Management of pregnant patient with Sjögren syndrome. Case report and literature review

Abstract

Sjögren’s syndrome, a chronic disease which affects particularly the female gender, represents one of the most common autoimmune diseases and does not affect women of childbearing age, but is especially prevalent during perimenopause. It may be primary when only exocrine glandular dysfunctin occurs (xerophthalmia, xerostomia) or it may be secondary, when is most often associated with rheumatoid arthritis or systemic lupus erythematosus and affects the whole body. Pregnancy cases in the context of this disease are relatively rare, precisely by their incidence in women for whom fertility is not a priority anymore, at the age of 40 or 50 years old, but observing the trend of the last decades, through the implications offered by the modern society, with the increasing age when the first baby is conceived, the number of cases and associated outcome increase. The pregnancy outcome in the context of this disease is insufficiently known comparing to the one appearing in women affected by other autoimmune disorders. The most fearfull complication is represented by the fetal risk of congenital heart block. We report the case of a 29-year-old patient diagnosed with primary Sjögren’s syndrome, consistent with AECG criteria (American-European Consensus Group, 2002) prior to pregnancy, which required a multidisciplinary approach and a close cooperation between the obstetrician, rheumatologist, ophthalmologist and internal medicine physicians. During the 36th week of pregnancy she delivered by caesarean section. Both the patient and the newborn postoperative evolutions were favorable, with no evidence of fetal heart complications. Taking into account the potential maternal and fetal complications, the management of pregnant women diagnosed with Sjögren’s syndrome represents a real challenge for the obstetrician and requires an interdisciplinary approach in order to decrease the morbidity and mortality, both of the mother, as well as the fetus.