Presentation
This is much higher and reaches up to 25% if the mother already had a child with neonatal lupus. 15-20% present as complete heart block and 6% present as cutaneous lupus. [wikidoc.org]
Case presentation: A healthy asymptomatic 41-year-old woman with twin pregnancy, primigravidia, was referred to a pediatric cardiologist at 30 weeks of gestation after detection of a decreased fetal heart rate upon obstetric ultrasonography examination [latunisiemedicale.com]
Cutaneous features of neonatal lupus erythematosus Skin changes may be present at birth or becomes apparent within 3 months of birth, typically soon after first sun exposure. [dermnetnz.org]
See Presentation for more detail. [emedicine.medscape.com]
Problems involving the liver, gallbladder, brain, and blood can be seen but are usually transient.[1] Heart Cardiac manifestations present more commonly in utero, but can also present after birth. [wikiwand.com]
Entire Body System
- Camping
J Immunol 171: 3253–3261 CAS Article PubMed Google Scholar Clancy RM et al. (2007) Role of hypoxia and cAMP in the transdifferentiation of human fetal cardiac fibroblasts: implications for progression to scarring in autoimmune-associated congenital heart [nature.com]
Workup
See Workup for more detail. Management of neonatal and pediatric lupus erythematosus Treatment of cutaneous NLE is not required, as the lesions resolve without scarring. [emedicine.medscape.com]
Treatment
Treatment Medical Therapy Prenatal treatment with fluorinated glucocorticoids is suggested for mothers of fetuses with second degree heart block, through the end of pregnancy depending upon the response. [wikidoc.org]
In our cases, there aren’t any signs of heart failure and the treatment consists of pacemakers’implantation. The efficiency of prophylactic treatment of complete congenital heart block is not established [9,11]. [latunisiemedicale.com]
Obstet Gynecol 92: 689–691 CAS Article Google Scholar Breur JMPJ et al. (2004) Treatment of fetal heart block with maternal steroid therapy: case report and review of the literature. [nature.com]
What is the treatment for neonatal lupus erythematosus? Hydroxychloroquine is reported to reduce the risk of cardiac neonatal LE in pregnant women with Ro and/or La autoantibodies. [dermnetnz.org]
The type of treatment for neonates with cardiac rhythm and conduction disturbances depends on the presence of underlying congenital heart abnormalities. See Treatment and Medication for more detail. [emedicine.medscape.com]
Prognosis
The prognosis following pacemaker implantation is excellent for most children, although development of heart failure may occur. [wikidoc.org]
Generally, the prognosis following pacemaker implantation in children with congenital heart block is good [9]. Our cases clearly illustrate this positive trend. [latunisiemedicale.com]
Renal involvement is a major determinant of prognosis. Joint disease does not lead to deformity but may be debilitating. Disease of the skin may lead to scar formation; however, in isolation, it is associated with a good prognosis. [emedicine.medscape.com]
The prognosis of NLE depends on the organs that are affected. Infants with cutaneous lesions alone have an excellent prognosis, and the rash generally resolves by six months of age with the clearance of maternal antibodies. [cureus.com]
Outlook / Prognosis What is the long-term outlook for children who have lupus (systemic lupus erythematosus)? Children who receive proper treatment for systemic lupus erythematosus generally do very well. [my.clevelandclinic.org]
Etiology
"Etiology of Atrioventricular block". UpToDate. Retrieved March 10, 2022. ^ Cooper LT. "Definition and classification of the Cardiomyopathies". UpToDate. Retrieved March 10, 2022. [wikiwand.com]
Etiology and Pathophysiology Lupus erythematosus (LE) is a rare condition in children; most cases take the form of systemic lupus erythematosus (SLE). [emedicine.medscape.com]
"Etiology of Atrioventricular block". UpToDate. Retrieved March 10, 2022. ^ Cooper LT. "Definition and classification of the Cardiomyopathies". UpToDate. Retrieved March 10, 2022. External links[edit] [en.wikipedia.org]
Friedman DM, Rupel A, Buyon JP (2007) Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus. Curr Rheumatol Rep 9: 101-108. [heraldopenaccess.us]
Teleangectasia may be present, as in our second case, and disordered angiogenesis can play a role in its etiology [4]. [ijponline.biomedcentral.com]
Epidemiology
Epidemiology and Demographics The prevalence of Anti-SSA antibodies in women is 1:200 while the incidence of neonatal lupus is only 1 in 20,000 live births.[5] Only 1-2% of the infants of mothers with anti-SSA/Ro with or without anti-SSB/La antibodies [wikidoc.org]
Epidemiology Neonatal lupus erythematosus (NLE) occurs in 1 of every 20,000 US live births. Lupus erythematosus (LE) of childhood occurs in 0.6-2.2 of every 100,000 children annually. [emedicine.medscape.com]
"Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis". UpToDate. Retrieved March 9, 2022. ^ a b c d e f g h i j Wallace DJ, Hahn B (2019). Dubois' lupus erythematosus and related syndromes. Daniel J. [wikiwand.com]
Friedman DM, Rupel A, Buyon JP (2007) Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital heart block in neonatal lupus. Curr Rheumatol Rep 9: 101-108. [heraldopenaccess.us]
Pathophysiology
Finally in 1980 Weston reported the association of neonatal lupus with maternal anti-Ro autoantibodies.[2] Pathophysiology Neonatal lupus is presumed to result from transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies. [wikidoc.org]
Oxford, UK: Taylor & Francis Google Scholar Alexander E et al. (1992) Anti-Ro/SS-A antibodies in the pathophysiology of congenital heart block in neonatal lupus syndrome, an experimental model: in vitro electrophysiologic and immunocytochemical studies [nature.com]
Etiology and Pathophysiology Lupus erythematosus (LE) is a rare condition in children; most cases take the form of systemic lupus erythematosus (SLE). [emedicine.medscape.com]
Alexander E, Buyon JP, Provost TT, Guarnieri T (1992) Anti-Ro/SS-A antibodies in the pathophysiology of congenital heart block in neonatal lupus syndrome, an experimental model. In vitro electrophysiologic and immunocytochemical studies. [heraldopenaccess.us]
Jill Buyon raised the possibility that a maternal microchimerism phenomenon may contribute to the pathophysiology of CAVB. [14] Innate immune mechanisms involving TLRs may be involved in neonatal lupus. [indianjrheumatol.com]
Prevention
Science 320: 373–376 CAS Article PubMed PubMed Central Google Scholar Preventive IVIG Therapy for Congenital Heart Block (PITCH) [http://clinicaltrials.gov/ct2/show/NCT00460928?term=NCT00460928&rank=1] (accessed 13 January 2009) [nature.com]
Women who have already had a child with neonatal lupus erythematosus should receive dexamethasone and intravenous immunoglobulin for prevention of recurrent complete congenital heart block from the time of conception with continuation through at least [latunisiemedicale.com]
Failure of intravenous immunoglobulin to prevent congenital heart block: Findings of a multicenter, prospective, observational study. Arthritis Rheum. 2010 Apr. 62(4):1147-52. [QxMD MEDLINE Link]. Izmirly PM, Kim MY, Llanos C, et al. [emedicine.medscape.com]
[…] self-limited.[3] Many of the clinical manifestations are transient, but certain heart problems can be permanent.[4] Diagnosis is based on maternal antibodies and clinical manifestations.[1] Treatment and management is mainly supportive and focused on preventing [wikiwand.com]
Timely and appropriate evaluation is necessary to prevent complications. We describe the case of a three-month-old infant born at term who developed a rash at six weeks of life and who was found to have cutaneous neonatal lupus erythematosus. [cureus.com]