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IgG4-Related Hypophysitis: A Rare Cause of Sellar Mass Presenting with Symptoms Mimicking Pituitary Apoplexy and Adrenal Crisis

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IgG4-Related Hypophysitis: A Rare Cause of Sellar Mass Presenting with Symptoms Mimicking Pituitary Apoplexy and Adrenal Crisis

Kevin C.J. Yuen, MD, FRCP (UK)1, Brandon C Gabel, MD2, Kelley Moloney1, Jennifer U Mercado1, Steven Rostad, MD3 and Marc R Mayberg, MD2

(1) Swedish Pituitary Center, Swedish Neuroscience Institute, Seattle, WA,
(2) Department of Neurosurgery and Neurology, Swedish Neuroscience Institute, Seattle, WA,
(3) CellNetix, Seattle, WA

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IgG4-Related Hypophysitis: A Rare Cause of Sellar Mass Presenting with Symptoms Mimicking Pituitary Apoplexy and Adrenal Crisis

Kevin C.J. Yuen, MD, FRCP (UK)1, Brandon C Gabel, MD2, Kelley Moloney1, Jennifer U Mercado1, Steven Rostad, MD3 and Marc R Mayberg, MD2

(1) Swedish Pituitary Center, Swedish Neuroscience Institute, Seattle, WA,
(2) Department of Neurosurgery and Neurology, Swedish Neuroscience Institute, Seattle, WA,
(3) CellNetix, Seattle, WA

Background: IgG4-related hypophysitis is a rare clinical entity, and is part of an emerging group of multi-organ IgG4-related fibro-inflammatory diseases. It is more common in older men, and typically causes anterior hypopituitarism and/or diabetes insipidus (DI). Diagnosis is confirmed with pituitary biopsy of IgG4 cell infiltrates, sellar mass and/or thickened pituitary stalk, biopsy-proven involvement of other organ/s, serum IgG4 levels > 140 mg/dl, and/or sellar mass reduction and symptom improvement after glucocorticoid therapy.
Case: A 71 year old man with no previous medical history presented to the ER with sudden onset of headaches, diplopia, nausea and vomiting. Serum sodium was 120 mEg/L, and neurologic exam revealed a disconjugate gaze and oculomotor abnormalities of the left eye. MRI revealed a sellar mass (19 x 10 x 14 mm) abutting the optic chiasm. Laboratory work up confirmed secondary hypoadrenalism (baseline cortisol 3.5 mg/dL, 30-min post-ACTH stimulation cortisol 10.81 mg/dL), hypothyroidism (TSH 0.39 uIU/mL, free T4 0.6 ng/dL) and hypogonadism (FSH 0.9 mIU/mL, LH 1.2 mIU/mL and AM testosterone < 3.0 ng/dL), but normal serum IgG4 level of 40 mg/dL. He was rehydrated with IV fluids, and treated with IV followed by oral Hydrocortisone 20 mg/day and Levothyroxine 50 mg/day. In light of his MRI findings and visual symptoms, transsphenoidal adenomectomy was performed. At surgery, the pituitary gland was diffusely fibrotic without evidence of tumor or infarction. Final pathology revealed adenohypophysis with dense lymphoplasmacytic infiltrate contained >10 IgG4-positive plasma cells per high-power field. At 1 week follow-up, the patient reported resolution of headaches and visual symptoms, decreased libido and no symptoms of DI. Due to low AM testosterone levels, IM testosterone cypionate 140 mg Q2weeks was commenced. Work up for systemic IgG-4 related disease with a CT chest, abdomen, and pelvis was negative.
Discussion: This case report highlights the importance of considering IgG4-related hypophysitis in the differential diagnosis of sellar masses in older men. To our knowledge, no other cases of IgG4-related hypophysitis have been reported where the presenting symptom mimics that of pituitary apoplexy (sudden headaches and visual symptoms) with concurrent adrenal crisis. Notably also in our patient, the disease was pituitary-restricted, no DI, and normal serum IgG4 levels suggesting a diversity of presenting symptoms of this rare entity.

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