OBJECTIVES: Maternal and fetal risk is high during pregnancy for young women with systemic lupus erythematosus. We analyzed outcome after a planned pregnancy program for these patients. METHODS: Between 1982 and 1994, 58 pregnancy were planned in 34 women with systemic lupus. Nine of them had renal and 5 central nervous system involvement; antiphospholipid syndrome was present in 8; steroid therapy was given in 24, immunosuppressors in 2 and plasma exchange in 1. At diagnosis of pregnancy, prednisone was prescribed (at least 10 mg/d), associated with aspirin in all non-symptomatic patients with antiphospholipid antibodies followed by heparin at pre partum. Heparin was used in case of antiphospholipid syndrome. Women with anti-SSA or B antibodies and no past history of congenital atrioventricular block were not given any specific treatment. RESULTS: An acute lupus flare-up occurred in 27% of the cases including 6% in post partum. The flare-up was mild in all cases and treatment had to be changed in half of the cases. There were 9 early abortions, 1 induced abortion for congenital malformation, 2 fetal deaths, 28 premature deliveries and 18 term deliveries. Cesarean section was indicated in 8 cases. Severe neonatal infection occurred in 2 premature infants and 1 other was growth retarded. Cutaneous neonatal lupus was observed in 2 infants. No atrioventricular blocks occurred. CONCLUSION: Fetal death or very premature birth were more frequent in patients with antiphospholipid syndrome. When pregnancy is planned in women with systemic lupus erythematosus, live birth rate reaches 96% after exclusion of early and therapeutic abortions. This rate is close to the rate in the general population. The high rate of premature birth is the main risk, but there were no maternal nor neonatal deaths.