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Systemic Lupus Erythematosus
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Maria A. Giraldo-Isaza, Bettina F. Cuneo
Neonatal lupus erythematosus (NLE) occurs in 1–2% of babies born to mothers with anti-SSA or anti-SSA/SSB antibodies. The female to male ratio of affected offspring is 14:1. All but the cardiac manifestations of NLE are transient, last up to 16 weeks. It is caused by passage of maternal Ig G (anti-Ro/SSA and anti-La/SSB) antibodies through the placenta and occurs regardless of maternal diagnosis or symptoms of lupus or other auto-immune disease. Non-cardiac manifestations include cutaneous (photosensitive annular erythematous rash), hematologic (anemia, thrombocytopenia, pancytopenia), and hepatic (elevated liver enzymes, cholestasis, fulminant liver disease) findings. The cardiac manifestations are permanent and include congenital atrioventricular (AV) block, dilated cardiomyopathy and endocardial fibroelastosis. These finding, whether isolated or occurring together, are known as cardiac neonatal lupus erythematosus (C-NLE).
Fetal arrhythmias
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Júlia Hajdú, Valéria Váradi, Zoltán Papp
In patients with fetal hydrops, cholestasis and severe and irreversible liver failure could be observed. Histologically, extensive collapse of the stroma and the absence of hepatocytes (foie vide) were observed. Cholestasis has been described in patients with neonatal lupus erythematosus without AV block (25).
Clinical practice guidance for Sjögren’s syndrome in pediatric patients (2018) – summarized and updated
Published in Modern Rheumatology, 2021
Minako Tomiita, Ichiro Kobayashi, Yasuhiko Itoh, Yuzaburo Inoue, Naomi Iwata, Hiroaki Umebayashi, Nami Okamoto, Yukiko Nonaka, Ryoki Hara, Masaaki Mori
Similar to adult cases, pediatric SS predominantly affects females and, accordingly, requires attention during pregnancy. Infertility and pregnancy-induced hypertension are rare and possibly complicated by other rheumatic diseases [92]. Transplacental passage of anti-SS-A/Ro antibody may cause neonatal lupus erythematosus (NLE), which is characterized by transient rash, liver dysfunction, and cytopenia. Although NLE usually disappears within 6 months after birth, rare cardiac diseases such as autoimmune congenital heart block (ACHB) [93], endocardial fibroelastosis [94], and dilated cardiomyopathy [95] are irreversible. Since ACHB is often fatal (mortality rate 14%−34%) and a pacemaker is required in more than 60% of cases [95–98], pregnant women with SS should receive early prenatal diagnosis and appropriate perinatal management. Transplacental administration of GC [99] and β-stimulators [100] has been reported in cases of fetal hydrops and cardiac hypofunction, although their efficacy and safety have not been established. Prophylactic transplacental GC, IVIG, and plasmapheresis may prevent neonatal ACHB, although there is limited evidence [101]. A clinical trial of maternal administration of hydroxychloroquine is currently under way [102]. MTX and MMF should be avoided in pregnancy or in those who plan to become pregnant in the future because of teratogenicity and increase in the spontaneous abortion rate.
Chorea revealing systemic lupus erythematosus in a 13-year old boy: A case report and short review of the literature
Published in International Reviews of Immunology, 2018
E. Athanasopoulos, I. Kalaitzidou, G. Vlachaki, S. Stefanaki, A. Tzagkaraki, G. Niotakis, I. Tritou, F. Ladomenou
Sweet syndrome may thus occur as a manifestation of SLE, and, as in our case, a moderate dose of an oral glucocorticoid will result in a good response.14 Thirty cases of Sweet syndrome associated with lupus erythematosus (LE) have been reported in the literature,16 including 19 cases of SLE, 3 cases of subacutecutaneous lupus erythematosus (SCLE), 3 cases of neonatal lupus erythematosus (NLE), 4 cases of drug-induced LE and one case of possible LE. Although the association of Sweet syndrome and SLE is rare, SLE should always be considered in the differential diagnosis of children or adults presenting with Sweet syndrome.
Clinical Features, Autoantibodies, and Outcome of Neonatal Lupus Erythematosus
Published in Fetal and Pediatric Pathology, 2022
Neonatal lupus erythematosus (NLE) is an uncommon and acquired disease caused by the passage of maternal autoantibodies traveling through the placenta [1,2]. Autoantibodies involved include anti-Sjogren’s syndrome antigen A (SSA)/Ro, anti-Sjogren’s syndrome antigen B (SSB)/La, and anti-ribonucleoprotein (anti-RNP). Some mothers of affected babies may suffer from systemic lupus erythematosus, Sjogren’s syndrome, rheumatoid arthritis, or other connective tissue diseases. Other mothers may remain asymptomatic throughout pregnancy and labor.