Neonatal lupus: Management and outcomes
- Jill P Buyon, MD
Jill P Buyon, MD
- Professor of Medicine
- New York University School of Medicine
- Section Editors
- Thomas JA Lehman, MD
Thomas JA Lehman, MD
- Section Editor — Pediatric Rheumatology
- Professor of Clinical Pediatrics
- Cornell University Medical College
- John K Triedman, MD
John K Triedman, MD
- Section Editor — Pediatric Cardiology
- Professor of Pediatrics
- Harvard Medical School
- Joseph A Garcia-Prats, MD
Joseph A Garcia-Prats, MD
- Section Editor — Neonatology
- Professor of Pediatrics
- Baylor College of Medicine
Neonatal lupus (NL) is an autoimmune disease that is passively transferred from the mother to the fetus. The major manifestations are cardiac and cutaneous findings. The most serious complication of NL is complete heart block (approximately 20 percent have an associated cardiomyopathy at the initial diagnosis or develop it later [1,2]). In this topic review, the cardiac manifestations of NL are referred to as cardiac-NL and can include any degree of block (referred to as congenital heart block [CHB]) that may or may not be accompanied by extranodal disease such as valvular abnormalities, endocardial fibroelastosis, and/or dilated cardiomyopathy. Occasionally, those manifestations may occur in the absence of heart block.
This discussion emphasizes issues related to the treatment and potential prevention of NL. Testing for candidate antibodies is important prior to initiating therapy for a presumed case of cardiac-NL because there are cases of heart block not associated with anti-Ro/SSA (Sjögren syndrome type A antigen) or La/SSB (Sjögren syndrome type B antigen), and, thus, the management may be different. The specific indications for cardiac pacing in infants with congenital complete heart block are discussed separately. (See "Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis", section on 'Screening and surveillance' and "Congenital third degree (complete) atrioventricular block".)
The epidemiology, pathogenesis, clinical manifestations, diagnosis, screening, and surveillance of NL are discussed in greater detail separately. Pregnancy in women with systemic lupus erythematosus (SLE) and diagnosis and management of fetal arrhythmias are also reviewed elsewhere. (See "Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis" and "Pregnancy in women with systemic lupus erythematosus" and "Overview of the general approach to diagnosis and treatment of fetal arrhythmias".)
IN UTERO MANAGEMENT
Complete heart block, once identified, is irreversible despite all therapies attempted to date, including glucocorticoids, pheresis, intravenous immune globulin (IVIG), and hydroxychloroquine [2-5]. Second-degree heart block may be reversible, but it also may progress to complete heart block despite therapy [4-7]. The clinical relevance of first-degree heart block is unclear since progression from first-degree block (defined as a PR interval longer than 150 msec) to more advanced heart block in untreated fetuses has not been well documented but can occur.
Efficacy and side effects of fluorinated glucocorticoids — Published data are limited and discordant regarding the efficacy of fluorinated glucocorticoids in reducing mortality in cardiac-NL. Dexamethasone and betamethasone, which are not inactivated by placental 11-beta dehydrogenase, may ameliorate pleuropericardial effusions or hydrops, and there are reports of improved outcomes [4,8-10]. However, there are risks of glucocorticoid therapy to both the mother (eg, infection, hypertension, avascular necrosis, insulin resistance, and gestational diabetes) and the infant (eg, oligohydramnios, growth restriction, and the still undetermined potential effect upon neurocognitive development) . The use of fluorinated glucocorticoids for each degree of heart block is discussed in the sections below. (See "Use of antiinflammatory and immunosuppressive drugs in rheumatic diseases during pregnancy and lactation", section on 'Glucocorticoids' and 'First-degree heart block' below and 'Second-degree heart block' below and 'Third-degree heart block' below.)
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- IN UTERO MANAGEMENT
- Efficacy and side effects of fluorinated glucocorticoids
- First-degree heart block
- Second-degree heart block
- Third-degree heart block
- - Maternal beta-agonist therapy
- - Indications for early delivery
- Cardiomyopathy/endocardial fibroelastosis
- POSTNATAL MANAGEMENT
- Infants and children at risk for complete heart block
- Complete heart block
- Adrenal insufficiency
- Autoimmune and/or rheumatic disease
- PREVENTION OF NL IN SUBSEQUENT PREGNANCIES
- SUMMARY AND RECOMMENDATIONS