Gynecologic manifestations of Sjögren's syndrome.

OBJECTIVE Sjögren's syndrome is a chronic, inflammatory autoimmune disease in which the salivary and lacrimal glands are progressively destroyed by lymphocytes and plasma cells. Because women are affected 10 times more often than men, we studied gynecologic manifestations of Sjögren's syndrome. STUDY DESIGN One thousand questionnaires were sent to women with Sjögren's syndrome in New York, New Jersey, Connecticut, and Pennsylvania. Five hundred thirty-nine women responded. RESULTS Women with Sjögren's syndrome reported significant vaginal dryness. There was no relationship of Sjögren's syndrome to either the incidence of infertility or miscarriage, although the 4% incidence of congenital anomalies in offspring was relatively high. Of the congenital anomalies, nine of 19 (47%) were cardiac. A long menstrual cycle (> 35 days) was associated with infertility and neuropathy. CONCLUSIONS The vaginal dryness in women with Sjögren's syndrome is not surprising, because the nasal and esophageal mucosae are also dry in this disorder. The relationship of infertility to a long menstrual cycle may simply indicate the presence of ovulatory dysfunction or inadequate luteal phase unrelated to Sjögren's syndrome. The relationship of neuropathy to a long menstrual cycle may be related to repeated, prolonged estrogen or progesterone exposure during the long cycles or to involvement of hypothalamic-pituitary-ovarian function.