Document Type

Conference Proceeding

Publication Date

5-1-2026

Abstract

Background: Nonclassic congenital adrenal hyperplasia (NCCAH) caused by 21-hydroxylase deficiency is generally a milder variant of congenital adrenal hyperplasia characterized by partial enzyme activity and variable increases in adrenal androgen levels. Patients presenting with such a condition may present in adolescence or adulthood with symptoms such as acne, hirsutism, irregular menses, and infertility. These symptoms often overlap with those of Polycystic Ovary Syndrome (PCOS), creating challenges in differentiation and management. It is critical to differentiate between PCOS and NCCAH in females of reproductive age, as hyperandrogenism treatment depends on the underlying cause, such as adrenal, ovarian, or a combination of both. For instance, although glucocorticoids may control hyperandrogenism caused by adrenal hyperplasia, they do not control hyperandrogenism caused by PCOS nor induce ovulation in females interested in pregnancy. In patients with NCCAH who desire fertility, the goal is to provide sufficient control of adrenal androgens while not suppressing ovulation. Additionally, failure to identify a secondary cause of hyperandrogenism from the ovary will lead to continued anovulation regardless of adequate NCCAH control, creating a management challenge requiring multidisciplinary expertise. Case Description: The patient is a 24-year-old female with a history of NCCAH and PCOS, both of which were diagnosed in her teenage years. She came in for fertility counseling because of irregular menses for many years. She has never experienced spontaneous menses unless on birth control pills. She has fluctuating levels of 17-hydroxyprogesterone that vary from less than 40 ng/dl to more than 2500 ng/dl. She also has high levels of testosterone and androstenedione. She has tried many therapeutic trials, including hydrocortisone, fludrocortisone, dexamethasone, birth control pills, and metformin. She has responded biochemically but has never ovulated. She has 20 follicles in the left ovary on pelvic ultrasound with normal ovarian volume. This indicates that there is a secondary ovarian source for her hyperandrogenism, consistent with PCOS. Even with good adrenal suppression on glucocorticoid therapy, she has never ovulated. Her cycles have always required progesterone supplementation. Metformin therapy was added for ovulation induction, but regular cycles have never returned. Discussion This case illustrates the challenges faced in fertility-directed management of NCCAH with the presence of secondary hyperandrogenism from PCOS. While the glucocorticoid therapy was effective in controlling adrenal hyperandrogenism, this patient's anovulatory status suggests the presence of a secondary source of hyperandrogenism from the ovary. This dual-source hyperandrogenism is important as it presents a therapeutic dilemma; higher glucocorticoid doses may control adrenal androgen excess but can negatively impact ovulation. Management required careful balancing of glucocorticoid dosing to suppress adrenal androgens without impairing ovulation, alongside PCOS-directed interventions. This case highlights the necessity to address adrenal and ovarian factors of hyperandrogenism in females with NCCAH complicated by PCOS pursuing pregnancy.

Comments

American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2026 Resident and Medical Student Day, May 1, 2026, Troy, MI

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