Hypophysitis: An update on the novel forms, diagnosis and management of disorders of pituitary inflammation

Publication date: Available online 12 December 2019Source: Best Practice &Research Clinical Endocrinology &MetabolismAuthor(s): Sriram Gubbi, Fady Hannah-Shmouni, Joseph G. Verbalis, Christian A. KochAbstractHypophysitis is a heterogeneous condition that leads to inflammation of the sella and/or suprasellar region, potentially resulting in hormonal deficiencies and/or mass effects. A preponderance of hypophysitis subtypes have an underlying autoimmune aetiology. The overall incidence and prevalence of hypophysitis has dramatically increased over the past decade, mainly due to increased awareness of the condition in the medical community, improvements in imaging techniques, and a rise in the occurrence of certain forms of hypophysitis such as IgG4 hypophysitis (IgG4Hy) and immune checkpoint inhibitor induced hypophysitis (ICIHy). The clinical presentation varies from an asymptomatic condition to a fatal disease often as a result of electrolyte abnormalities due to glucocorticoid deficiency in the context of adrenal crisis from central adrenal insufficiency. Milder forms of hypophysitis are treated with replacement of deficient hormones while more acute presentations with mass effects require glucocorticoid therapy, immunosuppressive therapy or surgery. Timely diagnosis and interventions are keys to prevention of the lethal complications of this disease. In this review, we provide an update on the recent advances in the field of pituitary autoimmunity, with an emphasis ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - Category: Endocrinology Source Type: research

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Abstract Synthetic glucocorticoids are widely prescribed for the treatment of numerous inflammatory and autoimmune diseases and they can also affect the way the adrenal gland produces endogenous glucocorticoids. Indeed, patients undergoing synthetic glucocorticoid treatment can develop adrenal insufficiency, a condition characterised by reduced responsiveness of the adrenal to ACTH stimulation or stressors (e.g. surgical or inflammatory stress). To better elucidate the long-term effect of synthetic glucocorticoids treatment and withdrawal on adrenal function, we have investigated the effects of prolonged treatment...
Source: Brain, Behavior, and Immunity - Category: Neurology Authors: Tags: Brain Behav Immun Source Type: research
Publication date: Available online 30 January 2020Source: Best Practice &Research Clinical Endocrinology &MetabolismAuthor(s): Serena Saverino, Alberto FalorniPrimary adrenal insufficiency (PAI) occurs in 1/5000–1/7000 individuals in the general population. Autoimmune Addison's disease (AAD) is the major cause of PAI and is a major component of autoimmune polyendocrine syndrome type 1 (APS1) and type 2 (APS2). Presence of 21-hydroxylase autoantibodies (21OHAb) identifies subjects with ongoing clinical or pre-clinical adrenal autoimmunity. AAD requires life-long substitutive therapy with two-three daily doses ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - Category: Endocrinology Source Type: research
Publication date: Available online 30 January 2020Source: Best Practice &Research Clinical Endocrinology &MetabolismAuthor(s): Serena Saverino, Alberto FalorniAbstractPrimary adrenal insufficiency (PAI) occurs in 1/5,000-1/7,000 individuals in the general population. Autoimmune Addison’s disease (AAD) is the major cause of PAI and is a major component of autoimmune polyendocrine syndrome type 1 (APS1) and type 2 (APS2). Presence of 21-hydroxylase autoantibodies (21OHAb) identifies subjects with ongoing clinical or pre-clinical adrenal autoimmunity. AAD requires life-long substitutive therapy with two-three da...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - Category: Endocrinology Source Type: research
Publication date: Available online 12 December 2019Source: Autoimmunity ReviewsAuthor(s): Ruth Percik, Gadi Shlomai, Amir Tirosh, Amit Tirosh, Raya Leibowitz-Amit, Yael Eshet, Gahl Greenberg, Alex Merlinsky, Ehud Barhod, Yael Steinberg-Silman, Tal SellaAbstractObjectiveImmune checkpoint inhibitors have introduced a new and heterogeneous class of immune-related adverse effects, with the endocrine system being a predominant target for autoimmunity. Autoimmune hypothalamic-pituitary-adrenal axis (HPA) diseases induced by checkpoint inhibitors are being increasingly recognized. We aimed to characterize the spectrum of checkpoi...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
In conclusion, in this review all the known pathog enic mechanisms causing disorders of nephrological interest in adrenal insufficiency are discussed.
Source: Internal and Emergency Medicine - Category: Emergency Medicine Source Type: research
In this study, we report an Iranian patient who presented with Addison disease, chronic mucocutaneous candidiasis, alopecia totalis, keratopathy and asplenia treated as an isolated endocrinopathy for 25 years. In the adulthood, the diagnosis of APECED was made by genetic analysis which demonstrated homozygous nonsense p.R257* (c.769C>T) mutation of AIRE. APECED has been shown to be frequent in some ethnicities including Iranian Jews. Therefore, we reviewed 39 Iranian APECED patients published in the literature. We found that most of the Iranian patients were of Jewish ethnic background and presented hypoparathyroidism, ...
Source: Immunological Investigations - Category: Allergy & Immunology Tags: Immunol Invest Source Type: research
Conclusion: To the best of our knowledge, this is the first description of autoimmune adrenalitis in a patient with 3βHSD2 deficiency and suggests a possible association between AAD and inborn errors of the steroidogenesis.
Source: Frontiers in Endocrinology - Category: Endocrinology Source Type: research
Publication date: Available online 8 August 2019Source: Autoimmunity ReviewsAuthor(s): Alberto Romano, Donato Rigante, Clelia CipollaAbstractThe contribution of autoimmune phenomena to dysfunction of hypophysis or hypothalamus is far to be unraveled and also the specific pathways of hypophysitis are poorly understood until now, mostly for the pediatric population. Primary hypophysitis is rare in children and often regarded as an autoimmune disorder, following the evidence of lymphoplasmacytic infiltration in the pituitary gland, detection of anti-pituitary antibodies (APA) and anti-hypotalamus antibodies (AHA) by indirect ...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
ConclusionQuantitative targeted GCMS steroid metabolome analysis could support monitoring of glucocorticoid replacement treatment in patients with PAI.Graphical abstract
Source: Steroids - Category: Drugs & Pharmacology Source Type: research
Shanshan Zhang1, Dongli Yuan2 and Ge Tan1* 1Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China 2The Institute of Medical Information, Chongqing Medical University, Chongqing, China Primary systemic vasculitis can affect every structure in both the central and peripheral nervous system, causing varied neurological manifestations of neurological dysfunction. Early recognition of the underlying causes of the neurological symptoms can facilitate timely treatment and improve the prognosis. This review highlights the clinical manifestations of primary systemic vasc...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
More News: Addison's Disease | Adrenal Insufficiency | Autoimmune Disease | Endocrinology | Hormones