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Objective The objective is to study the course and outcome of fetuses with congenital atrioventricular block (AVB) in a single centre. Methods Retrospective analysis of cases diagnosed prenatally with second and third degree AVB. The clinical characteristics and outcome of fetal AVB were evaluated including in utero treatment. Results Sixty-two cases were studied. AVB was associated with a congenital heart defect (CHD-AVB) in 17 cases (27%), whereas it was isolated (i-AVB) in 45 (73%), 42 of which were associated with maternal antibodies. There were nine (52.9%) live births in the CHD-AVB group, five of which (55%) resulted in infant deaths. In the i-AVB group, there were 40/45 (88.9%) live births and 1/40 (2.5%) infant death; 36 (90%) babies required a permanent pacemaker. The only factor predictive of postnatal death was the presence of CHD (5/9 vs 1/39 or 48.7 [3.6; 1457.7], p < 0.001). Nineteen fetuses (40.5%) with i-AVB received steroids in utero. No difference in outcome was found between the AVB treated in utero versus the no-treatment group in terms of permanent pacemaker placement, postnatal death or development of dilated cardiomyopathy. Conclusion The most important prognostic factor for congenital AVB is the association with CHD. In utero treatment remains questionable. © 2014 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objectives The aim of this study was to investigate whether the quadrivalent human papillomavirus ( HPV) vaccine Gardasil is associated with a change in the risk of autoimmune disorders ( ADs) in young female subjects. Design Systematic case-control study of incident ADs associated with quadrivalent HPV vaccination in young women across France. Participants and setting A total of 113 specialised centres recruited (from December 2007 to April 2011) females aged 14-26 years with incident cases of six types of ADs: idiopathic thrombocytopenic purpura ( ITP), central demyelination/multiple sclerosis ( MS), Guillain-Barré syndrome, connective tissue disorders (systemic lupus erythematosus, rheumatoid arthritis/juvenile arthritis), type 1 diabetes mellitus and autoimmune thyroiditis. Control subjects matched to cases were recruited from general practice. Analysis Multivariate conditional logistic regression analysis; factors included age, geographical origin, smoking, alcohol consumption, use of oral contraceptive(s) or vaccine(s) other than Gardasil received within 24 months before the index date and personal/family history of ADs. Results Overall, 211 definite cases of ADs were matched to 875 controls. The adjusted odds ratio ( OR) for any quadrivalent HPV vaccine use was 0.9 [95% confidence interval ( CI) 0.5-1.5]. The individual ORs were 1.0 (95% CI 0.4-2.6) for ITP, 0.3 (95% CI 0.1-0.9) for MS, 0.8 (95% CI 0.3-2.4) for connective disorders and 1.2 (95% CI 0.4-3.6) for type 1 diabetes. No exposure to HPV vaccine was observed in cases with either Guillain-Barré syndrome or thyroiditis. Conclusions No evidence of an increase in the risk of the studied ADs was observable following vaccination with Gardasil within the time periods studied. There was insufficient statistical power to allow conclusions to be drawn regarding individual ADs. [ABSTRACT FROM AUTHOR]

OBJECTIVE: (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) scanning has been proposed as a new way of assessing disease activity in Takayasu arteritis (TA), but previous studies have used the nonvalidated National Institutes of Health (NIH) global activity criteria, and thus might be biased. This study was undertaken to determine the value of PET scanning for assessment of disease activity in TA, by comparing PET scan data with clinical, biologic, and magnetic resonance imaging (MRI) data assessed separately. METHODS: Twenty-eight patients with TA (according to the American College of Rheumatology criteria) underwent a total of 40 PET scans. Images were reviewed by 2 pairs of independent nuclear medicine physicians and assessed for pattern and intensity of vascular uptake. TA activity data were obtained within 15 days of the PET scans. RESULTS: PET scanning revealed abnormal vascular uptake in 47% of the 40 examinations. The uptake intensity grade was 0 in 7 scans, grade 1 in 7 scans, grade 2 in 13 scans, and grade 3 in 13 scans. Morphologic analysis was conducted by grading the pattern of the vascular uptake as diffuse (73%), segmental (20%), or focal (13%). There was a trend toward an association between clinically active disease and the semiquantitative assessment of FDG uptake (P = 0.08). We found no statistical association between levels of acute-phase reactants and intensity of uptake. There was no significant association between the semiquantitative assessment of FDG uptake and the presence of vascular wall thickening (P = 0.23), gadolinium uptake (P = 0.73), or the presence of vascular wall edema (P = 0.56). CONCLUSION: Our findings indicate that there is no association between FDG vascular uptake intensity and clinical, biologic, or MRI assessment of disease activity. Previous studies using the nonvalidated NIH global activity criteria are likely biased. [ABSTRACT FROM AUTHOR]

