Anti-Parathyroid and Anti-Calcium Sensing Receptor Antibodies in Autoimmune Hypoparathyroidism

The parathyroid glands are an infrequent target for autoimmunity, the exception being autoimmune polyglandular syndrome type 1, in which autoimmune hypoparathyroidism is the rule. Antibodies that are directed against the parathyroid cell surface calcium-sensing receptor (CaSR) have recently been recognized to be present in the serum of patients with autoimmune hypoparathyroidism. In some individuals, these anti-CaSR antibodies have also been shown to produce functional activation of the receptor, suggesting a direct pathogenic role in hypocalcemia. Additionally, a few hypercalcemic patients with autoimmune hypocalciuric hypercalcemia owing to anti-CaSR antibodies that inhibit receptor activation have now been identified. Other novel parathyroid autoantigens are starting to be elucidated, suggesting that new approaches to treatment, such as CaSR antagonists or agonists (calcilytics/calcimimetics), may be worthwhile.