Mixed connective tissue disease in pregnancy: A case series and systematic literature review

Objective To investigate the impact of medical and obstetric complications associated with mixed connective tissue disease (MCTD) in pregnancy. Method We analyzed 68 pregnancies from a systematic literature review and 12 pregnancies affected by MCTD at our centre between 1986 and 2015 for medical and obstetric complications. Results During pregnancy 37.1% had active MCTD and 26.7% had relapsed. Maternal complications included caesarean section (31.1%, n = 19), preeclampsia (17.6%, n = 13), thromboembolism events, and death (2.5%, n = 2 for each). Fetal complications included prematurity (48.1%, n = 25), intrauterine growth restriction (38.3%, n = 19), and neonatal lupus (28.6%, n = 18, including chondrodysplasia punctata). More than half ( n = 10) of the neonatal lupus cases were explained by anti-U1RNP only. The perinatal mortality rate was 17.7% ( n = 14). Pregnant women with active disease had higher rates of prematurity (OR = 7.60; 95%CI [1.93; 29.95]) and perinatal death (OR = 16.83; 95%CI [1.90; 147.70]). Conclusion MCTD in pregnancy puts women at risk of medical and obstetric complications, and disease activity probably increases this risk.