Endocrine Abstracts

Introduction: Autoimmune polyglandular syndrome type 2 (APS type 2) is co-occurrence of primary adrenal insufficiency (Addison disease) and autoimmune thyroid disease – most commonly Hashimoto thyroiditis (Schmidt syndrome). If it is associated with type 1 diabetes mellitus it is known as Carpenter syndrome. We present clinical pictures of two 33-year-old patients (born in 1980) diagnosed with APS type 2 manifested as Carpenter syndrome to emphasize different courses of the disease.

Case report: A 33-year-old male diagnosed with diabetes type 1 at the age of 19, treated with intensive insulin therapy (currently Lispro+Glargine), at 22 presented with hypothyroidism in the course of autoimmunological thyroiditis required replacement therapy with levothyroxine. The patient was metabolically balanced until 2007 (HbA1c<6.5%), but at the age of 27 among severe symptoms of adrenal crisis he was diagnosed with primary hypoadrenalism. The patient received a typical replacement therapy (Hydrocortisone+Cortineff) but has not attained metabolic balance (HbA1c chronically >7%). Additionally, the subject was diagnosed with vitiligo.

A 33-year-old male diagnosed with diabetes type 1 at the age of six, treated with intensive insulin therapy (currently Glulisine+Glargine), additionally at 24 diagnosed with Addison disease, which required replacement therapy (Hydrocortisone+Cortineff), whereas when 33 the patient developed another endocrinopathy, i.e. hyperthyroidism in the course of Graves disease. Currently, the patient is undergoing a thyreostatic therapy. Coinciding endocrinopathies did not result in worsening of metabolic balance (HbA1c chronically <6.5%).

Conclusions: Coinciding endocrinopathies in the course of Carpenter syndrome require careful and prompt diagnosis as the types of endocrinopathies and their course may not be anticipated on the basis of the patient’s age. Hormonal replacement therapy must be individually adjusted to the specific coincidence of endocrinopathies in the course of APS type 2 manifested as Carpenter syndrome.