Endocrine Abstracts

Introduction: Autoimmune polyendocrine syndromes (APS) are rare endocrinopathies characterized by the coexistence of at least two glandular autoimmune diseases. APS comprise a wide spectrum of autoimmune disorders and are divided into a very rare juvenile (APS type 1) and a more common adult type with (APS 2) or without adrenal failure (APS 3). We present a patient with polyglanduler syndrome type 3a.

Case report: A 39-years-old female patient was presented to our emergency department with the complaints of nausea, vomiting and urinary incontinence. She was consulted to our endocrinology clinic with the diagnosis of diabetic ketoacidosis. In her physical examination, webbed neck, shortness of height (145 cm), low hairline at the back of the neck, exophthalmos and genu valgum was determined. In her past medical history, she had received intermittent hormone replacement therapy because of primary amenorrhea and she had been diagnosed with diabetes mellitus 2 years ago and she was using oral antidiabetic drugs also added insulin treatment for the last 6 months. The patient was admitted to our clinic and underwent insülin infusion and fluid replacement therapy.Laboratory testresults as follows glucose: 114 mg/dl, HBA1C: 11,8%, TSH >0.005 uIU/ml, FT4: 0.98 ng/dl, FT3: 4.8 pg/ml, anti-TPO: 600 IU/ml, anti TG: 341.2 IU/ml (0–115), FSH: 36 mIU/ml, LH: 15 mIU/ml, E2: <5 pg/ml, Cortisol: 18.96 ug/dl. The patient’s anti GAD and ICA antibodies were positive and the patient was diagnosed as Type 1 diabetes mellitus. The intensive insülin treatment was started after resolution of diabetic ketoacidosis. The thyroid stimulated antibody was positive and the scintigraphy and ultrasound was consistent with the graves. The genetic test for hypergonadotropic hypogonadism was consistent with Turner’s syndrom(46X DEL (X) Q13). ANA, celiac antibody, anti-parietal antibody and the other autoimmun antibodies which screening were negatively determined.

Discussion: The third type has been described in adults that, contrary to types 1 and 2, does not involve the adrenal cortex. No clinical differences between types 2 and 3 have been described except the absence of adrenal failure. APS-3A includes autoimmune endocrine diseases autoimmune thyroid disease, type 1 diabetes mellitus, lymphocytic hypophysitis, premature ovarian failure, and Hirata’s disease. It is concluded that patients with a single autoimmune component of polyendocrine syndrome should be screened to exclude other autoimmune endocrine disorders.