Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome

Lévesque, Kateri; Morel, Nathalie; Maltret, Alice; Baron, Gabriel; Masseau, A.; Orquevaux, P.; Piette, Jean‐Charles; Barrière, F.; Bidois, J. Le; Fermont, L; Fain, Olivier; Theulin, Arnaud; Sassolas, F; Pézard, P.; Amoura, Zahir; Guettrot-Imbert, G.; Mercier, Delphine Le; Georgin‐Lavialle, Sophie; Deligny, C.; Hachulla, É.; Mouthon, Luc; Ravaud, Philippe; Villain, Elisabeth; Bonnet, Damien; Costédoat-Chalumeau, Nathalie; Bézanahary, H.; Bienvenu, Boris; Blaison, G.; Blanche, P.; Bonnotte, B.; Cathébras, P.; Christides, C.; Cohen, Fleur; Cohen, Laurence; Devaud, Edouard; Diot, Élisabeth; Duhaut, P.; Dulac, Yves; Godeau, Bertrand; Gournay, Véronique; Gronier, Céline; Guillevin, Loı̈c; Hamidou, M.; Haroche, Julien; Hayem, Gilles; Heitz, Florian; Isnard, R.; Jallouli, M.; Korganow, Anne-Sophie; Jeunne, Claire Le; Lhote, F.; Lucron, Hugues; Lusson, Jean-René; Magnier, S; Ninet, J.; Pangaud, Nicolas; Papo, Thomas; Pellegrin, Jean‐Luc; Pennaforte, Jean Loup; Pouchot, J.; Sarrot-Reynauld, F.; Schleinitz, N.; Sève, P.; Stos, Bertrand; Vital-Durand, D; Wechsler, B.

doi:10.1016/j.autrev.2015.08.005

Cited by 125 publications

(137 citation statements)

References 27 publications

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“…Moreover, there is no proven efficacy of protocols for the prevention or treatment of complete CHB. 99 100 The efficacy of maternal fluorinated steroids has not been established in large cohorts [101][102][103][104] despite initial reports of favourable effects in cases of incomplete CHB, cardiomyopathy, endocardial fibroelastosis and hydrops fetalis. 99 Given the potential of fluorinated steroids for major maternal and fetal side effects, the benefit for fetuses with CHB should be stratified according to the presence of risk factors for adverse outcome.…”

Section: Predictive Biomarkers For Maternal Disease Activity In Sle Pmentioning

confidence: 99%

EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

et al. 2016

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ObjectivesDevelop recommendations for women's health issues and family planning in systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS).MethodsSystematic review of evidence followed by modified Delphi method to compile questions, elicit expert opinions and reach consensus.ResultsFamily planning should be discussed as early as possible after diagnosis. Most women can have successful pregnancies and measures can be taken to reduce the risks of adverse maternal or fetal outcomes. Risk stratification includes disease activity, autoantibody profile, previous vascular and pregnancy morbidity, hypertension and the use of drugs (emphasis on benefits from hydroxychloroquine and antiplatelets/anticoagulants). Hormonal contraception and menopause replacement therapy can be used in patients with stable/inactive disease and low risk of thrombosis. Fertility preservation with gonadotropin-releasing hormone analogues should be considered prior to the use of alkylating agents. Assisted reproduction techniques can be safely used in patients with stable/inactive disease; patients with positive antiphospholipid antibodies/APS should receive anticoagulation and/or low-dose aspirin. Assessment of disease activity, renal function and serological markers is important for diagnosing disease flares and monitoring for obstetrical adverse outcomes. Fetal monitoring includes Doppler ultrasonography and fetal biometry, particularly in the third trimester, to screen for placental insufficiency and small for gestational age fetuses. Screening for gynaecological malignancies is similar to the general population, with increased vigilance for cervical premalignant lesions if exposed to immunosuppressive drugs. Human papillomavirus immunisation can be used in women with stable/inactive disease.ConclusionsRecommendations for women's health issues in SLE and/or APS were developed using an evidence-based approach followed by expert consensus.

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Section: Predictive Biomarkers For Maternal Disease Activity In Sle Pmentioning

confidence: 99%

EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

et al. 2016

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“…The actual incidence may be higher as incomplete forms of CHB have been described, including first-degree heart block that can progress during childhood. Most children with CHB will need a permanent pacemaker (47%–64% in first year67,68 and 75% by 10 years),68 and up to 20% may die in the perinatal period 66,68. Neonatal CHB may also be the first presentation of otherwise asymptomatic connective tissue disease in up to 50% of women: all mothers of infants with CHB are tested for anti-Ro/La antibodies 69…”

Section: Antenatal/postnatal Challenge: Neonatal Lupusmentioning

confidence: 99%

“…If evidence of incomplete heart block, myocarditis, ascites, or hydrops is identified, then therapy with fetal steroids (dexamethasone or betamethasone) could be considered 22,68. Three large studies found no benefit on feto-neonatal mortality;68 one study of historical cases suggested an improvement in 1-year survival with steroid treatment 72.…”

Section: Antenatal/postnatal Challenge: Neonatal Lupusmentioning

confidence: 99%

Management of systemic lupus erythematosus during pregnancy: challenges and solutions

Knight¹,

Nelson‐Piercy²

2017

OARRR

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Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal–neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy. In pregnancy, early referral for hospital-coordinated care, involvement of obstetricians and rheumatologists (and other specialists as required), an individual management plan, regular reviews, and early recognition of flares and complications are all important. Women are at risk of lupus flares, worsening renal impairment, onset of or worsening hypertension, preeclampsia, and/or venous thromboembolism, and miscarriage, intrauterine growth restriction, preterm delivery, and/or neonatal lupus syndrome (congenital heart block or neonatal lupus erythematosus). A cesarean section may be required in certain obstetric contexts (such as urgent preterm delivery for maternal and/or fetal well-being), but vaginal birth should be the aim for the majority of women. Postnatally, an ongoing individual management plan remains important, with neonatal management where necessary and rheumatology followup. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and vice versa, and reviews antirheumatic medications with the latest guidance about their use and safety in pregnancy. Such information is required to effectively and safely manage each stage of pregnancy in women with SLE.

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“…In a recent large French study, the use of FS was not associated with CHB regression or increased survival [104]. Therefore, the decision to administer such FS, usually at high dosage (at least 4 mg of dexamethasone daily) should be weighed against potential side effects for both foetus (IUGR, oligohydramnios) and mother (infections, osteoporosis, osteonecrosis, diabetes).…”

Section: Lupus Anti-ro/ssa and Anti-la/ssb Antibodies And Neonatal Lmentioning

confidence: 99%

A comprehensive review of the clinical approach to pregnancy and systemic lupus erythematosus

Lazzaroni

Dall’Ara

Fredi

et al. 2016

Journal of Autoimmunity

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Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome

Cited by 125 publications

References 27 publications

EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

Management of systemic lupus erythematosus during pregnancy: challenges and solutions

A comprehensive review of the clinical approach to pregnancy and systemic lupus erythematosus

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