The prevalence of Addison's disease (AAD) in Europe has increased from 40-70 cases per million population in the 1960s to 93-144 cases per million population by the end of the last century and in recent years, with a currently estimated incidence of 4.4-6 new cases per million population a year. Now, patients with AAD need to receive lifelong therapy. This phenomenon shows that the lack of AAD therapeutic strategies is obvious. As a global-leading drug development services provider, Creative Biolabs has advanced technologies, experts with high-degree, and excellent technicians. We are confident in providing high-quality autoimmune drug development services to global clients.
Addison's disease (AAD), or primary adrenocortical insufficiency, is characterized by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and mineralocorticoids. The disease often manifests itself between the 2nd and 4th decade of life and affects women more often than men. Patients may present with several diffuse symptoms such as fatigue, nausea, dizziness, and weight loss, but can often be identified by specific signs like salt-craving and, especially, hyperpigmentation of the skin and mucosal surfaces. The latter is caused by increased stimulation of melanocortin receptors in the skin by ACTH and pro-opiomelanocortin (the precursor for ACTH), which builds up in patients due to lack of cortisol-mediated feedback inhibition of the HPA-axis. Consequently, elevated plasma ACTH, as well as low serum cortisol levels, serve as diagnostic criteria for AAD.
Fig.1 The simplified model of the pathogenesis of autoimmune Addison's disease. (Hellesen, 2018)
There is no antibody or cell-based therapeutic strategies for AAD. Because of the absolute deficiency of adrenal cortex hormones caused by the destructive autoimmune process, patients with AAD require life-long substitutive treatment with hydrocortisone (HC) (or cortisone acetate in those countries where oral HC is not available). Unfortunately, no single biochemical or hormonal marker can be used to adjust the dose of glucocorticoid substitutive therapy. Thus, measuring blood pressure, serum electrolytes, and plasma renin activity levels is useful to optimize the therapy. Patients with AAD should carry a medical identification card, outlining their current therapy, and containing advice for dealing with emergency situations such as febrile illnesses, injury, vomiting, surgical procedures, dental extractions, or pregnancies. In such situations, the glucocorticoid intake needs to be doubled or tripled, possibly via intramuscular or intravenous injection.
The development of radio binding assays has demonstrated that 21OH autoantibodies (21OHAb) have a high diagnostic sensitivity and specificity for AAD, being detected in over 95% of cases with clinically idiopathic adrenal insufficiency. 21OHAb are predominantly IgG1, with a minor expression of IgG2 and IgG4. Similar to other organ-specific autoantibodies, they have no major pathogenic role in the development of adrenal insufficiency. Because of the importance of 21OHAb determination for etiological diagnosis of AAD, diagnosis of autoimmune POI, and identification of at-risk subjects, standardization of this assay is highly relevant and inter-laboratories concordance studies with serum exchange programs are strongly needed.
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