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Planning Key for Successful Pregnancy in Women With SLE-PAH


The report is based on a series of 10 cases and a literature review involving 14 cases.

Women with systemic lupus erythematosus–associated pulmonary arterial hypertension (SLE-PAH) can have successful pregnancies if their disease is well controlled and their pregnancy is carefully monitored, according to new study findings.

The report, published in Lupus Science & Medicine, helps to clarify the feasibility of pregnancy in this high-risk population, and it is based on a case series as well as a review of previously published literature.

PAH is a rare but very serious complication of SLE, occurring in only about 3% of cases, the authors wrote. Yet, because SLE primarily affects women of childbearing age, the development of SLE-PAH can cause a significant hardship to women hoping to have children. Those who become pregnant face a high-risk scenario, they added, including a significant risk of maternal death. However, very little research has been conducted into these cases, leaving patients with little guidance in their decision-making, the authors highlighted.

The investigators therefore sought to study pregnant women with SLE-PAH confirmed by right-heart catheterization to find out about their clinical characteristics and outcomes.

Ten pregnant women (mean [SD] age, 30 [5.72] years) with confirmed SLE-PAH sought care at Peking Union Medical College Hospital during the study period of 2009 to 2020. In addition, 14 cases from 11 studies were identified in the literature review, covering 2002 to 2011.

Of the 10 patients, 7 developed PAH during the course of pregnancy, 2 of the pregnancies were planned, and 1 pregnancy was unplanned. Nine of the 10 patients had low disease activity at the time of pregnancy. The median length of the completed pregnancies among all the participants was 31 (range, 15-38) weeks.

Four patients had their labor induced, and none of them delivered live infants. One mother who underwent induction died on the 15th day following the procedure.

The remaining 6 patients delivered live infants; 5 were cesarean deliveries and 1 was a vaginal birth. In 70% of patients, SLE activity and PAH severity improved 6 months after delivery.

In the 14 cases included in the review, the median time of the completed pregnancies was 36 (range, 28-40) weeks. One mother died of heart failure prior to delivery, and one died post partum. All 13 neonates whose mothers survived to delivery survived. In the literature review, none of the mothers underwent induced labor, and all but 3 mothers delivered via cesarean section.

The authors noted that most of the research related to PAH in pregnancy focuses on PAH caused by congenital heart conditions, and most of the patients in those studies have superior cardiac function compared with the women in the present study.

“Therefore, more attention and more intensive interventions are needed in PAH secondary to connective tissue diseases,” they wrote.

The authors noted that planning appears to play an important role in outcomes, as the only deaths in the study were in cases where the pregnancy was unplanned. Furthermore, all of the women who successfully delivered babies had SLE Disease Activity Index scores of 0 to 4 at baseline.

“Therefore, low disease activity may contribute to successful pregnancies in patients with SLE-PAH,” the investigators said.

They concluded that a careful patient-management plan, along with treat-to-target therapy, can improve the odds of successful pregnancy in SLE-PAH, athough they added that the condition still presents a considerable challenge.


Li M, Tian Z, Qian J, et al. Impact of pregnancy in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: case series and literature review. Lupus Sci Med. Published online March 7, 2022. doi:10.1136/lupus-2021-000636

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