Therapy options for adrenal insufficiency and recommendations for the management of adrenal crisis (original) (raw)
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Adrenal insufficiency can be subdivided into three broad categories: (1) Chronic primary adrenal insufficiency, also called Addison's disease, in which autoimmune adrenalitis is the most frequent cause (Carey RM 1997). (2) Chronic secondary adrenal insufficiency is most frequently caused by hypothalamic-pituitary tumors (3) acute adrenal crisis may result from stress in patients with chronic adrenal insufficiency who are not adequately replaced, but it also occurs in patients with acute adrenal hemorrhage or pituitary apoplexy (Yoram Shenker 2001). Chronic replacement therapy with glucocorticoids and, in primary adrenal insufficiency, mineralocorticoids requires careful monitoring. However, current replacement strategies still require optimization as evidenced by recent studies demonstrating significantly impaired subjective health status and increased mortality due to inappropriate GC replacement therapy in patients with primary and secondary adrenal insufficiency (ArltW2009). Further studies have explored the potential of modified delayed-release hydrocortisone to improve the prospects of patients with adrenal insufficiency.
BMC endocrine disorders, 2014
Increased morbidity and mortality associated with conventional glucocorticoid replacement therapy for primary adrenal insufficiency (primary AI; estimated prevalence 93-140/million), secondary AI (estimated prevalence, 150-280/million, respectively) or congenital adrenal hyperplasia (estimated prevalence, approximately 65/million) may be due to the inability of typical glucocorticoid treatment regimens to reproduce the normal circadian profile of plasma cortisol. A once-daily modified-release formulation of hydrocortisone has been developed to provide a plasma cortisol profile that better mimics the daytime endogenous profile of cortisol. Here, we describe the protocol for the European Adrenal Insufficiency Registry (EU-AIR), an observational study to assess the long-term safety of modified-release hydrocortisone compared with conventional glucocorticoid replacement therapies in routine clinical practice (ClinicalTrials.gov identifier: NCT01661387). Patients enrolled in EU-AIR have ...
Clinical endocrinology, 2017
Treatment for adrenal insufficiency (AI) remains suboptimal. Despite glucocorticoid replacement, patients with AI have reduced life expectancy and quality of life. This study aimed to describe the spectrum of management of glucocorticoid replacement in patients with AI enrolled in the European Adrenal Insufficiency Registry (EU-AIR). EU-AIR is a prospective, multinational, multicentre, observational study initiated in August 2012 to monitor the long-term safety of glucocorticoid replacement in routine clinical practice in Germany, the Netherlands, Sweden and the UK (ClinicalTrials.gov identifier: NCT01661387). This analysis included 1166 patients with primary and secondary AI (mean disease duration 16·1 ± 11·6 years) receiving long-term glucocorticoid replacement therapy. Glucocorticoid type, dose, frequency and treatment regimen were examined. Most patients (87·4%) were receiving hydrocortisone. The most common dose range, taken by 42·2% of patients, was 20 to <25 mg/day; howeve...
European journal of endocrinology, 2017
To investigate the long-term safety and tolerability of a once-daily, dual-release hydrocortisone (DR-HC) tablet as oral glucocorticoid replacement therapy in patients with primary adrenal insufficiency (AI). Prospective, open-label, multicenter, 5-year extension study of DR-HC conducted at five university clinics in Sweden. Seventy-one adult patients diagnosed with primary AI who were receiving stable glucocorticoid replacement therapy were recruited. Safety and tolerability outcomes included adverse events (AEs), intercurrent illness episodes, laboratory parameters, and vital signs. Quality of life (QoL) was evaluated using generic questionnaires. Total DR-HC exposure was 328 patient-treatment years. Seventy patients reported 1060 AEs (323 per 100 patient-years); 85% were considered unrelated to DR-HC by the investigator. Most common AEs were nasopharyngitis (70%), fatigue (52%) and gastroenteritis (48%). Of 65 serious AEs reported by 32 patients (20 per 100 patient-years), four w...
