Over the following months, with continued corticosteroid replacement, her generalized symptoms of fatigue, weakness, and nausea improved, and her biochemical parameters gradually trended upward. By 8 months post presentation (September 2025), her morning serum cortisol had increased from its nadir of 0.03 µg/dL to 1.5 µg/dL (reference range 4.5-22.7 µg/dL); however, a repeat ACTH level remained suppressed at 5 pg/mL, confirming the persistent nature of her central adrenal insufficiency. Concurrently, her serum sodium levels had improved toward the normal range. Thyroid function remained stable on her home dose of levothyroxine (TSH 1.87 mIU/L). At the time of last follow-up, she remained clinically stable on hydrocortisone replacement and was scheduled for reevaluation of adrenal function in 3 months.
Discussion
This case illustrates several important considerations in the recognition and management of ICI-associated central adrenal insufficiency. The patient developed progressive fatigue, nausea, and generalized weakness shortly after initiating adjuvant pembrolizumab, ultimately presenting to the emergency department with hyponatremia and biochemical evidence of central adrenal insufficiency. This clinical presentation overlaps significantly with cancer-related fatigue and prior chemotherapy adverse effects, which can delay recognition of endocrine irAEs if adrenal insufficiency is not actively considered in the differential diagnosis.
The timing and pattern of toxicity in this case are noteworthy. Hypophysitis is classically associated with CTLA-4 inhibitors such as ipilimumab (Yervoy; Bristol Myers Squibb), with reported incidences up to 17% in some series, whereas PD-1 monotherapy (eg, pembrolizumab) carries a substantially lower overall risk, typically reported at less than 1%.5,10,11 Prior analyses report a median onset of adrenal insufficiency around 8 months, although onset varies widely across studies.1,6,12 By contrast, our patient developed central adrenal insufficiency after only 4 cycles (roughly 10 weeks) of pembrolizumab. Furthermore, although pharmacovigilance data frequently identify older males as the predominant demographic for this toxicity, likely reflecting the underlying prevalence of lung cancer and melanoma in studied populations, our patient was female.13 Additionally, she was receiving pembrolizumab monotherapy, which, like other PD-1 inhibitors, appears to confer a relatively low risk of adrenal insufficiency (approximately 1%) compared with CTLA-4–containing or combination ICI regimens.3,5,8,10-12 This case therefore underscores that ICI-related adrenal insufficiency can present early and should remain on the differential regardless of patient demographics or perceived lower-risk monotherapy regimens.
The diagnostic evaluation in this case illustrates a structured approach to distinguishing central from primary adrenal insufficiency and excluding alternative etiologies. The patient’s hyponatremia was attributed to a combination of reduced oral intake, a common confounder in oncology patients, and cortisol deficiency, which increases antidiuretic hormone secretion and impairs free-water excretion. However, the hormonal profile provided the definitive distinction: the combination of a profoundly reduced morning cortisol (0.03 µg/dL) with a suppressed ACTH (< 5 pg/mL) was diagnostic for a central etiology. Given the unequivocally low morning cortisol and suppressed ACTH, confirmatory testing with cosyntropin stimulation was not required to establish the diagnosis.
Clinical evaluation did not suggest mineralocorticoid deficiency. The presence of normokalemia (potassium 4.2 mmol/L) and the absence of hypotension on presentation strongly argued against a primary etiology. This preservation of mineralocorticoid function is a key feature distinguishing central from primary adrenal insufficiency, which classically presents with hyperkalemia and hemodynamic instability. Importantly, although the absence of hypotension helped distinguish central from primary adrenal insufficiency, it should not dissuade clinicians from considering the diagnosis, as life-threatening adrenal insufficiency can present without hemodynamic collapse. Finally, a negative adrenal PET scan supported the absence of adrenal metastases, further narrowing the differential to a central etiology.