Enoxaparin is frequently prescribed for pregnant women who are at high risk for thromboembolic complications. We conducted a population pharmacokinetics study with 75 pregnant women and 38 nonpregnant women as controls to evaluate enoxaparin pharmacokinetics during pregnancy and the postpartum period. Clearance of the drug was higher in the pregnant women throughout pregnancy when compared with nonpregnant women (0.78 ± 0.03 l/h vs. 0.52 ± 0.03 l/h, respectively P < 0.001) with the stage of the pregnancy having no influence. The volume of distribution was influenced by stage of the pregnancy, characterized by a two-step increase, with an initial rise paralleling the woman's increase in body weight during the first two trimesters, followed by an additional increase of 41% during the last 2 months of pregnancy, independent of changes in weight. Using enoxaparin pharmacokinetic parameters to simulate anti-Xa time profiles, we observed that the maintenance of the same doses throughout pregnancy resulted in a progressive reduction in mean and peak anti-Xa activities. We recommend the administration of doses normalized for body weight changes so as to counteract enoxaparin pharmacokinetic changes that accompany various stages of pregnancy.Clinical Pharmacology & Therapeutics (2008); 84, 3, 370–377 doi:10.1038/clpt.2008.73 [ABSTRACT FROM AUTHOR]

OBJECTIVE: To study the possible relationship between whole-blood hydroxychloroquine (HCQ) concentrations and clinical efficacy of HCQ in patients with systemic lupus erythematosus (SLE). METHODS: Whole-blood HCQ concentrations were measured, under blinded conditions, in 143 unselected patients with SLE who had been receiving HCQ 400 mg daily for at least 6 months. The relationship of these concentrations to current disease activity and to subsequent exacerbations during 6 months of followup was investigated. RESULTS: At baseline, 23 patients had active disease (mean +/- SD SLE Disease Activity Index 12.4 +/- 7.5). The mean whole-blood HCQ concentration in this group was significantly lower than that in the 120 patients with inactive disease (694 +/- 448 ng/ml versus 1,079 +/- 526 ng/ml; P = 0.001). Among the 120 patients who had inactive disease at baseline, the mean HCQ concentration at baseline in the 14 (12%) who had disease exacerbations during followup was significantly lower than that in the patients whose disease remained inactive. Multivariate logistic regression showed that the HCQ concentration was the only predictor of exacerbation (odds ratio 0.4 [95% confidence interval 0.18-0.85], P = 0.01). Receiver operating characteristic curve analysis showed that a whole-blood HCQ concentration cutoff of 1,000 ng/ml had a negative predictive value of 96% for exacerbation during followup. CONCLUSION: Low whole-blood HCQ concentrations are associated with SLE disease activity and are a strong predictor of disease exacerbation. Regular drug assaying and individual tailoring of treatment might help to improve the efficacy of HCQ treatment in patients with SLE. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Aside from congenital heart block (CHB), sinus bradycardia and prolongation of the corrected QT (QTc) interval have been reported in infants born to mothers with anti-SSA antibodies. To assess the pathologic nature of these manifestations, this study focused on electrocardiographic (EKG) variations in these children, comparing them with findings in a control group. METHODS: We studied 165 consecutive pregnancies in 106 anti-SSA-positive women with connective tissue diseases (CTDs). EKGs obtained on 58 children of this group were compared with those obtained on 85 infants born to mothers with CTD who were negative for both anti-SSA and anti-SSB. RESULTS: No statistically significant difference was seen between the 2 study groups with regard to gestational age, prematurity, birth weight, age of the children at the time of EKG, age of the mothers, or treatments received by the mothers during their pregnancies. Seven of 137 children developed cutaneous neonatal lupus syndrome; 1 child developed CHB (CHB risk of 1 in 99 [1%] if only the first prospectively observed pregnancy in women without a history of CHB is included in the analysis). For EKGs recorded during the first 2 months of life, the mean +/- SD PR interval was 96 +/- 16 msec in the anti-SSA-positive group and 96 +/- 13 msec in the anti-SSA-negative group (P = 0.84), with mean QTc values of 397 +/- 27 and 395 +/- 25 msec (P = 0.57) and mean heart rates of 141 +/- 23 and 137 +/- 21 beats per minute (P = 0.20), respectively. No difference in the PR interval, QTc interval, or heart rate was observed for EKGs obtained between 2 and 4 months of life. When EKGs obtained at 0-2 months were compared with those obtained at 2-4 months, a physiologic prolongation of the QTc interval was observed in both study groups. No sudden infant death or symptomatic arrhythmia occurred during the first year of life. CONCLUSION: The EKG findings in children of anti-SSA-positive and anti-SSA-negative mothers were not significantly different. Our results suggest that the prolongation of the QTc interval and sinus bradycardia that have recently been reported in children of mothers with anti-SSA antibodies occur independently of the anti-SSA antibodies. The pathologic nature of these EKG variations was not confirmed by our controlled study. Copyright 2004 American College of Rheumatology [ABSTRACT FROM AUTHOR]

