급성 루푸스 폐렴으로 발현된 전신성 홍반성 낭창
Acute Lupus Pneumonitis as Initial Manifestation of Systemic Lupus Erythematosus
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초록
전신성 홍반성 낭창은 여러 장기를 침범하는 대표적인 결체 조직 질환으로 주로 성인에서 발생하지만 15-20%는 소아에서 시작되고 자가 항체에 의해 급성으로 발생하는 폐병변으로 급성 루푸스 폐렴이 드물게 발생한다. 본 저자들은 늑막 삼출, 림프구 감소, 항 핵항제와 항 dsDNA 항체 양성으로 진단된 전신성 홍반성 낭창에서 조직검사를 통해 병리소견을 확진한 급성 루푸스 폐렴을 경험하였기에 보고하는바이다.
Abstract
We report case of a 14-year-old girl with systemic lupus erythematosus who initially presented with acute lupus pneumonitis. She had a 4-week history of exertion for the past 4 weeks, and was transferred from a regional hospital due to abnormality on chest radiographs. Chest radiographs revealed bilateral infiltration and pleural effusion on both lower lung fields. We assumed her to be infected and prescribed antibiotics. The response to antibiotics was ineffective, and viral, bacterial, and mycobacterial cultures were negative. Antinuclear and anti-dsDNA antibodies in serum were positive. The open lung biopsy revealed diffuse alveolar damage. She was diagnosed as having acute lupus pneumonitis in systemic lupus erythematosus and recovered gradually after receiving corticosteroids. Acute lupus pneumonitis may be considered even though interstitial lung involvement in systemic lupus erythematosus is relatively rare in pediatric practice and its diagnosis is difficult.
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