SUN-429 Pituitary Sarcoidosis: A Rare Cause of Secondary Amenorrhea

Introduction Sarcoidosis is a granulomatous disease that can affect multiple organ systems including the central nervous system in 5-15% of patients, yet it is a rare cause of intrasellar lesions (<1%). Its presence in the pituitary-hypothalamic region may be the first manifestation of the disease and result in various degrees of pituitary dysfunction, ranging from selective deficiency to panhypopituitarism. Herein, we present a case of pituitary neurosarcoidosis in a woman with hyperprolactinemia and amenorrhea. Case Presentation A 32-year-old African American woman with a past medical history of uterine fibroids and hypertension was referred for hyperprolactinemia and amenorrhea. She endorsed occasional headaches and complained of decreased libido and 10lbs weight gain. Her BMI was 41.93 kg/m(2), BP 148/69 mm Hg, HR 92. Her exam was significant for slight divergent eyes with mild superior defects in the visual fields, as well as cervical lymphadenopathy. Her serum sodium was 143 mmol/l, prolactin 72.2ng/ml, TSH 2.350 mIU/L, FT4 0.58 ng/dl, ACTH 26 pg/ml and AM cortisol 10.6 mcg/dl, and cosyntropin stimulation testing was normal. She was started on levothyroxine. Brain MRI showed heterogeneous enhancement of the pituitary gland and a thickened infundibulum. To evaluate for systemic disease a chest CT scan was done, revealing multiple pulmonary nodules, patchy infiltrates with tree-in-bud appearance, as well as mediastinal and hilar lymphadenopathy. She had circumferential thickening of the distal esophagus. Biopsies of the esophagus and a sub-carinal lymph node revealed acute esophagitis with ulceration, and well-formed noncaseating granulomas with stains negative for mycobacteria and fungi. A diagnosis of sarcoidosis was made with presumed pituitary involvement. Treatment with prednisone 35 mg a day was initiated with resulting decrease in size of the pituitary gland and infundibulum on repeat MRI after 8 months. In an attempt to decrease steroid use, she was started on azathioprine but failed treatment. She showed improvement on MRI imaging with methotrexate and her steroids were subsequently tapered, but unfortunately, her hyperprolactinemia and amenorrhea did not resolve despite 18 months of stable imaging and treatment. Conclusion In patients with diffuse infundibular and pituitary thickening, a diagnosis of neurosarcoidosis should be considered. Although corticosteroid therapy improves radiologic lesions, its resulting side effects may require steroid sparing therapy, and in this case azathioprine was not effective, yet methotrexate was. However, hormonal dysfunction persisted, and menses had not returned as of 1.5 years despite resolution of imaging abnormalities on MR. Further prospective studies are needed to determine how aggressive initial therapies should be and which ones might work best to preserve pituitary function.