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Immunoendocrinology: Scientific and Clinical Aspects pp 547–567Cite as

Pituitary Autoimmunity

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Part of the book series: Contemporary Endocrinology ((COE))

Summary

Lymphocytic hypophysitis (LYH) is an autoimmune disease characterized by selective destruction of pituitary hormone-secreting cells due to an autoimmune aggression to pituitary gland. Moreover, the organ-specific disease of the neurohypophyseal system is characterized by selective immune attack to hypothalamic vasopressin-secreting cells often accompanied by lymphocytic-infundibulo neurohypophysitis (autoimmune inflammatory process involving the infundibulum stem and the posterior lobe). Even though LYH is still considered uncommon, its true prevalence and incidence are underestimated because it is largely misdiagnosed. With regard to criteria for autoimmunity in LYH, animal models of LYH, as indirect evidence, and presence of listing of autoimmune markers, as circumstantial evidence, have been described. Pituitary biopsy is still considered the gold diagnostic standard for LYH in symptomatic patients, but it is not always consented because it is an invasive procedure. Morphological findings on magnetic resonance imaging (MRI) of hypothalamic-pituitary region may be helpful, but sometimes they overlap those of pituitary adenoma. Thus, search for antipituitary antibodies (APA) could be considered a further tool for diagnosing LYH. However, even though organ-specific antibodies are thought to be good markers of many autoimmune endocrine diseases, the role of APA in LYH is still discussed owing to various methodological and clinical interpretative difficulties. In fact, several methods have been proposed to detect APA; immunoblotting assay and in vitro transcription–translation and immunoprecipitation of pituitary protein assay have identified a significant number of putative pituitary antigens. A recent reevaluation of APA by immunofluorescence method in patients with other autoimmune diseases and in patients with apparently idiopathic hypopituitarism allowed us to find out those with pituitary impairment (APA positive at high titers), suggesting that the occurrence of LYH in these patients is more frequent than that so far considered. Moreover, a selective staining of somatotrophs and gonadotrophs in patients with idiopathic isolated growth hormone deficiency and in patients with isolated hygonadotropic hypogonadism, respectively, has been evidenced by a four-layer fluorochrome method. The detection of APA immunostaining selectively the pituitary hormone-producing cells could be suggested as an ideal tool in diagnosing LYH in patients with hypopituitarism and normal morphological findings on hypothalamic-pituitary MRI. With respect to the therapeutic strategy of LYH, a careful follow-up is required in patients without important adrenal impairment and symptoms and/or signs of severe compression as the visual field impairment, since a possible spontaneous remission can occur. Corticosteroid or other immunosuppressive therapies in patients with pituitary-mass symptoms have been proposed not only to reduce the pituitary mass but also to recover pituitary function. Moreover, replacement therapy of pituitary hormone deficiencies, when occurring, has to be associated. Cabergoline therapy in patients with hyperprolactinemia and hypopituitarism has been observed to induce remission of pituitary impairment in some patients with LYH, probably interrupting the immune damage of the pituitary cells through the normalization of PRL levels and/or a direct immunosuppressive effect of the drug, but further studies are required to confirm these assumptions.

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Authors and Affiliations

  1. Endocrinology and Metabolism, Second University of Naples, Naples, Italy

    Annamaria De Bellis

  2. Department of Clinical and Experimental Medicine and Surgery, “F. Magrassi, A. Lanzara”, Second University of Naples, Naples, Italy

    Antonio Bizzarro & Antonio Bellastella

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  1. Annamaria De Bellis
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  2. Antonio Bizzarro
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  3. Antonio Bellastella
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Editors and Affiliations

  1. Health Sciences Center, Children's Hospital, University of Colorado, East 9th Avenue 4200, Denver, 80262, Colorado, USA

    George S. Eisenbarth

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De Bellis, A., Bizzarro, A., Bellastella, A. (2011). Pituitary Autoimmunity. In: Eisenbarth, G. (eds) Immunoendocrinology: Scientific and Clinical Aspects. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-478-4_33

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