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Hypophysitis: Evaluation and Management Alexander Faje Faje Clinical Diabetes and Endocrinology (2016) 2:15 DOI 10.1186/s40842-016-0034-8 REVIEW ARTICLE Open Access Hypophysitis: Evaluation and Management Alexander Faje Abstract Hypophysitis is the acute or chronic inflammation of the pituitary gland. The spectrum of hypophysitis has expanded in recent years with the addition of two histologic subtypes and recognition as a complication of treatment with immune checkpoint inhibitors. Despite the increased number of published cases, the pathogenesis of hypophysitis is poorly understood, and treatment strategies are diverse and controversial. The diagnosis of hypophysitis generally requires histopathologic confirmation. The presentation and clinical course of hypophysitis varies. Hypophysitis can resolve spontaneously, relapse may occur, and some cases can be refractory to treatment. Keywords: Hypophysitis, Hypopituitarism, Diabetes insipidus Abbreviations: ADCC, Antibody-dependent cell-mediated cytotoxicity; CTLA-4, Cytotoxic T-lymphocyte antigen-4; ECD, Erdheim-Chester disease; IIF, Indirect immunofluorescence; LCH, Langerhans cell histiocytosis; MRI, Magnetic resonance imaging; PD-1, Programmed cell death 1 Background involve the pituitary gland (such as sarcoidosis, Wegener’s Hypophysitis has gained greater clinical recognition over granulomatosis, Crohn’s disease, Takayasu’s arteritis, time. Several histologic variants and causative agents Cogan’s syndrome), inflammatory cell proliferative have been identified. Although hypophysitis remains a disorders (Langerhans cell histiocytosis [LCH] and rare diagnosis, the number of published cases has in- Erdheim-Chester disease [ECD]), infections (tubercu- creased substantially and expanded to involve a more losis, syphilis, Whipple’s disease, mycoses), and gender and age diverse population. The quantity and tumor-associated inflammatory infiltrate (germinoma). quality of available information is limited, however, and Morphologic categorization is made according to consensus, especially regarding treatment, has been elu- whether inflammation involves the anterior pituitary gland sive. Prospective studies are necessary to better define (adenohypophysitis), posterior gland and stalk (infundibu- optimal diagnostic and management strategies. loneurohypophystis), or entire gland (panhypophysitis). Hypophysitis can be classified according to etiology, Histologic subtypes of hypophysitis include the follow- morphology, and/or histopathology. Etiology refers to ing: lymphocytic, granulomatous, xanthomatous, and primary or secondary cases of hypophysitis. Primary plasmacytic (Table 1). Occasionally, mixed histology is hypophysitis refers to isolated inflammation of the pituit- encountered [16]. Necrotizing hypophysitis has also been ary not associated with medications, systemic inflamma- proposed as an additional variant, but it has only been tory disorders, infections, or other diseases. Secondary reported in 3 cases [17, 18]. Lymphocytic hypophysitis is hypophysitis includes cases associated with immunother- characterized by diffuse lymphocyte infiltration (primar- apy (interleukin 2, interferon, and medications targeting ily T cells) of the pituitary gland. Lymphoid follicles can cytotoxic T-lymphocyte antigen-4 [CTLA-4] or pro- be observed and occasional plasma cells, eosinophils, grammed cell death 1 [PD-1]) [1–6], rupture of sellar cysts and fibroblasts may also be present [19]. Granulomatous (Rathke’s cleft cysts and craniopharyngiomas), and rarely, hypophysitis shows large numbers of multinucleated pituitary adenomas [7–15]. Some authors utilize the giant cells and histiocytes with granuloma formation term secondary hypophysitis more broadly and also [20, 21]. Xanthomatous hypophysitis demonstrates include systemic inflammatory processes which may lipid-laden “foamy” histiocytes without the presence of granulomas [22, 23]. Plasmacytic hypophysitis, also Correspondence: [email protected] termed IgG4-related hypophysitis, has extensive gland Neuroendocrine Unit, Massachusetts General Hospital and Harvard Medical infiltration by plasma cells with a high degree of IgG4 School, 55 Fruit Street, Boston, MA 02114, USA © 2016 The Author(s). Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Faje Clinical Diabetes and Endocrinology (2016) 2:15 Page 2 of 8 Table 1 Histologic subtypes of hypophysitis and patient subsequently described in men [37] and children [38– characteristics 40]. Lymphocytic hypophysitis does occur more fre- Gender Association with Mean age of quently in women compared to men (approximately 3:1 predominance pregnancy presentation ratio of cases [27]), in large part because of its associ- Lymphocytic Female, ~3:1 Yes 4th decade ation with pregnancy [41]. Though recent series have Granulomatous Female, ~3:1 No 5th decade not shown as strong a relationship [42, 43], the majority Xanthomatous Female, ~3:1 No 4th decade of cases among reproductive-aged women appear to Plasmacytic Male, ~2:1 No 7th decade occur during the end of pregnancy or the first few (IgG4-related) months after delivery [16, 27]. The incidence of lympho- Mixed histology is observed occasionally, and necrotizing hypophysitis has cytic hypophysitis peaks during the fourth decade of life been proposed as an additional category. Data abstracted from references and is uncommon in children and the elderly. [20, 23, 25, 27, 66] Granulomatous and xanthomatous hypophysitis also occur more frequently in women (approximately 3:1 positivity [24–26]. Pituitary gland fibrosis and atrophy ratio of cases), but neither form is linked with preg- may occur in later stages of these hypophysitis variants. nancy. Xanthomatous hypophysitis and lymphocytic Precise usage of the term hypophysitis is important. hypophysitis have a similar mean age of presentation, Loose or inconsistent application (such as grouping ger- but granulomatous hypophysitis is diagnosed more minoma-associated inflammation and primary lympho- often at a slightly later timepoint in the fifth decade cytic hypophysitis) can cause reader confusion and [20, 23]. IgG4-related hypophysitis occurs more fre- suggest inappropriate treatments rather than provide diag- quently in men and tends to develop at a more ad- nostic clarification. Unfortunately, such cases have been vanced age in the seventh decade of life. IgG4-related mixed with primary hypophysitis in some review paper hypophysitis also does not have an association with data sets [27]. Unless otherwise stated, further discussion pregnancy [25, 26]. in this manuscript will focus on patients with primary Immunotherapy-associated hypophysitis occurs in up hypophysitis. A caveat exists for IgG4-related hypophysi- to 10–15 % of patients receiving agents targeting CTLA- tis, which is often a manifestation of systemic disease with 4, on average 2–3 months after starting therapy. Older involvement of multiple organs. Most authors have not age and male gender may be risk factors for the develop- grouped IgG4-related hypophysitis in the general category ment of hypophysitis with anti-CTLA-4 medications. of secondary hypophysitis, though it may be reasonable to Hypophysitis is comparatively rare following treatment do so. This manuscript does include an examination of with anti-PD-1 agents. Hypophysitis has also been re- IgG4-related hypophysitis. Given the expanding applica- ported very rarely after treatment with interleukin 2 and tions of immune checkpoint inhibitors and increasing fre- interferon [6]. quency of this form of secondary hypophysitis, brief discussion will also be devoted to immunotherapy- Clinical presentation associated hypophysitis. Patients with hypophysitis present with symptoms re- lated to mass effect from pituitary gland enlargement Epidemiology and pituitary/hypothalamic dysfunction. Headache is the The annual incidence of hypophysitis is estimated to be most common presenting symptom, occurring in about 1in7–9 million. Hypophysitis accounts for approxi- half of patients. Visual symptoms due to compression of mately 0.4 % of pituitary surgery cases (based on a group the optic nerves and/or cranial nerves III, IV, and VI in of large surgical series totaling nearly 10,000 procedures the cavernous sinuses can occur in a substantial mi- at 5 centers) [28–32]. nority of patients [16, 27, 42]. Cavernous carotid ar- Lymphocytic hypophysitis was first reported in 1962 tery occlusion is a rare complication of hypophysitis [33], and granulomatous hypophysitis was described in [31, 44–46]. The onset of symptoms, including headache, the early twentieth century [34, 35]. The first cases of can be insidious, subacute, or acute even mimicking apo- xanthomatous hypophysitis and IgG4-related hypophysi- plexy [42, 47, 48]. tis were published more recently in 1998 and 2004, re- The majority of patients with hypophysitis have mul- spectively [22, 36]. tiple anterior pituitary hormone deficiencies, and anter- Lymphocytic hypophysitis is the most common histo- ior panhypopituitarism is not uncommon. The severity logic variant, with over
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