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

Abstract

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.

Keywords: Mixed connective tissue disease; Sharp syndrome; U1RNP antibody; chondrodysplasia punctata; neonatal systemic lupus erythematosus; pregnancy.

Figure 1.

Case selection. W: women; P: pregnancy; Dx: diagnosis; PP: post-partum; APLS: antiphospholipid syndrome.