Health-related quality of life in primary and secondary adrenal insufficiency

Abstract

Adrenal insufficiency (AI) is characterized by a deficient production of glucocorticoids with or without associated mineralcorticoid and/or adrenal androgen deficiencies. Despite the low prevalence of AI, its impact on the affected patient is very high, and can be life-threatening disease if not adequately treated. Several glucocorticoid treatment regimens are available, but none is capable of perfectly imitating the cortisol circadian rhythm. Cortisol rhythmicity and treatment of other possible concomitant conditions often associated (e.g., autoimmune disorders and panhypopituitarism) are essential to improve outcome of AI. Morbidity often present in treated AI include an unhealthy metabolic profile, bad quality of sleep, infertility, sexual dysfunction and worse health-related quality of life. This review focuses on psychological morbidity and impaired quality of life in patients with primary or secondary AI of any origin, including a special section devoted to congenital adrenal hyperplasia.

Keywords::

• Addison’s disease
• congenital adrenal hyperplasia
• health-related quality of life
• primary adrenal insufficiency
• quality of life
• secondary adrenal insufficiency

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Adrenal insufficiency is a disease characterized by a deficient production of glucocorticoids, with or without deficiency in mineralcorticoids and/or adrenal androgens.

• Its impact on the affected individual is very high as it can be life-threatening if not treated or lead to health problems and impaired quality of life (QoL) if inadequately treated.

• The main goals of therapy are to obtain a good QoL, a full professional activity (or housekeeping), normal sex life and to avoid adrenal crises.

• The reported data on long-term effects on QoL of dehydroepiandrostenedione sulfate or testosterone replacement therapy in female patients with primary adrenal insufficiency are conflicting and not well known.

• Identifying potential biomarkers or monitoring treatment will be of the utmost importance for both clinical practice and research.

• An improvement of the medical treatment, monitoring and the general care of patients with congenital adrenal hyperplasia is required. It is extremely important to adjust treatment to individual needs, balancing biochemical markers, compliance and long-term risks.

• Annual follow-up of adrenal insufficiency patients should be provided to evaluate the biological condition; remind about replacement therapy dose, both every day and during intercurrent disease and QoL and screening for possible associated conditions.

• Whether improvement in QoL will occur with a more physiological replacement strategy is still a matter for debate, and awaits confirmation in future studies.

Notes