Endocrine Abstracts

Introduction: Autoimmune polyendocrine syndrome type 2 is a rare disease characterized by the presence of autoimmune Addison’s disease in combination with thyroid autoimmune disease and/or type 1 diabetes mellitus, and possible occurrence of other autoimmune non-endocrine disorders. The combination of the three glands diseases is even rarer and is referred to as Carpenter’s syndrome.

A long time interval is often present between the manifestations of the first to second/third component of the disease which may reach years and decades and it is unusual to find simultaneous occurrence of two or more glands disorders.

Case report: We present here a case of 47 years old British woman who was admitted with hyponatremia and significant postural hypotension and was subsequently confirmed to have Addison’s disease. Upon starting her on steroids replacement, she was re-admitted within 3 weeks with non-ketotic hyperglycaemia where an underlying autoimmune diabetes was confirmed. Subsequently she was found to have an autoimmune subclinical hypothyroidism, fulfilling the criteria of Carpenter’s syndrome. We believe that the long standing cortisol deficiency in our patient had masked her underlying autoimmune diabetes and prevented her from having marked hyperglycaemia which was only spotted upon replacing her with glucocorticoids.

Conclusion: Autoimmune polyendocrine syndrome type 2 is a rare entity, but can lead to serious life-threatening problems if not identified and treated early, so a high index of suspicion is required.

Carpenter’s syndrome is rare and only few cases have been reported in literature. Our case to the best of our knowledge is singular as the syndrome manifested with all the features presenting almost simultaneously within a short time span.