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      Pregnancy-Related Systemic Lupus Erythematosus: Clinical Features, Outcome and Risk Factors of Disease Flares — A Case Control Study

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          Abstract

          Objective

          To investigate the clinical features, outcome, and risk factors of disease flares in patients with pregnancy-related lupus (PRL).

          Methods

          Medical charts of 155 consecutive PRL inpatients were systematically reviewed, including demographic data, clinical features, laboratory findings, treatment, complications, and outcome.

          Results

          PRL cases were divided into active (a-PRL) (n = 82, 53.0%) and stable lupus (s-PRL) (n = 73, 47.0%). Compared with nonpregnant active female systemic lupus erythematosus (SLE) patients, a-PRL including new-onset lupus (n-PRL) and flare lupus (f-PRL) (n = 41 respectively), had a higher incidence of renal and hematological involvement but less mucocutaneous and musculoskeletal involvement (p<0.05). The incidence of preeclampsia/eclampsia, fetal loss, and preterm birth were significantly higher in a-PRL than in s-PRL (p<0.05). Despite receiving a more vigorous glucocorticoid treatment, a-PRL mothers had a poorer prognosis (p<0.001). Five (6.1%) of them died and 13 (15.9%) developed severe irreversible organ failure, whereas none of these events was observed in the s-PRL group. Multivariate logistic analysis indicated that a history of lupus flares and serological activity (hypocomplementemia and/or anti-dsDNA positivity) at the time of conception were associated with lupus flares in PRL mothers.

          Conclusions

          SLE patients with a flare history and serological activity at the time of conception were at an increased risk of disease flares during pregnancy and puerperium. a-PRL patients were more prone to renal and hematological involvement, pregnancy complications, and a poorer prognosis despite more vigorous glucocorticoid treatment.

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          Most cited references 15

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          Decrease in pregnancy loss rates in patients with systemic lupus erythematosus over a 40-year period.

           A Spitzer,  C Laskin,  Kaitlin C Clark (2005)
          To determine if there has been a statistically significant change in pregnancy loss and preterm delivery rates in patients with systemic lupus erythematosus (SLE). We analyzed the pregnancy outcomes of our SLE patients over the past 3 years and reviewed the literature over the past 40 years. We extracted pregnancy loss and preterm delivery data from reports of postdiagnosis SLE pregnancies. Studies were grouped into 5-year periods and weighted according to sample size. Group means, calculated for each study period, were plotted using linear regression to determine significance, and compared with population norms for the same periods. The rate of loss in SLE pregnancies over the past 40 years decreased from a mean of 43% in 1960-1965 to 17% in 2000-2003 (r2 = 0.648). This approximates the pregnancy loss rate in the general US population. Preterm deliveries were not uniformly reported and were rarely stratified into spontaneous or physician-initiated. Prior to 1980, it was not possible to derive group means for each time period. From 1980 to 2002, however, there was a trend toward a decrease in preterm births in SLE pregnancies, although they continue to be more frequent in SLE than in the general population. Improvements in disease management and perinatal monitoring have resulted in a significant decrease in pregnancy loss in SLE over the last 40 years and a trend toward decreased preterm deliveries over the last 20 years in comparison to the general population. These advances highlight the importance of collaboration between rheumatologists and perinatologists. Given these data, the description of SLE-associated pregnancy could be revised to reflect a more positive prognosis for mother and fetus.
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            Imitators of severe preeclampsia.

             B Sibai (2007)
            There are several obstetric, medical, and surgical disorders that share many of the clinical and laboratory findings of patients with severe preeclampsia-hemolysis, elevated liver enzymes, and low platelets syndrome. Imitators of severe preeclampsia-hemolysis, elevated liver enzymes, and low platelets syndrome are life-threatening emergencies that can develop during pregnancy or in the postpartum period. These conditions are associated with high maternal mortality, and survivors may face long-term sequelae. Perinatal mortality and morbidity also remain high in many of these conditions. The pathophysiologic abnormalities in many of these disorders include thrombotic microangiopathy, thrombocytopenia, and hemolytic anemia. Some of these disorders include acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and acute exacerbation of systemic lupus erythematosus. Because of the rarity of these conditions during pregnancy and postpartum, the available literature includes only case reports and case series describing these syndromes. Consequently, there are no systematic reviews or randomized trials on these subjects. Differential diagnosis may be difficult due to the overlap of several clinical and laboratory findings of these syndromes. It is important that the clinician make the accurate diagnosis when possible because the management and complications from these syndromes may be different. For example, severe preeclampsia and acute fatty liver of pregnancy are treated by delivery, whereas it is possible to continue pregnancy in those with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and exacerbation of systemic lupus erythematosus. This review focuses on diagnosis, management, and counseling of women who develop these syndromes based on results of recent studies.
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              Predictors of maternal and fetal outcomes in pregnancies of patients with systemic lupus erythematosus.

