Management of Sjögren’s Syndrome During Pregnancy

There is a scarcity of data on the management of primary Sjogren’s syndrome (pSS) in pregnancy. As there is a higher risk of complications in pregnant women with pSS, including the possibility of neonatal lupus, these women should be managed by a multidisciplinary team. At the preconceptional stage itself, there is a need to ensure that the disease activity is well controlled and the patient is not on any teratogenic drugs. It is also important to verify that there is no severe organ involvement that could adversely impact maternal and fetal health. Disease activity needs to be monitored throughout pregnancy. Baseline antibody profile (anti-Ro52, anti-Ro60, and anti-La) should be performed in all women planning pregnancy and if they are seropositive, appropriate counseling about possible risks of neonatal lupus should be done without imparting undue anxiety. Weekly fetal echocardiography is done from 16th to 28th weeks of gestation and less frequently till term hoping that impending complete heart block can be detected and treated, though evidence to support this protocol is weak. Fluorinated steroids are associated with adverse effects and have not been found to have survival advantage or decrease requirement for pacing. Routine use of fluorinated steroids should be avoided and may be considered only in certain scenarios such as recent-onset incomplete heart block and in case of myocardial involvement/hydrops. Hydroxychloroquine protects against occurrence of cardiac and cutaneous lupus and hence is a potential preventive therapy.