Gougerot disease is an autoimmune disorder characterized by the lymphoid infiltration of salivary and lachrymal glands easily accessible favoring the diagnostic, and it could be primary or more often associated with another autoimmune disease (1).
PHPT preceded by about 1 year the Gougerot disease in only one case; the two disorders were discovered concomitantly in two cases; and PHPT diagnosis succeeded Gougerot disease in two cases.
A 54-year-old woman with a 1-year history of Gougerot disease diagnosed by (xerostomy; anti-SSa- and anti-SSb-positive, and Chisholm stage IV lymphocytic sialadenitis).
The association of PHPT with Gougerot disease is quite rare, with five such cases retrieved from the literature; all cases had hypercalcemia, four had moderate hypercalcemia, and only one patient had severe hypercalcemia, as in our case (3).
Gougerot disease, as other connective tissue disorders, is a very rare cause of hypercalcemia, with only 10 cases reported for systemic lupus erythematosus (SLE) (4, 5).
The pathogenesis involves polyclonal overactivation of B lymphocytes in connective tissue disorders such as Gougerot disease and could produce anti-PTH receptor autoantibodies that might activate the PTH receptor, leading to hypercalcemia with suppressed levels of PTH and PTH-related protein (PTH-rP) (1, 6, 7).
Endocrine symptoms are documented in up to 15% of primary Gougerot disease patients mainly due to concomitant thyroid dysfunction (9).
Two cases of a rare association of Gougerot disease with hypoparathyroidism were also reported in the literature (11, 12).
Primary hyperparathyroidism and Gougerot Disease. Eur J Rheumatol 2018; 5: 72-4.
In the present case, Gougerot disease was stable and could be safely eliminated as the main cause of hypercalcemia because PTH rate was very high and serum calcium level normalized soon after parathyroidectomy.