Neonatal lupus erythematosus: a 20-year retrospective study.

OBJECTIVE

To study clinical manifestations, investigations, treatment and outcomes of neonatal lupus erythematosus (NLE) patients and their mothers.

MATERIAL AND METHOD

A retrospective descriptive study was performed to review the neonatal lupus erythematosus patients and their mothers at Queen Sirikit National Institute of Child Health during January 1993 to December 2013. The diagnostic criteria required the presence of clinical symptoms plus positive anti-Ro/SSA or anti-La/SSB or both.

RESULTS

There were 34 cases, 12 males and 22 females. Age of onset of clinical manifestations was from birth to 60 days with median age of 21 days. Cutaneous, hepatobiliary, hematological and cardiac abnormalities were found in 88.2%, 61.2%, 50%, 14.7%, respectively. Cutaneous lesions included erythematous rash (70%), annular lesions (75.8%), petechiae (26.6%), raccoon eyes (26.6%), and telangiectasia (20%). Among those with hepatic involvement (n = 18), transaminitis was the most common finding (100%) followed by hepatosplenomegaly (38.8%) and cholestasis (22.2%). Seventeen cases (50%) had hematological problems including anemia (29.4%) and anemia with thrombocytopenia (20.6%). The most severe complication, complete heart block was found in 14.7% (n = 5). Anti-Ro/SSA and anti-La/SSB were positive in 91.1% and 58.8% of cases, respectively. All four babies with complete heart block were treated with pacemaker. Systemic corticosteroids were given to eleven babies due to severe skin lesions and hepatic involvement. There was no mortality during the study. Most neonatal lupus erythematosus mothers (24 cases, 74.2%) were asymptomatic. Ten mothers (25.8%) were diagnosed as autoimmune diseases (systemic lupus erythematosus in 6 cases and other autoimmune diseases in 4 cases). However, 7 of 24 asymptomatic mothers developed SLE within 3 years after delivery.

CONCLUSION

NLE should be suspected among neonates or young infants presenting with congenital heart block or skin rash with multi-system involvement despite a lack of concurrent maternal autoimmune diseases. Anti-Ro/SSA and/or anti-La/SSB are the most useful laboratory diagnosis. Most NLE patients without congenital heart block have relatively good prognosis.