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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Lymphocytic hypophysitis


Other Names for this Disease
  • Autoimmune hypophysitis
  • LH
  • LYH
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Overview



What is lymphocytic hypophysitis?

What are the signs and symptoms of lymphocytic hypophysitis?

What causes lymphocytic hypophysitis?

How might lymphocytic hypophysitis be treated?


What is lymphocytic hypophysitis?

Lymphocytic hypophysitis (LH) is a condition in which the pituitary gland becomes infiltrated by lymphocytes, resulting in pituitary enlargement and impaired function.[1][2] It most often occurs in women in late pregnancy or the postpartum period, but can also occur in prepubertal or post-menopausal women, and in men.[1][2] Symptoms of LH may include headache, visual field impairment and more rarely, double vision (diplopia).[1] The exact cause is unknown but is thought to be autoimmune-related.[2][1] Although some cases resolve on their own or after a short course of steroids, other cases cause persistent problems even with aggressive medical or surgical treatment.[3]
Last updated: 10/1/2012

What are the signs and symptoms of lymphocytic hypophysitis?

Individuals affected with lymphocytic hypophysitis typically have headaches as their first symptom.[2][1] This usually precedes or occurs with visual field impairment; rarely, double vision (diplopia) may be present.[1] Hyperprolactinemia affects approximately one third of individuals, causing amenorrhea (absence of menstruation) or galactorrhea (production of breast milk) in women and sexual dysfunction in men.[1]

Other features that occur more rarely and are related to alterations in pituitary secretions may include:[1]
Last updated: 10/1/2012

What causes lymphocytic hypophysitis?

The exact cause of lymphocytic hypophysitis (LH) is still under debate and remains unclear. It has been suggested that LH has an autoimmune etiology. This has generally been supported by the frequent postpartum occurrence and the lymphocytic infiltration, as well as several histopathological, laboratory and clinical findings.[1][2]
Last updated: 10/1/2012

How might lymphocytic hypophysitis be treated?

Lymphocytic hypophysitis can evolve differently among affected individuals, so different treatment strategies may be required. Some individuals experience spontaneous remission (improvement or reversal of the condition without any formal treatment). Careful follow-up is typically recommended.[1]

Many affected individuals only have headaches as a manifesting symptom, and there have been multiple reports of these individuals improving with glucocorticoids alone.[4] Although the use of glucocorticoids or other anti-inflammatory and immunosuppressive drugs have been suggested as medical treatment, their long-term efficacy still needs to be confirmed. High-dose methylprednisolone pulse therapy (short, intensive administration given at set intervals) seems to be effective in about 30% of treated patients.[1]

Transsphenoidal surgery (accessed "through the nose") to confirm diagnosis and save viable pituitary tissue may be required in individuals with symptoms or signs of severe compression.[1] Visual improvement following decompression with this type of surgery has been reported.[4] In some cases, pituitary biopsy is both diagnostic and therapeutic, because after this procedure a progressive recovery of pituitary function may occur.[1]
Last updated: 10/1/2012

References
  1. Antonio Bellastella, Antonio Bizzarro, Concetta Coronella, Giuseppe Bellastella, Antonio Agostino Sinisi and Annamaria De Bellis. Lymphocytic hypophysitis: a rare or underestimated disease?. European Journal of Endocrinology. 2003; 149:363-376.
  2. Peter J Snyder, MD. Causes of Hypopituitarism. UpToDate. Waltham, MA: UpToDate; 2012;
  3. Schreckinger M, Francis T, Rajah G, Jagannathan J, Guthikonda M, Mittal S. Novel strategy to treat a case of recurrent lymphocytic hypophysitis using rituximab. J Neurosurg. June 2012;
  4. Bernard Corenblum. Pituitary Disease and Pregnancy. Medscape Reference. July 6, 2011; http://emedicine.medscape.com/article/127650-overview#a30. Accessed 10/1/2012.