Department of Rheumatology, Peking Union Medical College Hospital (PUMCH), Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Rheumatism Data Center (CRDC), Chinese SLE Treatment and Research Group (CSTAR), State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
Lupus Sci Med. 2022 Mar;9(1). doi: 10.1136/lupus-2021-000636.
This study aimed to investigate the clinical characteristics and outcomes of pregnancy complicated by SLE-associated pulmonary arterial hypertension (SLE-PAH) in a case series and literature review.
This single-centre retrospective study included 10 consecutive pregnancies complicated by SLE-PAH confirmed by right heart catheterisation (RHC) at Peking Union Medical College Hospital between 2009 and 2020. A literature search was conducted and 14 pregnancy cases complicated by SLE-PAH were reviewed.
At the time of 10 patients' initial visits, the average age was 30.00±5.72 years and the median disease duration of SLE and PAH was 34.5 (range 1-164) months and 2 (1-51) months. Two patients carried planned pregnancy, seven patients developed PAH during pregnancy and one pregnancy was unplanned. Further, nine patients had low disease activity, with Systemic Lupus Erythematosus Disease Activity Index between 0 and 4, and 30%, 30% and 40% of patients were of WHO functional class II, III and IV, respectively. All patients were evaluated by RHC and echocardiography. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels were elevated in 70% of patients, with a median level of 776 (56-18 023) pg/mL. The median time of completed pregnancies in all patients was 31 (15-38) weeks and six patients delivered live infants. SLE activity and PAH severity improved in 70% of patients within 6 months after delivery. One patient died on the 15th day after induction of labour. In the remaining patients, all achieved a lupus low disease activity state; according to the European Society of Cardiology/European Respiratory Society risk stratification, seven were categorised at a lower risk state compared with their risk stratification during pregnancy, and two remained at intermediate risk. Additionally, 80% of patients exhibited mild impairments with WHO functional class I or II. The median NT-proBNP level was 184 (32-4003) pg/mL within 6 months after delivery. In the reviewed literature, the average age of patients was 30.09±5.37 years. The median time of completed pregnancies was 36 (28-40) weeks. More cases were planned and successful, and the survival rates of mothers and neonates were 85.71% and 92.86%, respectively.
Successful pregnancy could be possible in women with SLE-PAH if SLE-PAH treatment goals are achieved under proper therapies, careful monitoring and thorough evaluations.
本研究通过病例系列和文献回顾，旨在探讨经右心导管检查（RHC）确诊的系统性红斑狼疮（SLE）相关肺动脉高压（SLE-PAH）合并妊娠的临床特征和结局。
本单中心回顾性研究纳入了 2009 年至 2020 年期间在北京协和医院因 SLE-PAH 行 RHC 确诊的 10 例连续妊娠患者。同时进行了文献检索，共纳入了 14 例 SLE-PAH 合并妊娠的病例。
10 例患者首次就诊时的平均年龄为 30.00±5.72 岁，SLE 和 PAH 的中位病程分别为 34.5（1-164）个月和 2（1-51）个月。2 例患者为计划性妊娠，7 例患者在妊娠期间发生 PAH，1 例妊娠为非计划性。进一步的，9 例患者的疾病活动度较低，SLE 疾病活动指数（SLEDAI）为 0-4，30%、30%和 40%的患者分别处于世界卫生组织（WHO）功能分级 II、III 和 IV 级。所有患者均接受了 RHC 和超声心动图检查。70%的患者 N 端脑利钠肽前体（NT-proBNP）水平升高，中位数为 776（56-18023）pg/ml。所有患者中，中位完成妊娠时间为 31（15-38）周，6 例患者分娩出活婴。分娩后 6 个月内，70%的患者 SLE 活动度和 PAH 严重程度改善。1 例患者在诱导分娩后第 15 天死亡。在其余患者中，所有患者均达到狼疮低疾病活动状态；根据欧洲心脏病学会/欧洲呼吸学会（ESC/ERS）风险分层，与妊娠时相比，7 例患者的风险分层降低，2 例患者仍处于中危。此外，80%的患者的 WHO 功能分级为 I 级或 II 级，有轻度损伤。分娩后 6 个月内，NT-proBNP 中位数为 184（32-4003）pg/ml。在文献回顾中，患者的平均年龄为 30.09±5.37 岁，中位完成妊娠时间为 36（28-40）周。更多的妊娠为计划性且成功，母亲和新生儿的存活率分别为 85.71%和 92.86%。
如果在适当的治疗、仔细的监测和全面的评估下，使 SLE-PAH 治疗目标得以实现，那么女性仍有可能成功妊娠。
