• Mohammad Reza Ghanbari Boroujeni, Elahe Meftah, Fatemeh Zarimeidani, Rahem Rahmati, and Fatemeh Esfahanian.
• Students Research Committee, Shahrekord University of Medical Sciences, Shahrekord, Iran.
• Medicine (Baltimore). 2024 Oct 11; 103 (41): e40050e40050.

RationalePrimary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing syndrome (CS), accounting for <2% of CS cases. Diagnosing PBMAH can be difficult and challenging for clinicians.Patient ConcernsWe report a 52-year-old female, a patient with a history of intermittent fever for 3 years. She presented with nausea, headache, and dizziness for several days, along with fatigue, myalgia, muscle weakness, exertional dyspnea, hoarseness, spontaneous bruising over the past several months, and long-term psychological complaints. Additionally, we observed periorbital and facial edema, right lower quadrant tenderness, and abdominal striae during the examination.DiagnosesHer laboratory results showed increased cortisol and suppressed ACTH, and an abdominal CT scan revealed 2 heterogeneous masses in the adrenal glands. These findings led us to the diagnosis of PBMAH in this patient. The existence of aberrant receptors was evaluated, and the tests were negative.InterventionsThe patient underwent left adrenalectomy and corticosteroid therapy after the surgery. Her clinical complaints improved after the surgery.OutcomesHowever, her dependency on corticosteroids was not transient after unilateral adrenalectomy, and she still needs glucocorticoid supplementation 1 year after surgery.LessonsThis patient is a case of PBMAH who presented with fever and CS symptoms and underwent unilateral adrenalectomy. Interestingly, she had suppressed cortisol levels for at least 1 year after the unilateral adrenalectomy. Therefore, we suggest further research on the most effective treatment strategies for PBMAH.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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