新生兒狼瘡合併非感染性肝炎，中樞神經侵犯及透明膜病變

Neonatal Lupus with Non-lnfective Hepatitis, CNS Involvement and Hyaline Membrane Disease

37天之新生兒罹患新生兒狼瘡合併皮膚紅疹，肝脾腫大及血小板減少等特徵，此一病例之母體接受腎臟組織病理切片，自體免疫抗體檢驗及配合臨床特徵為已證實的全身性紅斑性狼瘡病患。此一新生兒狼瘡病例具有陽性抗核抗體，抗Sm抗體及抗RNP抗體。然而其抗體Ro/SSA抗體與抗La/SSB抗體和其母親同樣為陰性反應。病人最後死於急性腎休克及呼吸衰竭。這個病例提供了新生兒狼瘡與非感染性肝炎以及中樞神經侵犯相關聯之證據，同時也說明了新生兒狼瘡發生透明膜病變的可能性。

A 37 days old age neonate had neonatal lupus erythematosus manifested by skin rash, hepatosplenomegaly and thrombocytopenia. His mother was a well-documented lupus patient proved by renal tissue pathology, autoantibodies and many clinical manifestations. The neonate had positive anti-nuclear, anti-Sm, and anti-RNP antibodies. However, his anti-Ro/ SSA, anti-La/ SSB antibodies were negative and which was same as his mother. He died of acute renal shut down and respiratory failure. A necropsy was done and pathological findings are reported. This case provided further convincing evidence of neonatal lupus syndrome (NLS) associated with non-infective hepatitis and CNS involvement, also the probable relationship between NLS and low pressure pulmonary edema characterizing hyaline membrane disease.