Arterial thrombotic events, thrombocytopenia, and hemolytic anemia with schistocytes may be encountered in the setting of both thrombotic thrombocytopenic purpura (TTP) and primary antiphospholipid syndrome (APS). We report 2 cases of TTP occurring in patients with definite primary APS. We also describe the results of tests for ADAMTS-13 activity in 20 consecutive patients with primary APS, as well as tests for antiphospholipid antibodies in 26 patients who had TTP, severe ADAMTS-13 deficiency, and ADAMTS-13-inhibiting antibodies. In both of the patients with primary APS and TTP, ADAMTS-13 activity was undetectable, and ADAMTS-13-inhibiting antibodies were present. None of the 26 patients with TTP and severe ADAMTS-13 deficiency was positive for the lupus anticoagulant. One of these patients had a low level of anticardiolipin antibodies (22 IgG phospholipid units). In the 20 patients with primary APS, mean ADAMTS-13 activity was 116% (range 44-250%), and no severe deficiency (<5%) was observed. Our findings suggest that primary APS must be added to the list of autoimmune disorders that can be complicated by TTP. [ABSTRACT FROM AUTHOR]

An abstract for the article "Can hydroxychloroquine ( HCQ) reduce the risk of recurrent fetal heart block?" by J.A. Copel, P. Izminly, N. Costedoat-Chalumeau and others is presented.

Although splenectomy is still considered the most effective curative treatment for immune thrombocytopenia (ITP), its use has significantly declined in the last decade, especially since the approval of thrombopoietin receptor agonists (TPO-RAs). The main objective of the study was to determine whether splenectomy was still as effective nowadays, particularly for patients with failure to respond to TPO-RAs. Our secondary objective was to assess, among patients who relapsed after splenectomy, the pattern of response to treatments used before splenectomy. This multicenter retrospective study involved adults who underwent splenectomy for ITP in France from 2011 to 2020. Response status was defined according to international criteria. We included 185 patients, 100 (54.1%) and 135 (73.0%) patients had received TPO-RAs and/or rituximab before the splenectomy. The median follow-up after splenectomy was 39.2 months [16.5-63.0]. Overall, 144 (77.8%) patients had an initial response and 23 (12.4%) experienced relapse during follow-up, for an overall sustained response of 65.4%, similar to that observed in the pre-TPO-RA era. Among patients who received at least one TPO-RA or rituximab before splenectomy, 92/151 (60.9%) had a sustained response. Six of 13 (46%) patients with previous lack of response to both TPO-RAs and rituximab had a sustained response to splenectomy. Among patients with relapse after splenectomy, 13/21 (61.2%) patients responded to one TPO-RAs that failed before splenectomy. In conclusion, splenectomy is still a relevant option for treating adult primary ITP not responding to TPO-RAs and rituximab. Patients with lack of response or with relapse after splenectomy should be re-challenged with TPO-RAs., (© 2021 Wiley Periodicals LLC.)