Although a pituitary MRI was not performed because it was not expected to alter management in the absence of mass effect symptoms, the constellation of findings (including low cortisol, suppressed ACTH, and negative adrenal imaging) strongly supported a diagnosis of ICI-induced hypophysitis. Although hypophysitis often affects multiple pituitary axes, the preservation of thyroid function in this case suggests an isolated corticotroph deficiency. This specific pattern of isolated ACTH failure is a well-documented presentation of PD-1 inhibitor toxicity, contrasting with the pan-hypopituitarism more commonly seen with CTLA-4 inhibitors.11,14 Accordingly, the biochemical profile alone was sufficient to support pembrolizumab as the most likely etiology.
Management in this case is consistent with contemporary guideline-based recommendations for ICI-related adrenal insufficiency, which emphasize prompt glucocorticoid administration, fluid resuscitation, transition to physiologic hydrocortisone replacement, and patient education on stress dosing and emergency injections.7-9 Dexamethasone is often preferred when diagnostic testing is needed because it minimally interferes with cortisol assays. Our patient received intravenous dexamethasone and normal saline in the emergency department, followed by a taper to physiologic glucocorticoid replacement. She was ultimately transitioned to hydrocortisone 10 mg twice daily and prescribed a hydrocortisone intramuscular emergency kit, consistent with guideline recommendations for long-term management of central adrenal insufficiency and prevention of adrenal crisis.7-9 Because this was central adrenal insufficiency, mineralocorticoid replacement was not required.
Despite discontinuation of pembrolizumab, the patient's morning cortisol remained subnormal at follow-up (1.5 µg/dL), with persistently suppressed ACTH (5 pg/mL), confirming the persistent nature of her central adrenal insufficiency. This finding aligns with prior reports suggesting that ICI-induced central adrenal insufficiency is often permanent, and long-term or lifelong glucocorticoid replacement is typically required.15 Accordingly, ongoing education regarding stress dosing and sick-day rules remains essential. Pharmacists are well positioned to lead this effort by providing comprehensive counseling on dose adjustments during acute illness and ensuring patients can confidently demonstrate the technique for administering their emergency hydrocortisone injection. Beyond education, pharmacists play a crucial role in performing medication reconciliation to screen for drug interactions with glucocorticoids (eg, CYP3A4 inducers that may accelerate steroid metabolism) and in facilitating access to these life-sustaining therapies.
Taken together, this case highlights several important lessons for clinicians. First, even nonspecific symptoms such as fatigue, anorexia, and nausea in patients receiving ICIs should prompt consideration of endocrine irAEs, particularly when accompanied by hyponatremia or other electrolyte abnormalities. Second, early, structured evaluation with morning cortisol and ACTH is critical to differentiate central from primary adrenal insufficiency, whereas thyroid function tests and appropriate imaging help exclude alternative etiologies such as adrenal metastases or hypothyroidism. Third, timely initiation of glucocorticoid replacement and adherence to guideline-based management strategies can prevent progression to adrenal crisis and improve patient outcomes. As the use of ICIs continues to expand, increased awareness of ICI-associated adrenal insufficiency among oncologists, pharmacists, and the broader oncology care team is essential to ensure early detection and optimal management of this potentially life-threatening complication.
Conclusion
ICI-induced central adrenal insufficiency is an uncommon but clinically significant complication that may present with subtle constitutional symptoms often mistaken for cancer- or treatment-related sequelae. This case reinforces the need for clinicians to maintain a high index of suspicion, pursue a structured diagnostic workup (including timely evaluation of cortisol, ACTH, and electrolyte abnormalities), and promptly initiate guideline-directed glucocorticoid replacement to prevent adrenal crisis.
Continued vigilance, interdisciplinary communication, and patient education are essential to ensuring safe and effective care for individuals receiving these widely used immune-modulating agents. The early onset observed during adjuvant PD-1 monotherapy highlights the necessity of rapid recognition even when the patient appears clinically stable. As medication experts, pharmacists are central to this mission, driving patient education on sick-day management and emergency hydrocortisone use, while ensuring safe and effective long-term monitoring.
