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Primary hypophysitis and other autoimmune disorders of the sellar and suprasellar regions

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Abstract

The pituitary gland and the hypothalamus can be affected by autoimmune-mediated structural and functional disruption. These autoimmune-mediated diseases occur more commonly in females and are often found during pregnancy or in the post-partum period. Autoimmune diseases can either affect parts of the pituitary or hypothalamus, or can involve both sellar and suprasellar structures. Most of these cases comprise primary hypophysitis (PRH). Over the years, there has been a tremendous increase in the number of reported PRH cases and related disorders, including hypophysitis induced by immune checkpoint inhibitors. With this increasing data, more light is being shed on the spectrum of clinical presentations, biochemical and imaging abnormalities of these disorders. Regardless, these disorders are still relatively rare. The clinical presentation can vary vastly, based on the type of pituitary cell or the area of the suprasellar region affected. The severity can range from clinically silent disease to progressive and rapid deterioration and death, likely due to unrecognized central adrenal insufficiency. Although biopsy remains a gold standard for diagnosing these disorders, the current standard of practice is biochemical assessment for hormonal deficiencies and imaging studies. In several instances, these disorders spontaneously resolve, but medical or surgical intervention might be necessary to treat symptomatic disease. Due to the subtlety and a vast spectrum of clinical manifestations which could often be asymptomatic, and the rarity of the occurrence of these diseases in clinical practice, the diagnosis can be easily missed which could potentially lead to substantial morbidity or mortality. Therefore, it is crucial to have a strong clinical suspicion and pursue timely biochemical and imaging studies to initiate prompt treatment. In this article, we review the various autoimmune conditions that affect the sellar and suprasellar structures, their diagnostic approach and management of these disorders.

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Abbreviations

LAH:

Lymphocytic adenohypophysitis

LINH:

Lymphocytic infundibuloneurohypophysitis

LPH:

Lymphocytic panhypophysitis

PRH:

Primary hypophysitis

GRH:

Granulomatous hypophysitis

XH:

Xanthomatous hypophysitis

NH:

Necrotizing hypophysitis

IgG4H:

IgG4 mediated hypophysitis

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Funding

This work was supported in part by the Intramural Research Program, NICHD, NIH.

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Authors and Affiliations

  1. Department of Endocrinology, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health (NIH), Bethesda, MD, USA

    Sriram Gubbi

  2. Section on Endocrinology & Genetics (SEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD, 20892, USA

    Fady Hannah-Shmouni & Constantine A. Stratakis

  3. Medicover GmbH, Berlin/Hannover, Germany

    Christian A. Koch

  4. Carl von Ossietzky University, Oldenburg, Germany

    Christian A. Koch

  5. Technical University of Dresden, Dresden, Germany

    Christian A. Koch

  6. University of Louisville, Louisville, KY, USA

    Christian A. Koch

  7. University of Tennessee Health Science Center, Memphis, TN, USA

    Christian A. Koch

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Correspondence to Christian A. Koch.

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Prof. Koch declares no direct conflict with this article. He has served on the Advisory Board of Novartis on the topic acromegaly and has participated in educational conferences on the topics neuroendocrine tumors and acromegaly sponsored by Novartis and Ipsen. The other authors Drs. Sriram Gubbi, Fady Hannah-Shmouni, and Constantine A. Stratakis declare that they have no conflicts of interest.

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Gubbi, S., Hannah-Shmouni, F., Stratakis, C.A. et al. Primary hypophysitis and other autoimmune disorders of the sellar and suprasellar regions. Rev Endocr Metab Disord 19, 335–347 (2018). https://doi.org/10.1007/s11154-018-9480-1

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