Síndrome do lúpus neonatal

Abstract

Neonatal Lupus Syndrome (NLS) is an autoimmune disease associated to the presence of autoantibodies against ribonucleoproteins SSA/Ro and/or SSB/La in the maternalfetal circulation and is characterized by isolated congenital heart block (ICHB) and/or cutaneous and hematological manifestations. Despite the rarity, NLS is the main cause of ICHB, which is responsible for the significant mortality (20%-30%) and morbidity of these patients. The neonatal lupus designation comes from the observation of cutaneous lesions similarities between NLS and systemic lupus erythematosus. The isolated term to define the CHB in NLS is employed to exclude cardiac abnormalities secondary to congenital structural malformations or infections. NLS is considered a model of passively acquired autoimmunity in which maternal autoantibodies cross the placenta and, once in fetal circulation, may play a role in the pathogenesis of the syndrome. The almost universal presence of anti-SSA/Ro and/or anti-SSB/La antibodies in the maternal and fetal circulation validates them as serological markers of NLS. Contrasting with the fetal cardiac tissue lesion, which is an irreversible process, cutaneous and hematological involvements are transitory manifestations and disappear after the clearance of maternal autoantibodies from the infant's circulation. It is important to notice that NLS represents a multidisciplinary challenge involving rheumatologists, obstetricians, neonatologists, dermatologists and pediatric cardiologists to identify the pregnancy at risk for NLS development and to introduce appropriate in utero or postnatal therapeutic strategies.

Scars on the skin, scars in the heart

“…[3] The rash is typically annular or polycyclic, erythematous and scaly. It commonly appears weeks after birth and is distributed usually on sun exposed areas, mainly face, scalp and neck.…”

“…However, multisystem involvement is less common. [ 3 4 5 ] Both mother and the child are at an increased risk of developing some rheumatologic or autoimmune disease in future. [ 3 5 6 7 ]…”

“…NLE dermatitis is a transitory benign condition disappearing in approximately seven months coinciding with the disappearance of maternal IgG in fetal circulation. [ 3 ] The rash is typically annular or polycyclic, erythematous and scaly. It commonly appears weeks after birth and is distributed usually on sun exposed areas, mainly face, scalp and neck.…”