The aim of the study was to assess the efficacy and safety of rituximab (RTX) for treating systemic lupus erythematosus (SLE)-associated immune cytopenias. This multicenter retrospective cohort study of adults from French referral centers and networks for adult immune cytopenias and SLE involved patients ≥18 years old with a definite diagnosis of SLE treated with RTX specifically for SLE-associated immune cytopenia from 2005 to 2015. Response assessment was based on standard definitions. In total, 71 patients, 61 women (85.9%), with median age 36 years [interquartile range 31-48], were included. The median duration of SLE at the time of the first RTX administration was 6.1 years [2.6-11.6] and the reason for using RTX was immune thrombocytopenia (ITP) for 44 patients (62.0%), autoimmune hemolytic anemia (AIHA) for 16 (22.5%), Evans syndrome for 10 (14.1%), and pure red cell aplasia for one patient. Before receiving RTX, patients had received a mean of 3.1 ± 1.3 treatments that included corticosteroids (100%), and hydroxychloroquine (88.5%). The overall initial response rate to RTX was 86% (91% with ITP, 87.5% with AIHA, and 60% with Evans syndrome), including 60.5% with complete response. Median follow-up after the first injection of RTX was 26.4 months [14.3-71.2]. Among 61 initial responders, relapse occurred in 24 (39.3%); for 18, RTX retreatment was successful in 16 (88.8%). Severe infections occurred after RTX in three patients, with no fatal outcome. No cases of RTX-induced neutropenia were observed. In conclusion, RTX seems effective and relatively safe for treating SLE-associated immune cytopenias., (© 2017 Wiley Periodicals, Inc.)

Objective: To describe the interplay between Behçet's disease (BD) and pregnancy., Methods: This retrospective study included 76 pregnancies in 46 patients fulfilling the international criteria for BD. The median age of the patients at the time of entry into the study was 28.4 years (interquartile range 22.8-30.9 years). Patients were used as their own historical controls to assess the incidence of BD flares during pregnancy and before or after pregnancy. Factors associated with the occurrence of complications during pregnancy were assessed., Results: Among the 76 pregnancies with BD analyzed, 27 (35.5%) were associated with worsening of the symptoms of BD flare; oral and genital ulcerations (78.4% and 67.6%, respectively) as well as ocular complications (32.4%) were the most frequent. The mean ± SD annual rates of BD flares were 0.49 ± 0.72 during pregnancy and 1.46 ± 2.42 during the nonobstetric period (P = 0.018). The proportion of BD flares tended to be lower in patients treated with colchicine (27.9% versus 45.4% of patients not treated with colchicine; P = 0.11). The overall rate of complications during pregnancy was 15.8%. The complications included miscarriage (5 patients), cesarean delivery (3 patients), medical termination of pregnancy (2 patients), hemolysis, elevated liver enzymes, and low platelets syndrome (1 patient), and immune thrombocytopenia (1 patient). There was a statistically significant association between a history of deep vein thrombosis in BD and the risk of obstetric complications (odds ratio 7.25, 95% confidence interval 1.21-43.46, P = 0.029). Neither gestational age at delivery nor neonatal outcome was influenced by BD., Conclusion: The disease course in BD seems to improve during pregnancy, mostly in patients who are treated with colchicine. Pregnancy in patients with BD appears not to be associated with an increased rate of pregnancy-related complications., (Copyright © 2013 by the American College of Rheumatology.)

Objective: Giant cell arteritis (GCA) is a large-vessel vasculitis of unknown origin. Recent findings indicate that at least 2 separate lineages of CD4+ T cells, Th1 and Th17 cells, participate in vascular inflammation. The pathways driving these T cell differentiations are incompletely understood, but may provide novel therapeutic targets. This study was undertaken to identify cytokines involved in the pathogenesis of GCA., Methods: Thirty GCA patients fulfilling the American College of Rheumatology criteria, with active disease or disease in remission, and 30 age-matched controls were included. Levels of 27 cytokines were determined in culture supernatants, and flow cytometric analysis of peripheral blood mononuclear cells (PBMCs) and immunohistochemical analysis of temporal artery samples were performed., Results: Multiparametric analysis of cytokines produced by PBMCs associated with GCA disease activity identified a signature involving interleukin-2 receptor (IL-2R), IL-12, interferon-γ (IFNγ), IL-17A, IL-21, and granulocyte-macrophage colony-stimulating factor (GM-CSF). An expansion of Th1 and Th17 cells and a decrease in Treg cells were observed in the peripheral blood of patients with active GCA. An expansion of IL-21-producing CD4+ T cells was also observed in patients with active GCA and correlated positively with Th17 and Th1 cell expansion. Immunohistochemical analysis revealed IFNγ, IL-17A, and IL-21 expression within inflammatory infiltrates. Stimulation of purified CD4+ T cells with IL-21 increased Th1 and Th17 cell frequencies and decreased FoxP3 expression. In contrast, blockade of IL-21 using IL-21R-Fc markedly decreased the production of IL-17A and IFNγ and increased FoxP3 expression., Conclusion: Our findings indicate that IL-21 plays a critical role in modulating Th1 and Th17 responses and Treg cells in GCA, and might represent a potential target for novel therapy., (Copyright © 2012 by the American College of Rheumatology.)

