Endocrine Abstracts

Introduction: Autoimmune Polyglandular Syndrome (APS) is a very rare endocrinopaty, characterized by the coexistence of at least two glandular autoimmune mediated diseases. In this case a patient presented with hyperpigmentation of the whole body, significant weight loss for last 6 months.

Case: A-49 year old male were refered to our deparment with loss of appetite, fatigue, nausea, weight loss and with marked buccal pigmentation. Blood pressure was 80/50 mmHg. Biochemistry revealed fasting glucose:95 mg/dl, Na:127 mmol/l, K:5.35 mmol/l, Hb:16.6. CT abdomen showed normal adrenal gland morphology with no local masses or infectious pathology. Pituitary MRI demonstrated enlarged heterogeneous gland with 9 mm height, probably due to high ACTH levels. All findings were consistent with Addison disease, which was confirmed with morning cortisole of 2.44 mg/dl (normal range 6–22), ACTH: 1250 pg/ml (0–46).

Once Addison’s disease had been confirmed, other autoimmune diseases regarding APS were undertaken. Subclinic hypothyroidism with automimmune thyroiditis; TSH:8 (0.4–5.3), fT₄: 0.9 ng/dl (0.6–1.25), anti-TPO:1016 IU/ml (0–9) was detected. Despite his normoglycemia; C-peptit:1.69 ng/ml, fasting insulin:22.3 uIU/ml, A1c:5.6%, he had high anti-GAD and anti-islet cell antibodies, widely used as a diagnostic and predictive tool for type 1 diabetes mellitus, indicating that he may develop type 1 diabetes mellitus in future.

After a week of steroid replacement his physical discomforts were alleviated with improved electrolytes.

Discussion and Conclusion: Herein, we reported an uncommon case, who had complete two-glandular deficiency with third component; predicted type 1 diabetes. This patient is being monitored closely on glycaemic state. Therefore, once an autoimmune disease is identified, the early screening for antibodies against associated disease such as anti-TPO, GADA, ICA, and antibodies to 21-hydroxylase is crucial, which facilitate the diagnosis of further disorder at an early stage.