Introduction: The combination of two or more, autoimmune, endocrine dysfunctions defines the presence of polyglandular syndromes.
Case report: Female patient, 43 years old, history of type 1 diabetes for 23 years and autoimmune hypothyroidism for 7 years, with no other relevant clinical history and without family history of autoimmune endocrinopathies. She was under therapy with insulin and levothyroxine. She came into the Endocrinology Department in May 2010 with frequent episodes of severe hypoglycemia during the previous month. She had no other new symptoms. Insulin therapy was then optimized until re-evaluation in 2 weeks. At that time she referred persistence of the episodes of hypoglycemia with increasing frequency. A new adjustment in treatment was made and, at the same time, levels of morning serum ACTH and cortisol were requested.
Meanwhile she developed, darkening of the skin, especially the creases and oral mucosa. She presented no other symptoms, namely, fatigue, weakness, nausea or vomiting. There was no weight loss or sings of hypotension. From the study requested, ACTH was 1098 ng/l and cortisol 2.5 μg/dl, no hyponatremia was found. Hydrocortisone therapy was initiated (10 mg+5 mg+5 mg/day) with hyperpigmentation improvement. No other hypoglycemic episodes were registered and there was the need to increase total insulin dose. Two weeks after the beginning of treatment serum ACTH was 238.6 ng/l and cortisol 2.9 μg/dl. Seven months after de beginning of treatment she was steel clinically well.
Conclusion: Autoimmune polyglandular syndrome type II is not a common disease, but it has life threatening consequences when the diagnosis is overlooked. It is defined by the combination of chronic autoimmune adrenal insufficiency with autoimmune thyroid disease, type 1 autoimmune diabetes mellitus, or both. In patients with type 1 diabetes, a sudden drop in insulin requirement point out to early adrenal insufficiency.