Epstein-Barr-virus와 연관된 lymphocytic interstitial pneumonia 1례
A Case of Epstein-Barr-Virus-Associated Lymphocytic Interstitial Pneumonia
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초록
저자들은 반복되는 기침과 호흡 곤란을 주소로 내원한 5세 남아에서 흉강경하 폐조직 생검을 시행하여 정상 면역 상태의 소아이지만 EBV 감염과 연관된 LIP를 확진하고, methylprednisolone과 acyclovir 치료를 시도하였으나 아직 EBV 감염이 조절되지 않고 기관지확장증이 진행하고 있는 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
Abstract
Lymphocytic interstitial pneumonia (LIP) is a benign lymphoproliferative disorder characterized histologically by diffuse infiltration with predominantly mature lymphocytes, plasma cells and reticuloendothelial cells in the alveolar septae and along the lymphatic vessels. LIP is an uncommon disease frequently associated with autoimmune diseases such as Sjögren's syndrome, systemic lupus erythematosus, chronic active hepatitis, thyroiditis, myasthenia gravis, autoimmune hemolytic anemia and Human Immunodeficiency Virus (HIV) or Epstein- Barr virus (EBV) infection. LIP can progress to pulmonary or systemic lymphoma. LIP in children has variable disease courses such as spontaneous resolution, episodic worsening or progression to frank respiratory failure. Intravenous immunoglobulin and corticosteroids yield variable results, and some children may respond to them for several years. We report herein a case of EBV-associated, HIV-negative LIP diagnosed by thoracoscopic lung biopsy and a clonality study in a 5 year-old boy who was admitted with recurrent coughing and dyspnea. The patient was treated and fully recovered with methylprednisolone and high dose acyclovir. At the 3-year follow-up, he showed no clinical symptom despite increased EBV copy in bronchoalveolar lavage and progressive bronchiectasis in the right middle lobe.
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