Update on the diagnosis and management of hypophysitis

The Pituitary Center, Department of Medicine, Division of Endocrinology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
Current opinion in endocrinology, diabetes, and obesity (Impact Factor: 3.37). 04/2012; 19(4):314-21. DOI: 10.1097/MED.0b013e32835430ed
Source: PubMed


To discuss the multiple forms of hypophysitis, the various methods of classification, the recent findings in pituitary autoimmunity and novel methods of treating resistant or recurrent hypophysitis. (January 2010-December 2011).
Multiple novel presentations of hypophysitis have been described including cytotoxic T-lymphocyte antigen-4 antibody-related hypophysitis, an adverse event associated with a novel treatment for cancer, Food and Drug Administration approved for use in the treatment of metastatic melanoma. A rare, but newly described entity of immunoglobulin G4 related plasmacytic hypophysitis has been described and reviewed. Multiple investigations addressing the role of autoimmunity in the diagnosis and pathogenesis of hypophysitis have been reported with positive antipituitary antibodies found in patients with autoimmune hypophysitis, other autoimmune diseases, and nonimmune-related pituitary diseases. Several case series and case reports present new associations with concomitant diseases and novel therapy for cases requiring treatment when standard therapy fails or is contraindicated.
Hypophysitis is a rare disease with multiple subtypes. The description of hypophysitis related to cytotoxic T-lymphocyte antigen-4 antibody treatment is one of the first descriptions of hypophysitis triggered by medication. As the use of this novel treatment for cancer increases, so must our awareness of immune-related adverse effects and their treatment. Pituitary autoimmunity is a challenging field with multiple discoveries reported to help further our understanding of the disease and assist in diagnosis. Insufficient sensitivity and specificity of the currently reported methods prevents recommending measurement of antipituitary antibodies as standard of care in the diagnosis of hypophysitis. The treatment of hypophysitis remains controversial with recommendations ranging from hormonal replacement to newly described therapies such as azathioprine and radiation.

36 Reads
  • [Show abstract] [Hide abstract]
    ABSTRACT: Pituitary metastases (PM) from thyroid cancer are rare, but their management can represent a difficult challenge for the endocrinologist. Our aim was to review all reported cases of PM from thyroid cancer. PubMed was consulted and 19 papers reporting 20 cases were found. We moreover discuss two of our own cases, which had come to our attention within a few months of one another. Eleven cases were follicular cancer, eight cases were papillary cancer, two cases were medullary cancer, and one case was an insular cancer. In nine cases, PM was the first sign of the disease. Cranial nerve involvement was the most common sign of its presence, and no neuroradiological imaging could lead to a sure diagnosis of PM. Neurosurgical intervention was performed in almost all cases, and post-surgical treatment comprised radioiodine, external beam radiotherapy, and radiosurgery. Prognosis was poor for larger metastases, cranial nerve palsy disappeared in only one case, and in only one case of intrasellar metastasis was the disease cured. PM from thyroid cancer are rare, but are burdened by a poor prognosis. An early diagnosis appears important, and a comprehensive strategy for treatment (neurosurgery, radioiodine, external radiotherapy, and radiosurgery) appears advisable.
    Endocrine 09/2012; 43(3). DOI:10.1007/s12020-012-9806-9 · 3.88 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: To report an unusual case of biopsy-proven autoimmune hypophysitis with predominant hypothalamic involvement associated with empty sella, panhypopituitarism, visual disturbances and antipituitary antibodies positivity. We present the history, physical findings, hormonal assay results, imaging, surgical findings and pathology at presentation, together with a 2-year follow-up. A literature review on the hypothalamic involvement of autoimmune hypophysitis with empty sella was performed. A 48-year-old woman presented with polyuria, polydipsia, asthenia, diarrhea and vomiting. The magnetic resonance imaging (MRI) revealed a clear suprasellar (hypothalamic) mass, while the pituitary gland appeared atrophic. Hormonal testing showed panhypopituitarism and hyperprolactinemia; visual field examination was normal. Pituitary serum antibodies were positive. Two months later an MRI documented a mild increase of the lesion. The patient underwent biopsy of the lesion via a transsphenoidal approach. Histological diagnosis was lymphocytic "hypothalamitis". Despite 6 months of corticosteroid therapy, the patient developed bitemporal hemianopia and blurred vision, without radiological evidence of chiasm compression, suggesting autoimmune optic neuritis with uveitis. Immunosuppressive treatment with azathioprine was then instituted. Two months later, an MRI documented a striking reduction of the hypothalamic lesion and visual field examination showed a significant improvement. The lesion is stable at the 2-year follow-up. For the first time we demonstrated that "hypothalamitis" might be the possible evolution of an autoimmune hypophysitis, resulting in pituitary atrophy, secondary empty sella and panhypopituitarism. Although steroid treatment is advisable as a first line therapy, immunosuppressive therapy with azathioprine might be necessary to achieve disease control.
    Pituitary 05/2013; 17(3). DOI:10.1007/s11102-013-0487-z · 3.20 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: This article reviews recent literature on sellar region masses that most closely mimic non-secretory pituitary adenomas: hypophysitis, pituicytoma, spindle cell oncocytoma, and granular cell tumor of neurohypophysis. Even today, these 4 entities often cannot be confidently distinguished from each other clinically or by preoperative neuroimaging features. Thus, they often come to biopsy/surgical resection and require tissue confirmation of diagnosis. Causes of secondary and primary hypophysitis will be discussed, including two newly-described types, IgG4 plasma cell hypophysitis and hypophysitis caused by anti-CTLA4 antibody therapy for cancer. For the neoplastic conditions, emphasis will be placed on literature that has emerged since these entities were first codified in the 2007 World Health Organization fascicle (38, 39, 119). The finding that immunohistochemical staining for thyroid transcription factor-1 (TTF-1) is shared by pituicytoma, spindle cell oncocytoma, and granular cell tumor of neurohypophysis suggests common lineage and explains why histological overlap can be encountered. We incorporate our own experiences over the last 30 years from two referral institutions with specialty practices in pituitary region masses.
    Brain Pathology 05/2013; 23(5). DOI:10.1111/bpa.12068 · 3.84 Impact Factor
Show more