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

To investigate the impact of medical and obstetric complications associated with mixed connective tissue disease (MCTD) in pregnancy. 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. 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]). MCTD in pregnancy puts women at risk of medical and obstetric complications, and disease activity probably increases this risk