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Acute central hypothyroidism, diabetes insipidus and central hypogonadism in a 36‐year‐old woman

The patient is a 36-year-old woman who presented with a new history of double vision for 1 week and sharp pain in the left eye and bridge of nose after 6 months of progressively worsening headaches. Nine and a half years before, she presented with intractable sinus symptoms and a strong suspicion of granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis). During her initial workup, she was noted to have positive proteinase 3 – anti-neutrophil cytoplasmic antibodies (PR3-cANCA) and conductive/ sensorineural hearing loss. Bilateral nasal biopsies demonstrated acute necrotizing vasculitis associated with inflammatory giant cells and a marked chronic active inflammation. She was diagnosed with organ-limited Wegener’s granulomatosis (GPA); prednisone and methotrexate were started. Over the next 3 years, she developed recurrent flares in the sinuses and nose, whenever the dose of prednisone was tapered. During a workup for headaches and eye pain the year before this most recent episode, she was noted to have mild left sixth cranial nerve weakness.A CT scan and MRI study showed no acute abnormality. At the time of her most recent presentation, MRI imaging of the sella showed a 2.3 × 1.9 × 1.4 cm mass that was reported to be consistent with a pituitary macroadenoma with mass effect on the optic chiasm (Fig. 1). Pituitary function tests were notable for an adrenocorticotropic hormone (ACTH) level of 5 pg/mL (8–42 pg/ mL), prolactin of 7.8 ng/mL (2.0–17.4 ng/mL), and thyroid stimulating hormone (TSH) of 0.070 μU/mL (0.400– 5.500 μU/mL). Abnormal thyroid function tests included serum thyroxine (T4) of 2.7 μg/dL (5.0–11.0 μg/dL), and Free T4 of 0.3 ng/dL (0.7–1.8 ng/dL). Endocrine workup revealed central hypothyroidism, diabetes insipidus and central hypogonadism. Postoperatively, the patient’s vision nearly returned to normal and her headaches greatly improved on daily prednisone.