Objective: To assess the factors influencing the efficacy of 2 injections of a pandemic 2009 influenza A (H1N1) vaccine in patients with systemic lupus erythematosus (SLE)., Methods: We conducted a single-center, observational prospective study of 111 patients who were vaccinated with a monovalent, inactivated, nonadjuvanted, split-virus vaccine during December 2009 and January 2010 and received a second dose of vaccine 3 weeks later. The antibody response was evaluated using the hemagglutination inhibition assay according to the guidelines recommended for the pandemic vaccine, consisting of 3 immunogenicity criteria (i.e., a seroprotection rate of 70%, a seroconversion rate of 40%, and a geometric mean ratio [GMR] of 2.5)., Results: The 3 immunogenicity criteria were met on day 42 (seroprotection rate 80.0% [95% confidence interval (95% CI) 72.5-87.5%], seroconversion rate 71.8% [95% CI 63.4-80.2%], and GMR 10.3 [95% CI 2.9-14.2]), while only 2 criteria were met on day 21 (seroprotection rate 66.7% [95% CI 57.9-75.4%], seroconversion rate 60.4% [95% CI 51.3-69.5%], and GMR 8.5 [95% CI 3.2-12.0]). The vaccine was well tolerated. Disease activity, assessed by the Safety of Estrogens in Lupus Erythematosus National Assessment version of the SLE Disease Activity Index, the British Isles Lupus Assessment Group score, and the Systemic Lupus Activity Questionnaire, did not increase. In the multivariate analysis, vaccination failure was significantly associated with immunosuppressive treatment or a lymphocyte count of ≤ 1.0 × 10⁹/liter. The second injection significantly increased the immunogenicity in these subgroups, but not high enough to fulfill the seroprotection criterion in patients receiving immunosuppressive treatment., Conclusion: Our findings indicate that the efficacy of the vaccine was impaired in patients who were receiving immunosuppressive drugs or who had lymphopenia. A second injection increased vaccine immunogenicity without reaching all efficacy criteria for a pandemic vaccine in patients receiving an immunosuppressive agent. These results open possibilities for improving anti-influenza vaccination in SLE., (Copyright © 2011 by the American College of Rheumatology.)

Objective: The determinants of vessel targeting are largely unknown in vasculitides. This study was undertaken to identify patterns of vascular involvement in Takayasu arteritis (TA), using objective classification of vascular beds. We postulated that cluster analysis could unveil preferential associations between vascular beds commonly affected by TA., Methods: Peripheral vascular Doppler, computed tomography angiography, and angio-magnetic resonance imaging data from 82 patients with TA (according to the American College of Rheumatology criteria) were studied between January 1995 and May 2006. Cross-relationships of involvement between 24 main arteries were assessed using the phi correlation coefficient. Identification of patterns of vascular involvement was performed using agglomerative hierarchical cluster analysis., Results: Data were obtained from 82 patients (68 women [82.9%] and 14 men [17.1%]). The median duration of followup was 5.1 years (range 1 month to 30 years). For 16 (80%) of 20 paired arteries, the highest correlation of involvement was observed with the contralateral artery. Conversely, disease extension was contiguous in the aorta. Cluster analysis further confirmed that all paired arterial beds, except for the internal and external carotid arteries, clustered with their contralateral counterpart and that the aortic arch, the descending thoracic aorta, and the abdominal aorta clustered together., Conclusion: Our findings reveal that TA lesions mostly develop in a symmetric manner in paired vascular territories and that disease extension is contiguous in the aorta. This may prove useful for improving the radiologic followup of patients with TA and for providing a pattern for further investigations focusing on the mechanisms of vessel specificity in vasculitides., (Copyright © 2011 by the American College of Rheumatology.)

