Background: Primary Addisons disease (AD) is a rare endocrine condition, with reduced or absent secretion of adrenal hormones. Steroid replacement therapy normalizes endocrine assess and the quality of life. More than a half of patients display additional autoimmune conditions, which represent a considerable clinical concern.
Objective and hypotheses: Antiparietal cells antibodies (APC-Ab) are commonly found in patients with autoimmune Addisons disease, usually pointing to autoimmune atrophic gastritis and pernicious anemia.
Method: A 12-year-old girl, with a diagnosis of Addison disease made 1.6 years previously (anti-adrenal Ab negative) attended her routine clinic appointment. She had a history of good adherence. Regular medications were: hydrocortisone 10 mg morning, 5 mg afternoon and evening, fludrocortisone 100 μg morning and 50 μg evening. On examination she presented with increasing darkening of the skin and easy fatigue. Investigations showed: plasma cortisol h. 8: in the normal range (29 μg/dl); raised ACTH levels (2976 pmol/ml). PTH, TSH, fT3, fT4, anti-TG and anti-TPO antibodies were within the normal range. She presented anemia with the following parameters: Hb 11 g/dl MCV 74fL, ferritin 18 ng/ml. Autoimmunity tests showed APC 1:640. She is waiting for gastroscopy.
Results: It was therefore necessary to increase the corticosteroid replacement therapy in order to obtain an appropriate reward: Hydrocortisone 10 mg three times per day, fludrocortisone 100 μg morning and 50 μg evening. After 1 and 3 months examinations she showed a progressive reduction of ACTH levels (ACTH 127 pg/ml cortisol 16.9 μg/dl).
Conclusion: Autoimmune atrophic gastritis (AAG) is common in patient with AD and may coexist with various hematological presentations. Iron deficiency is often associated with AAG. Addisons patients with coexisting elevated gastric APC-Ab are at risk of enterochromaffin-like cells hyper/dysplasia. In case of rapid onset of anemia and/or iron deficiency we should exclude the presence of gastric adeno-carcinoma or gastric carcinoid. Probably in our patient the concomitant atrophic gastritis, suggested by the appearance of AAG, has been the trigger that required an increased hydrocortisone dose.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology