Best Practice & Research Clinical Endocrinology & Metabolism
5Pituitary antibodies and lymphocytic hypophysitis
Section snippets
Criteria for organ-specific autoimmunity in lymphocytic hypophysitis
For a long time, on the basis of the criteria for defining a disease as autoimmune, some endocrine diseases previously considered as idiopathic have been recognized as autoimmune.10, 11, 12, 13, 14, 15, 16, 17 LYH fulfils most of the criteria required to define a disease as organ-specific autoimmune (Table 1).
Anti-pituitary antibodies
Organ-specific antibodies are good markers of many autoimmune endocrine diseases. Until now, APAs have not been considered good markers of LYH because of various difficulties in methodology and clinical interpretation. In fact, conflicting results in detecting APAs using different methods has impaired the clinical relevance of these antibodies. Moreover, a longitudinal study demonstrated that these antibodies can disappear over time43; for this reason the time of detection could influence their
Pathogenesis
Autoimmune mechanisms characterizing other organ-specific autoimmune diseases are well known because of the ability to study animal models and the availability of target organ tissues from patients in the subclinical stage or at the clinical onset of the disease. At present, the mechanisms triggering the development and progression of LYH are not known, although the presence of animal models of LYH may open the way to understanding these events.26, 27, 28 The role of CD8 T cells in autoimmune
Different expressions of LYH during the natural history of the disease
Some patients may present with symptoms and signs related to pituitary enlargement with possible extrasellar extension but without pituitary dysfunction, while others with normal characteristics on MRI may present with varying degrees of pituitary failure.25, 65, 71 Moreover, many patients with LYH can show high levels of PRL, while others may have normal or even low levels of PRL (women with failure to lactate in the postpartum period).1, 6 The natural history of LYH is very variable, often
Diagnosis of LYH: role of anti-pituitary antibodies
LYH has to be suspected in patients presenting with hyperprolactinaemia, headache, and impairment of visual field—especially if they are pregnant women or women with recent delivery and have symptoms of hypopituitarism of unclear aetiology. In these patients a trans-sphenoidal pituitary biopsy is thought to be the diagnostic gold standard for LYH.9 However, this procedure is invasive and not always feasible. Moreover, despite the recent development of sophisticated techniques of imaging, the
Therapeutic strategies
Before the start of therapeutic strategies of LYH, two important considerations may be made: first, the different expressions of this autoimmune disease require different therapeutic strategies; and second, since a possible spontaneous remission during the natural history of LYH can be observed, the improvement occurring after surgical or medical treatment could be related to spontaneous resolution rather than to the treatment itself. For this reason a careful follow-up is advisable in patients
Summary
LYH fulfils most of the criteria required to define a condition as an organ-specific autoimmune disease. It has a special place among the spectrum of autoimmune diseases, particularly because of the different forms it takes during its natural history. The presence of clear morphological pituitary characteristics of LYH on MRI, followed by trans-sphenoidal pituitary biopsy, is the diagnostic gold standard for LYH in patients with hypopituitarism and symptoms and signs of an expanding pituitary
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Cited by (57)
Clinical Features, Magnetic Resonance Imaging, and Treatment Experience of 20 Patients with Lymphocytic Hypophysitis in a Single Center
2019, World NeurosurgeryCitation Excerpt :However, conflicting results in detecting APAs by different methods have impaired the clinical relevance of those antibodies. In addition, those antibodies can disappear over time, as shown by longitudinal research.4 Caturegli et al.1 reported that an immunoblotting method is more sensitive (64% vs. 57%) and specific (86% vs. 76%) than an immunofluorescence method.
Guidelines for the diagnosis and treatment of adrenal insufficiency in the adult
2014, Endocrinologia y NutricionBilateral intracavernous carotid artery occlusion caused by invasive lymphocytic hypophysitis
2012, Journal of Stroke and Cerebrovascular DiseasesCitation Excerpt :However, because the criterion standard for the diagnosis of LYH remains trans-sphenoidal pituitary biopsy, more feasible tools are needed to identify this disease at early time points.5 Remarkably, positivity for APAs is emerging as a reliable marker for autoimmune hypophysitis.6 In our case, in fact, early testing could have prevented the bilateral occlusion of the ICAs, stroke, and subsequent disability.
Unveiling the Etiopathogenic Spectrum of Hypophysitis: A Narrative Review
2023, Journal of Personalized MedicineEvaluation of autoantibody signatures in pituitary adenoma patients using human proteome arrays
2022, Proteomics - Clinical ApplicationsOutcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis
2022, Journal of Clinical Endocrinology and Metabolism