From the Guidelines
Schmidt syndrome, also known as autoimmune polyendocrine syndrome type 2 (APS-2), requires comprehensive management focusing on hormone replacement therapy for the affected endocrine glands, with treatment primarily involving glucocorticoid replacement with hydrocortisone (15-25 mg daily in divided doses) or prednisone (5-7.5 mg daily) for adrenal insufficiency, as recommended by the consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency 1. The management of APS-2 is complex and necessitates a multidisciplinary approach, considering the autoimmune nature of the syndrome and the potential for multiple endocrine gland involvement.
- Key aspects of management include:
- Glucocorticoid replacement with hydrocortisone (15-25 mg daily in divided doses) or prednisone (5-7.5 mg daily) for adrenal insufficiency, which is essential for survival 1.
- Thyroid hormone replacement with levothyroxine (typically starting at 1.6 mcg/kg/day) for hypothyroidism.
- Insulin therapy for patients with type 1 diabetes, with regimens individualized based on blood glucose monitoring.
- Fludrocortisone (0.05-0.2 mg daily) may be needed if aldosterone deficiency is present.
- Patient education and emergency preparedness are crucial, with patients required to carry emergency medical identification and have injectable hydrocortisone (100 mg) available for adrenal crisis, as emphasized in the consensus statement 1 and general principles of management 1.
- Regular monitoring includes periodic assessment of hormone levels, blood glucose, electrolytes, and screening for additional autoimmune conditions that may develop over time, with follow-up recommendations outlined in the consensus statement 1.
- The importance of stress dosing of glucocorticoids during illness or surgery cannot be overstated, typically requiring 2-3 times the normal dose, as highlighted in the general principles of management 1.
From the Research
Management of Schmidt's Syndrome
The management of Schmidt's syndrome, also known as Autoimmune Polyendocrine Syndrome type 2 (APS 2), involves treating the underlying autoimmune conditions, such as autoimmune thyroiditis and adrenal insufficiency. The following are key aspects of management:
- Treatment of acute hypoglycemic episodes with glucose or glucagon 2
- Long-term glucocorticoids and mineralocorticoids for adrenal insufficiency 2, 3
- Thyroid hormone supplements for hypothyroidism 2, 4
- Caution is needed when managing high-dose glucocorticoids along with fludrocortisone in elderly patients with cardiac disease to limit the risk of excessive mineralocorticoid activity and heart failure 5
- Identification of patients at risk for hypoglycemia in Schmidt's syndrome provides an opportunity for precision management beyond the manipulation of antihyperglycemic agents 3
- Treatment of thyroid storm with antithyroid drugs and intravenous glucocorticoids 6
- Substitution therapy for other organ-specific autoimmune disorders, such as hypergonadotropic hypogonadism 4
Treatment Considerations
When initiating treatment, the following considerations should be taken into account:
- Thyroxine therapy may precipitate Addisonian crisis in patients with Schmidt's syndrome through increasing cortisol clearance and metabolic rate 4
- The use of glucocorticoids in the treatment of thyroid storm has become standard practice due to the possibility of relative adrenal insufficiency or undiagnosed Addison's disease 6
- Patients with Schmidt's syndrome can be susceptible to nocturnal hypoglycemia with inadequate steroid replacement 3