ReviewEndocrine alterations in primary Sjogren's syndrome: An overview
Highlights
► Hypothalamic-pituitary-adrenal axis is hypoactive in SS. ► Anti-21(OH) Abs in some SS patients are associated with adrenal hyporesponsiveness. ► Lack of both estrogens and active androgens favor apoptosis and immune triggering. ► Autoimmune thyroiditis is the most common endocrine dysfunction observed in SS. ► Prolactin is increased in a subset of SS patients in association with B-cell hyperactivity.
Introduction
Primary Sjogren's syndrome (SS) is classically defined as a chronic autoimmune disorder which affects the exocrine glands; as a result, impaired secretions and complaints of mucosal dryness occur. Although the exact etiology of the syndrome is not yet fully elucidated, epidemiological features such as the female predominance, the disease onset around menopause and commonly after a major stressful event, imply hormonal factors as significant pathogenetic determinants [1].
In the current review, our aim was to summarize how the complex interplay between several endocrine circuits, including the hypothalamic-pituitary-adrenal (HPA) axis, the hypothalamic-pituitary-gonadal (HPG) axis, the hypothalamic-pituitary-thyroid (HPT) axis as well the growth hormone/Insulin growth factor-1 (GH/IGF-1) axis contributes to the development of primary SS. Prolactin alterations will be also discussed (Table 1).
Section snippets
Endocrine axes in primary SS
In this section we are going to present the role of aberrations of endocrine axes in the pathogenesis of primary SS.
Conclusions
Epidemiological observations in primary SS, such as the woman-dominant phenotype as well as the disease onset around menopause, often following the occurrence of major stressful life events, are highly suggestive of hormonal implications in disease pathogenesis. Cumulative evidence so far suggested alterations of HPA, HPG, GH/IGF-1 axis and prolactin secretion as major mediators of epithelial cell apoptosis with subsequent triggering of an aberrant self directed immune response and alterations
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2018, Autoimmunity ReviewsCitation Excerpt :This affects women in particular, who must produce their salivary T and DHT locally from DHEA [61]. Low activity of the HPA axis in the setting of SS could be related to dysfunction either at a hypothalamic, pituitary or adrenal gland level due to atrophy [62]. Alterations in estrogen and androgen levels are considered important in pathogenesis of SS.
The evolution of methods for urinary steroid metabolomics in clinical investigations particularly in childhood
2018, Journal of Steroid Biochemistry and Molecular BiologyCitation Excerpt :A state of low cortisol production is found with acute intermittent porphyria [288]. Adrenal androgens and particularly DHEAS is low in individuals with primary Sjogren’s syndrome [289]. There is much potential to improve productivity of steroid profiles particularly with GC–MS. Microwave and ultrasound assisted hydrolysis and derivative formation will benefit the slowest steps in USP analysis with GC [290–295].
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Equally contributed.