but the patient quickly developed HELLP syndrome which led to premature delivery at 26 weeks, with a concomitant right ovarian cystectomy. Pathology revealed a benign serous cystadenoma. On Postpartum day 2, workup showed a drop in morning cortisol to 26 mcg/dL, ACTH 4.9 pg/mL, total testosterone 25 ng/dL. Three months postpartum, the patient had an improvement in the cushingoid features. Hormonal reassessment demonstrated morning cortisol of 9 mcg/dL, suppressed ACTH < 5 pg/mL, and total testosterone 47 ng/dL. Conclusion: Cushing's syndrome in pregnancy is rare, leading to a low suspicion among physicians and delayed diagnosis, posing risks for both mother and fetus. Over 260 cases of CS in pregnancy have been reported, with only 15 cases of pregnancy-induced Cushing's. Maintaining a high index of clinical suspicion can facilitate prompt diagnosis and early intervention, thereby minimizing maternal and fetal morbidity and improving pregnancy outcomes. Presentation: 6/3/2024