SUN-057 - A Hypophysitis Parody

A 36 year old African American presented to ED on July 27 with progressive musculoskeletal weakness and 50 lb weight loss in 4 months. No known medical illness. On exam he was cachectic and had dry skin.Muscle strength was decreased in all peripheral joint extremities but no neurological deficits. Labs with WBC 6.31 Hb 10.5 Platelet 257 Na135 K 4.2 BUN 12 Cr 1.18 Glucose 86 Calcium 8.8 AST 31 ALT 22 T Bilirubin 0.3 Prealbumin 7.2 TSH 1.65 Free T4 0.6 Imaging of the chest showed mediastinal lymphadenopathy. Imaging of head, neck abdomen did not show abnormal findings that correlated with his failure to thrive. A random serum cortisol level was obtained.

A 6AM serum cortisol resulted 1.4. ACTH collected 8:22 AM was 18.
A baseline cortisol of 2.3 followed by Cosyntropin stimulation test resulted in 30 min cortisol 6.30 60 min cortisol 7.8 confirming Central AI.
IGF 1 34 Z score - 5.7 Prolactin 68.6 LH < 0.3 FSH < 1 Total Testosterone 3.3 Free Testosterone 0.3
MRI Pituitary revealed abnormal thickening and enhancement of pituitary infundibulum. A hypoenhancing focus in posterior right pituitary was seen. Patient started on Hydrocortisone 15/5mg and discharged on 7/31.

He visited outpatient endocrinology on 8/12. He had gained 16 lb and had headaches. A repeat MRI head was done 11/04. The pituitary gland was normal in size and architecture. Previous hypoenhancing nodule in posterior pituitary was not seen. Pituitary infundibulum was less enhanced than on 7/31.

Extensive leptomeningeal enhancement was found on cerebral hemisphere, cerebellum along perivenular regions of brainstem. Leptomeningeal enhancement of cranial nerves 3 5 6 7 8 seen bilaterally as well as on basal ganglia. A CSF analysis obtained 11/10 showed 48 nucleated cells predominant lymphocytes protein 403 glucose 12 4 bands and cultures were sterile.

Our differential narrowed to neurosarcoidosis, lymphoma or atypical fungal infection.
He received Methylprednisolone 1000mg IV x 5 days and IVIG ( 2g/kg divided over 5 days ) for empiric treatment of granulomatous disease.

Hematology workup revealed low LDH SPEP without M spike Flow cytometry with few atypical B cells, 75% T cells without clonality. Following this inflammatory diagnosis was favored and a biopsy of mediastinal lymph node was obtained.
EBUS of mediastinal LN was done on 12/03 and showed Granulomatous inflammation that confirmed diagnosis of sarcoidosis.

Since then patient has been initiated on Infliximab in addition to prednisone.
PJP prophylaxis started. Ophthalmology referral for uveitis screening.

The patient was experiencing loss of libido and erections on subsequent visit. After diagnosis, he has now been initiated on Testosterone replacement therapy.

This case thoroughly reflects a stepwise approach in determining etiology of pituitary dysfunction. Determining etiology is crucial before initiating chronic long term hormonal replacement in any pituitary insufficiency.

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