Endocrine Abstracts

Background: Primary autoimmune hypophysitis (PAH) is a rare chronic inflammatory condition of the pituitary gland that occurs more commonly in females during pregnancy or in the post-partum period. It is strongly associated with other autoimmune disorders. The various forms of hypophysitis are misdiagnosed as pituitary adenoma in 40% cases.

Objective: First of all, we propose to present a case of PAH associated with other autoimmune diseases. Our second aim is to review the current literature about autoimmune disorders with a tendency toward familial aggregation.

Case presentation: We report a case of a 59 year-old woman who was diagnosed in post-menopause with Hashimoto’s disease, hypothyroidism, alopecia aerata, chronic hepatitis and PAH. Her mother was also diagnosed with liver cirrhosis, Graves and Addison’s diseases. Laboratory tests revealed a very low FT4 levels (<0.35 ng/dL) with slightly elevated levels of TSH (8.84 mlU/L) and thyroid peroxidase antibody (62.3 IU/ml, normal range <5.6. U/L), hypocortisolism (3.63 μg/dL), hypoglycemia (58 mg/dL), elevated red blood cells sedimentation (24/h) and lymphocytosis. The other pituitary hormones (prolactin, ACTH) and IGF-1 were in normal range. FSH (1.5 UI/L) and LH (0.1 UI/L) levels was very low and inadequate for post-menopause. MRI of the hypothalamic-pituitary region showed glandular parenchyma with diffusely reduced contrast. Hormone replacement therapy with levothyroxine and prednisone was initiated. Patient presented also an episode of fulminant, but reversible autoimmune hepatitis. Conclusion: PAH clinical diagnosis remains an exclusion diagnosis. Association of PAH with another autoimmune disorders occurring before, after or concurrently with PAH emphasizes the importance of close follow-up of such patients.

Keywords: autoimmune hypophysitis, alopecia areata, Hashimoto's disease