Endocrine Abstracts

The polyglandular autoimmune syndromes (PAS) are rare disorders characterized as multiple autoimmune-mediated organ failure. PAS III includes chronic autoimmune thyroiditis with type 1 diabetes mellitus or pernicious anemia and/or vitiligo or alopecia and many other organ-specific autoimmune diseases, but never involves adrenal cortex. Several patients with PAS III and associated type 1 gastric neuroendocrine tumor (NET) have already been reported. We report probably the first case of PAS III, type 1 gastric NET and concomitant secondary autonomic failure as the main clinical manifestation. A 65-years-old woman with severe postural and postprandial orthostatic hypotension admitted to the hospital. The orthostasis has been debilitating and gradually has worsened in the past year. She has lost about 25 kg due to postprandial sickness and consequent dietary restrictions. The patient has a history of mild type 2 diabetes mellitus. She has been treated with metformin and her HbA1c has been < 6% during the last 8 years. Diabetes has resolved due to weight loss and the patient has had a good glycemic control without any treatment for the past year. The patient has a history of primary hypothyroidism due to chronic autoimmune thyroiditis. Besides that she has suffered from hair loss with global alopecia over the last decades of life. Initial B12 vitamin deficiency without anemia has been detected. Upper endoscopy was performed, showing multiple gastric polyps. Histological examination revealed chronic atrophic gastritis, hyperplasia of endocrine cells and gastric NET G1, Ki-67=1%. Numerous gastric polyps have been removed during endoscopy. The patient has been treated with intramuscular B12 vitamin injections with a gradual positive effect on the autonomic dysfunction. Annual endoscopic follow-up and lanreotide injections are recommended to prevent progression and dissemination of the gastric NET.