PREDICTIVE FACTORS OF BIOLOGICAL ADRENAL INSUFFICIENCY AFTER A LONG-TERM GLUCOCORTICOID THERAPY.
International Journal of Advanced Research (IJAR), 2019
Purpose:The predictive factors of adrenal insufficiency after a prolonged, continuous course of corticosteroids is poorly documented. We evaluated it retrospectively in our department of endocrinology. Methods:The patients were included between January 2015 to January 2017 and were administered a Synacthene? 250 ug test (ST250) after substitution with hydrocortisone for at least 4 to 6 weeks. A non-responsive test was defined by a cortisol increase below 21ug/dL, 60 min after stimulation. We studied the risk factors associated with biological adrenal insufficiency by SPSS analysis. Results:sixty seven patients were included (mean age: 42 ? 13,10 years). Mean initial dose of corticosteroids was 41,95 ? 34,16 mg/d. forty-three patients failed to respond to the ST250. The comparison between the responder group and the non-responder group at TS250 showed that the difference is significant for the basal cortisol level (p= 0,016) and for the duration of the CTC (p=0.045). Conclusion:Biological adrenal insufficiency is very common after a prolonged course of corticosteroids. Hence, clinicians should be vigilant for adrenal insufficiency at all degrees of glucocorticoid exposure.
European Journal of Endocrinology, 2013
BackgroundConventional glucocorticoid (GC) replacement for patients with adrenal insufficiency (AI) is inadequate. Patients with AI continue to have increased mortality and morbidity and compromised quality of life despite treatment and monitoring.Objectivesi) To review current management of AI and the unmet medical need based on literature and treatment experience and ii) to offer practical advice for managing AI in specific clinical situations.MethodsThe review considers the most urgent questions endocrinologists face in managing AI and presents generalised patient cases with suggested strategies for treatment.ResultsOptimisation and individualisation of GC replacement remain a challenge because available therapies do not mimic physiological cortisol patterns. While increased mortality and morbidity appear related to inadequate GC replacement, there are no objective measures to guide dose selection and optimisation. Physicians must rely on experience to recognise the clinical sign...
European journal of endocrinology / European Federation of Endocrine Societies, 2014
The objective was to assess the long-term safety profile of dual-release hydrocortisone (DR-HC) in patients with adrenal insufficiency (AI). Randomised, open-label, crossover trial of DR-HC or thrice-daily hydrocortisone for 3 months each (stage 1) followed by two consecutive, prospective, open-label studies of DR-HC for 6 months (stage 2) and 18 months (stage 3) at five university clinics in Sweden. Sixty-four adults with primary AI started stage 1, and an additional 16 entered stage 3. Patients received DR-HC 20-40 mg once daily and hydrocortisone 20-40 mg divided into three daily doses (stage 1 only). Main outcome measures were adverse events (AEs) and intercurrent illness (self-reported hydrocortisone use during illness). In stage 1, patients had a median 1.5 (range, 1-9) intercurrent illness events with DR-HC and 1.0 (1-8) with thrice-daily hydrocortisone. AEs during stage 1 were not related to the cortisol exposure-time profile. The percentage of patients with one or more AEs ...
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists, 2016
Various glucocorticoid regimens have been used in the treatment of patients with adrenal insufficiency, yet the differences between such regimens on health outcomes are unclear. We performed a systematic review and meta-analysis to compare the effect of various glucocorticoid regimens on quality of life, bone density, incidence of adrenal crisis and death. In pediatric studies, we searched for final adult height. We searched 6 databases through July 2016. Studies were selected and appraised by independent reviewers. Data were pooled using the profile likelihood random-effects model. We included 34 studies. We found no difference in quality of life scores between higher (≥30 mg/day of hydrocortisone equivalence) vs. lower daily doses (<30 mg/day of hydrocortisone equivalence) (P=0.15) or based on frequency of daily dosing (once, twice or thrice daily). Extended (1 study), dual release/modified release (3 studies) and continuous subcutaneous (3 studies) forms of glucocorticoids wer...