REFERENCES
Husebye ES, Castinetti F, Criseno S, et al. Endocrine-related adverse conditions in patients receiving immune checkpoint inhibition: an ESE clinical practice guideline. Eur J Endocrinol. 2022;187(6):G1-G21. doi:10.1530/EJE-22-0689
Sharma P, Goswami S, Raychaudhuri D, et al. Immune checkpoint therapy–current perspectives and future directions. Cell. 2023;186(8):1652-1669. doi:10.1016/j.cell.2023.03.006
O'Brien M, Paz-Ares L, Marreaud S, et al; EORTC-1416-LCG/ETOP 8-15 – PEARLS/KEYNOTE-091 Investigators. Pembrolizumab versus placebo as adjuvant therapy for completely resected stage IB-IIIA non-small-cell lung cancer (PEARLS/KEYNOTE-091): an interim analysis of a randomised, triple-blind, phase 3 trial. Lancet Oncol. 2022;23(10):1274-1286. doi:10.1016/S1470-2045(22)00518-6
Barroso-Sousa R, Barry WT, Garrido-Castro AC, et al. Incidence of endocrine dysfunction following the use of different immune checkpoint inhibitor regimens: a systematic review and meta-analysis. JAMA Oncol. 2018;4(2):173-182. doi:10.1001/jamaoncol.2017.3064
Kurokawa K, Mitsuishi Y, Shimada N, et al. Clinical characteristics of adrenal insufficiency induced by pembrolizumab in non-small-cell lung cancer. Thorac Cancer. 2023;14(5):442-449. doi:10.1111/1759-7714.14761
NCCN. Clinical Practice Guidelines in Oncology. Management of immune checkpoint inhibitor-related toxicities, version 1.2026. Accessed November 11, 2025. https://www.nccn.org/professionals/physician_gls/pdf/ici_tox.pdf
Schneider BJ, Naidoo J, Santomasso BD, et al. Management of immune-related adverse events in patients treated with immune checkpoint inhibitor therapy: ASCO guideline update. J Clin Oncol. 2021;39(36):4073-4126. doi:10.1200/JCO.21.01440
Higham CE, Olsson-Brown A, Carroll P, et al; Society for Endocrinology Clinical Committee. Society for Endocrinology endocrine emergency guidance: acute management of the endocrine complications of checkpoint inhibitor therapy. Endocr Connect. 2018;7(7):G1-G7. doi:10.1530/EC-18-0068
Faje A, Reynolds K, Zubiri L, et al. Hypophysitis secondary to nivolumab and pembrolizumab is a clinical entity distinct from ipilimumab-associated hypophysitis. Eur J Endocrinol. 2019;181(3):211-219. doi:10.1530/EJE-19-0238
Yuen KCJ, Samson SL, Bancos I, et al. American Association of Clinical Endocrinology disease state clinical review: evaluation and management of immune checkpoint inhibitor-mediated endocrinopathies: a practical case-based clinical approach. Endocr Pract. 2022;28(7):719-731. doi:10.1016/j.eprac.2022.04.010
Johnson J, Goldner W, Abdallah D, Qiu F, Ganti AK, Kotwal A. Hypophysitis and secondary adrenal insufficiency from immune checkpoint inhibitors: diagnostic challenges and link with survival. J Natl Compr Canc Netw. 2023;21(3):281-287. doi:10.6004/jnccn.2022.7098
Bai X, Lin X, Zheng K, et al. Mapping endocrine toxicity spectrum of immune checkpoint inhibitors: a disproportionality analysis using the WHO adverse drug reaction database, VigiBase. Endocrine. 2020;69(3):670-681. doi:10.1007/s12020-020-02355-9
Doodnauth AV, Klar M, Mulatu YS, Malik ZR, Patel KH, McFarlane SI. Pembrolizumab-induced hypophysitis with isolated adrenocorticotropic hormone (ACTH) deficiency: a rare immune-mediated adverse event. Cureus. 2021;13(6):e15465. doi:10.7759/cureus.15465
Nguyen H, Shah K, Waguespack SG, et al. Immune checkpoint inhibitor related hypophysitis: diagnostic criteria and recovery patterns. Endocr Relat Cancer. 2021;28(7):419-431. doi:10.1530/ERC-20-0513