MULTI-SYSTEM SARCOIDOSIS CAUSING PANHYPOPITUITARISM: RAPID IMPROVEMENT WITH CORTICOSTEROID THERAPY Rashid Mahboob, MD; Ali A.

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1 ENDOCRINE PRACTICE Rapid Electronic Article in Press Rapid Electronic Articles in Press are preprinted manuscripts that have been reviewed and accepted for publication, but have yet to be edited, typeset and finalized. This version of the manuscript will be replaced with the final, published version after it has been published in the print edition of the journal. The final, published version may differ from this proof. Visual Vignette EP MULTI-SYSTEM SARCOIDOSIS CAUSING PANHYPOPITUITARISM: RAPID IMPROVEMENT WITH CORTICOSTEROID THERAPY Rashid Mahboob, MD; Ali A. Rizvi, MD Running Title: Sarcoidosis and Panhypopituitarism From: Division of Endocrinology, Department of Internal Medicine, University of South Carolina School of Medicine Corresponding address: Ali A. Rizvi, MD Two Medical Park, Suite 502 Columbia, SC

2 Case Presentation: A 54-year-old male with a history of hypertension and type 2 diabetes was admitted to the neurosurgical service with abrupt onset of altered mental status and gait and speech difficulties. He was found confused and lying on the kitchen floor at home. Physical examination revealed a pleasant but disoriented person with pulse rate of 74 beats per minute and blood pressure of 94/64 mm Hg. There was no facial asymmetry, lymphadenopathy, thyromegaly, or tremor. The neurologic examination was otherwise unremarkable with no focal deficits. Liver function tests were mildly elevated, serum creatinine 1.1 mg/dl, hemoglobin level 12.1 g/l, and WBC count was 1.5 K/UL. Chest X-Ray revealed extensive bilateral mediastinal and hilar lymphadenopathy (Fig. 1, left panel). Magnetic resonance imaging of the brain showed the snow burst appearance of a large thalamic/sellar mass (Fig. 1, arrow in right panel) and multiple, scattered areas of extensive infiltrative and poorly defined abnormalities with hyperintense signals on T2 images. TSH was MIU/mL, free T ng/dl ( ), random serum cortisol <0.1 mg/dl, FSH 0.41 MIU/mL, LH <0.09 MIU/mL, total testosterone <4.33 ng/dl, and prolactin 3.7 ng/ml. ESR was 63 mm/hour, and angiotensin-converting enzyme level was 81 U/L (9-67). Cerebrospinal fluid analysis showed markedly elevated protein (236.9 mg/dl), reduced glucose level (39 mg/dl), and normal flow cytometry. Abdominopelvic lymphadenopathy was noted on computed tomography. Endobronchial ultrasound with lung biopsy and mediastinoscopy with lymph node biopsy were negative for malignancy or infection. What is the diagnosis? Answer: Extensive systemic sarcoidosis with prominent brain involvement. The lung biopsy was reported as fibrotic lung tissue with scattered noncaseating granulomas. High-dose corticosteroid therapy (dexamethasone 6 mg intravenously every 6 hours) led to a rapid and nearcomplete resolution of the right hilar, paratracheal, and subcarinal adenopathy and bilateral

3 pulmonary opacities upon repeat imaging one week later (Fig 2, left panel). There was also improvement in brain MRI appearance with significant resolution of the dominant hypothalamic/pituitary lesion (Fig 2, left panel). The rapid response suggested the presence of inflammatory edema that improved with steroid treatment and led to the dramatic radiographic changes. The patient had a favorable and noteworthy clinical improvement. Multisystem sarcoidosis can mimic a variety of processes including intracranial mass, lymphoma, metastatic disease, infiltrative conditions, and infection (1). This case serves as a reminder that neurosarcoidosis can lead to panhypopituitarism and the radiological changes may closely resemble a hypothalamic lesion or pituitary mass (2). The latter may resolve considerably with steroid administration, although recovery of pituitary function is variable (3). Diabetes insipidus (DI) with polyuria and polydipsia can be present at diagnosis or manifest itself after glucocorticoid replacement. However, DI was not evident in our case. Accurate diagnosis depends upon careful consideration of the clinical presentation and laboratory and radiological studies, satisfactorily ruling out masqueraders, and the presence of tissue findings consistent with sarcoidosis. References: 1. Vardhanabhuti V, Pang CL, Ninan T, Adams WM, Raju V, Suresh P. Sarcoidosis-- the greatest mimic. Semin Ultrasound CT MR. 2014;35: Bakshi R, Fenstermaker RA, Bates VE, Ravichandran TP, Goodloe S Jr, Kinkel WR. Neurosarcoidosis presenting as a large suprasellar mass. Magnetic resonance imaging findings. Clin Imaging. 1998;22:

4 3. Tedford M, Horine L, Burns ML. The resolution of pituitary mass in neurosarcoidosis. J Ark Med Soc. 2012;108:

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