               E Li,  Ivan Y-F Leung,  John S L Tam … (2011)
              Disease activity 6 months before pregnancy of patients with systemic lupus erythematosus (SLE) associated with adverse maternal and fetal outcomes is not well studied. The aim of the study was to identify predictors of adverse maternal and fetal outcomes in pregnant SLE patients, based on patients' background characteristics, clinical and laboratory data 6 months before pregnancy. Of 103 pregnancies, 55 pregnancies in 39 SLE patients were investigated. Clinical and laboratory data were recorded at regular intervals from 6 months before conception to 1 year after delivery. Primary outcomes included the predictors of combined adverse maternal and fetal outcomes. Potential explanatory variables included demographic, clinical and laboratory data 6 months before conception. Using logistic regression, history of nephritis (p = 0.001, odds ratio [OR] 13.3, 95% confidence interval [CI] 2.7-65.1) and a high SLE Disease Activity Index (SLEDAI) score 6 months before pregnancy (p = 0.015, OR 1.7, 95% CI 1.1-2.7) were associated with combined adverse maternal outcome, whereas flare during pregnancy (p = 0.003, OR 29.3, 95% CI 3.1-273.1) predicted combined adverse fetal outcome. The area under the curve for SLEDAI score of combined maternal outcome was 0.73 (95% CI 0.58-0.87). The optimal cut-off point according to the receiver operating characteristic curve was 4, with a sensitivity of 64% and a specificity of 75%. In conclusion, a history of nephritis or a SLEDAI score of 4 or more in SLE patients 6 months before conception predicts adverse maternal outcomes, while disease flare during pregnancy predicts adverse fetal outcomes. Pregnancies should be delayed until the disease has been in remission for 6 months.
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                Author and article information

                Contributors
                Masataka Kuwana: Role: Editor
                Journal
                Journal ID (nlm-ta): PLoS One
                Journal ID (iso-abbrev): PLoS ONE
                Journal ID (publisher-id): plos
                Journal ID (pmc): plosone
                Title: PLoS ONE
                Publisher: Public Library of Science (San Francisco, USA )
                ISSN (Electronic): 1932-6203
                Publication date Collection: 2014
                Publication date (Electronic): 13 August 2014
                Volume: 9
                Issue: 8
                Affiliations
                [1 ]Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
                [2 ]Department of Rheumatology, Dongfang Hospital, The Second Clinical Medical College of Beijing University of Chinese Medicine, Beijing, China
                Keio University School of Medicine, Japan
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Conceived and designed the experiments: XZ FZ. Performed the experiments: LZ QW XL WZ FT. Analyzed the data: HY HL DX XZ. Contributed to the writing of the manuscript: HY XZ.

                Article
                Publisher ID: PONE-D-14-12992
                DOI: 10.1371/journal.pone.0104375
                PMC ID: 4131906
                PubMed ID: 25118692
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                Page count
                Pages: 7
                Funding
                This work was partly supported by the grants from the National Natural Science Foundation of China (81325019, 81172859, and 81273312), Beijing Municipal Natural Science Foundation (7141008), the Research Special Fund for Public Welfare Industry of Health (20120217), the Capital Health Research and Development of Special (2011-4001-02), and the Youth Foundation of Peking Union Medical College Hospital (20110110). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Subject: Research Article
                Subject: Biology and Life Sciences
                Subject: Immunology
                Subject: Clinical Immunology
                Subject: Autoimmune Diseases
                Subject: Lupus Erythematosus
                Subject: Systemic Lupus Erythematosus
                Subject: Medicine and Health Sciences
                Subject: Rheumatology
                Subject: Women's Health
                Subject: Maternal Health
                Subject: Pregnancy
                Subject: Obstetrics and Gynecology
                Custom metadata
                Data Availability The authors confirm that all data underlying the findings are fully available without restriction. All relevant data are within the paper and its Supporting Information files.

                Data availability:

                ScienceOpen disciplines: Uncategorized

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