Hacer YAPICIOĞLU, Mustafa ÖZDEMİR, Tugay TEPE, Tolga AKBAŞ, Ferda ÖZLÜ, Sevcan ERDEM

Maternal Sjögren sendromuna bağlı perikardiyal tamponadı olan neonatal lupus sendromu

Neonatal lupus sendromu (NLS), otoimmün hastalığı olan annelerin bebeklerinde anti-Ro/ SSA ve anti-La/ SSB antikorlarının plasental transferine bağlı olarak dermatolojik, kardiyak, hepatik ve hematolojik tutulumla seyreden nadir bir hastalıktır. Bebeklerde multisistemik tutulum sık görülür. Burada, annesinde Sjögren sendromu olan neonatal lupus sendromlu bir olgu sunulmuştur. Bebekte önce trombositopeni, ardından konvülsiyon ve perikardiyal tamponada ikincil kardiyak arrest gelişmiştir. Otoimmün hastalığı olan annelerin bebeklerinde NLS riski akılda tutulmalıdır.

Anahtar Kelimeler:

Sjögren sendromu, Neonatal lupus sendromu, Konvülziyon, Perikardiyal tamponad

A neonatal lupus syndrome with pericardial tamponade due to maternal Sjögren’s syndrome

Neonatal lupus syndrome (NLS) is a rare disease with dermatological, cardiac, hepatic and hematologic involvement due to placental transfer of anti-Ro/ SSA and anti-La/ SSB antibodies in infants of mothers with autoimmune diseases. Multisystemic involvement is common in infants. Here we present a baby with neonatal lupus syndrome whose mother had Sjögren's syndrome. He presented with thrombocytopenia, then convulsion and cardiac arrest due to pericardial tamponade. So, the risk of NLS should be kept in mind in infants of mothers with autoimmune diseases.

Keywords:

Sjögren’s syndrome, Neonatal lupus syndrome, Convulsion, Pericardial tamponade,

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