Objective: Mycophenolic acid (MPA) is the active metabolite of mycophenolate mofetil (MMF), which is widely used to treat systemic lupus erythematosus (SLE). In transplantation, MPA area under the plasma concentration-time curve from 0 to 12 hours (MPA AUC(0-12)) is correlated with clinical outcome. We undertook the present study to assess possible relationships between SLE activity and MPA AUC(0-12)., Methods: Using a Bayesian estimator, MPA AUC(0-12) was determined in 71 consecutive SLE patients (61 women and 10 men; mean +/- SD age 34 +/- 10 years) receiving a stable MMF dose. On the same day, SLE activity was assessed using the SLE Disease Activity Index (SLEDAI; active disease defined as a SLEDAI score > or = 6) and the British Isles Lupus Assessment Group (BILAG) index (active disease defined as BILAG A or B)., Results: Two groups were studied: patients with active SLE (mean +/- SD SLEDAI score 11.6 +/- 4.4; n = 26) and patients with inactive SLE (mean +/- SD SLEDAI score 1.9 +/- 1.6; n = 45). MPA AUC(0-12) correlated weakly with the dose of MMF (r = 0.33, P = 0.005). Mean +/- SD MPA AUC(0-12) in the group with active SLE was significantly lower than that in the group with inactive SLE (26.8 +/- 13.6 microg.hour/ml versus 46.5 +/- 16.3 microg.hour/ml; P < 0.0001). MPA AUC(0-12) was negatively correlated with the SLEDAI (r = -0.64, P < 0.0001). In multivariate analysis, MPA AUC(0-12) was the sole parameter associated with SLE activity (odds ratio 0.89 [95% confidence interval 0.83-0.96], P = 0.002). The MPA AUC(0-12) threshold value of 35 microg.hour/ml was associated with the lowest risk of active SLE., Conclusion: Our data show that SLE activity is strongly correlated with MPA AUC(0-12). An individualized dosing regimen of MMF, with a target AUC(0-12) of 35 microg.hour/ml, should be considered for SLE patients.

Objective: Rituximab, a chimeric monoclonal antibody directed against the CD20 antigen expressed by B cells, is now considered an effective second-line therapy in various systemic diseases. We describe here the effects of rituximab in patients with relapsing polychondritis., Methods: This was a retrospective study of 9 patients with relapsing polychondritis who received different regimens of rituximab in addition to their ongoing therapies. Clinical, laboratory, physiologic, and radiologic indicators were used to assess disease activity. We also examined their corticosteroid doses and any change in immunosuppressive agents. We then compared disease activity in the 6 months preceding rituximab administration and at 6 and 12 months after., Results: At 6 months, 2 patients showed partial improvement, 4 were stable, and 3 had worsened disease; however, no patient had complete remission. At 12 months (after exclusion of the 3 patients whose disease had worsened at 6 months), 2 patients remained stable and 4 had worsened disease; however, there were no partial or complete remissions. B cells were counted in 8 patients during the first 6 months after treatment, and B cell depletion was observed in all of the patients., Conclusion: Although we cannot rule out the possibility that rituximab had a small effect, our patients' clinical courses did not improve significantly with this treatment.

Objective: The use of hydroxychloroquine (HCQ) in pregnancy remains controversial. The recent demonstration that HCQ passes across the placenta, with cord blood concentrations nearly identical to those found in maternal blood, emphasizes the need for careful evaluation of pregnancies in women receiving HCQ. However, only small series of HCQ-treated pregnant women have been reported, and most of these studies had no control group. We now report our experience with 133 pregnancies in women being treated with HCQ, resulting in 117 live births. Results in the HCQ group are compared with those in a control group., Methods: One hundred thirty-three consecutive pregnancies in 90 women treated with 200 mg of HCQ either twice daily (122 pregnancies) or once daily (11 pregnancies) were studied. These pregnancies were compared with 70 consecutive pregnancies in 53 women with similar disorders who did not receive HCQ. Electrocardiography was performed in 47 children of mothers treated with HCQ and in 45 children in the control group., Results: Eighty-eight percent of pregnancies in the HCQ group and 84% of those in the control group ended successfully with a live birth. The outcomes of pregnancy were not statistically different between groups. One child in each group died of causes related to prematurity. Three malformations were observed in the HCQ group (1 hypospadias, 1 craniostenosis, and 1 cardiac malformation) versus 4 in the control group. On the electrocardiograms, the PR interval and the corrected QT interval were not statistically different between groups. No visual, hearing, growth, or developmental abnormalities were reported in any of the children at the last follow-up (ages 12-108 months; mean age 26 months)., Conclusion: Our findings support preliminary evidence for the safety of HCQ therapy during pregnancy. This treatment probably should be maintained throughout pregnancy in patients with systemic lupus erythematosus.