ECE2017 Eposter Presentations: Interdisciplinary Endocrinology Endocrine tumours and neoplasia (9 abstracts)
Polyglandular autoimmune syndrome type III b
Raquel Vaz de Castro 1 , José Maria Aragüés 2 , Florbela Ferreira 1 , Vânia Gomes 1 , Ana Wessling 1 , Lurdes Tavares 1 , Joana Ribeiro 1 , Irina Alves 1 , Afonso Camilo Fernandes 1 & Maria João Bugalho 1
1Santa Maria Hospital, Lisbon, Portugal; 2Beatriz Ângelo Hospital, Lisbon, Portugal.
: We report the case of a 57-year-old female diagnosed with Grave’s disease fifteen years before (TSH <0.001 U/ml; FT4 2.3 ng/dl; FT3 7.2 ng/dl, TSAbs 17 U/l). Persistency of hyperthyroidism despite antithyroid therapy led to total thyroidectomy three years after the diagnosis. Thereafter, thyroid function remained normal under hormonal replacement with levothyroxine. The patient had also been diagnosed with Pernicious Anemia at 47 years of age after investigation for complaints like fatigue and shortage of breath. Hemoglobin levels were 10.2 g/dl and Intrinsic Factor antibody titer was positive. She was treated with cobalamin and folic acid and hemoglobin levels became stable in the low-normal range. A year ago, the patient presented with left upper quadrant abdominal pain and asthenia, raising the suspicion of gastric disease. Specific blood tests were performed and the presence of high titers of gastric parietal cells antibodies (PCA), elevated gastrin (>1000 pg/ml; normal range: 13–115 pg/ml) and chromogranin A (936 ng/ml; normal range <102 ng/ml) confirmed the hypothesis of autoimmune gastritis. Furthermore, an elevated NSE level of 13.2 ug/l was detected. Endoscopy of the gastrointestinal tract found a pedunculated polypoid lesion in the stomach fundus which was resected. Histological results revealed a well differentiated neuroendocrine tumor (NET) with no evidence of vascular invasion or necrosis and Ki-67 index <2%; immunohistochemical studies for gastrin, somatostatin and serotonine were negative; atrophic gastritis was also documented. The association of Graves’ disease, pernicious anemia and gastrointestinal neuroendocrine tumor suggests the diagnosis of the very rare type IIIb polyglandular autoimmune syndrome. Gastric NETs associated to chronic atrophic gastritis (Type 1) are often small (<1–2 cm) multiple in 65% of cases and polypoid in 78% of cases, often G1 and with an excellent prognosis.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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