Lymphocytic Hypophysitis with a Long Latent Period from Onset of Central Diabetes Insipidus to Development of Pituitary Enlargement

Abstract

A 61-year-old man presented with central lymphocytic hypophysitis. Initial pituitary MRI imaging was normal, except for loss of the "bright spot" of the posterior lobe. A diagnosis of idiopathic diabetes insipidus was made. Two years later, pituitary gland enlargement with panhypopituitarism was detected. Eight months after commencing a replacement dose of corticosteroid, the pituitary enlargement was reduced in size. These findings resulted in a diagnosis of lymphocytic hypophysitis. In patients with idiopathic diabetes insipidus, it is important to suspect lymphocytic hypophysitis and to perform a long follow-up to repeat endocrinological examinations and pituitary imaging.

An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience

“…As described by other authors [13,24,25] , primary autoimmune hypophysitis is frequently associated with other autoimmune diseases, which in our study included autoimmune polyglandular syndrome type IV, Sjögren syndrome, systemic lupus erythematosus, mixed connective tissue disease, primary biliary cirrhosis, autoimmune atrophic gastritis, pernicious anemia, celiac disease, Graves' disease, and chronic autoimmune thyroiditis.…”

Hypopituitarism Possibly due to Lymphocytic Hypophysitis in a Patient with Type 1 Diabetes

“…Therefore, this case meets the diagnostic criteria for a suspected case of lymphocytic hypophysitis. However, there have been several case reports of lymphocytic hypophysitis with a long period of latency between the onset of hypopituitarism and enlargement of the pituitary gland (7,8). For example, Akahori et al reported a patient with lymphocytic hypophysitis who developed pituitary enlargement and thickening of the pituitary stalk two years after the onset of central diabetes insipidus (8).…”

“…However, there have been several case reports of lymphocytic hypophysitis with a long period of latency between the onset of hypopituitarism and enlargement of the pituitary gland (7,8). For example, Akahori et al reported a patient with lymphocytic hypophysitis who developed pituitary enlargement and thickening of the pituitary stalk two years after the onset of central diabetes insipidus (8). In the current case, it is possible that the lymphocytic hypophysitis was detected at an early stage or constituted the first manifestation of another systemic disease.…”

Lymphocytic Hypophysitis in a Dog with Diabetes Insipidus

“…After treatment with glucocorticoids, the pituitary reduces in size (Akahori and Sugimoto, 2010). Pituitary insufficiency may affect all pituitary hormones, but normal and elevated plasma levels have also been reported, probably representing different stages of the disease (Shimono et al, 1999).…”

“…Radiological differentiation from neoplasia is impossible (Schubiger, 1996). Pituitary magnetic resonance imaging in people with lymphocytic hypophysitis presenting with diabetes insipidus shows loss of the 'bright spot' of the posterior lobe, thickening of the pituitary stalk and pituitary gland enlargement with suprasellar extension (Shimono et al, 1999;Akahori and Sugimoto, 2010). …”