Familial Sarcoidosis, The First Report in Korea

가족형 폐유육종증

  • Uhm, Wan-Sik (Department of Internal Medicine, Asan Medical Center, College of Medicine, Ulsan University) ;
  • Lim, Chae-Man (Department of Internal Medicine, Asan Medical Center, College of Medicine, Ulsan University) ;
  • Kim, Woo-Sung (Department of Internal Medicine, Asan Medical Center, College of Medicine, Ulsan University) ;
  • Kim, Dong-Soon (Department of Internal Medicine, Asan Medical Center, College of Medicine, Ulsan University) ;
  • Kim, Won-Dong (Department of Internal Medicine, Asan Medical Center, College of Medicine, Ulsan University)
  • 엄완식 (울산대학교 의과대학 서울중앙병원 내과학교실) ;
  • 임채만 (울산대학교 의과대학 서울중앙병원 내과학교실) ;
  • 김우성 (울산대학교 의과대학 서울중앙병원 내과학교실) ;
  • 김동순 (울산대학교 의과대학 서울중앙병원 내과학교실) ;
  • 김원동 (울산대학교 의과대학 서울중앙병원 내과학교실)
  • Published : 1994.12.31

Abstract

Sarcoidosis can affect two or more members of the same family, and the reported occurrence of such familial sarcoidosis is variable from 0.5 to 14%. Recent1y we have experienced familial sarcoidosis affected mother and daughter, for the first time in Korea. Mother was diagnosed as Stage 11 sarcoidosis 4 years ago by transbronchial lung biopsy and cervical lymph node biopsy with compatible BAL finding in our hospital. This time, the daughter was admitted with bilateral hilar enlargement and anterior uveitis. Even though she had positive tuberculin skin test and atypical BAL finding(lymphocyte: 61%, CD4/CD8: 1.22). Transbronchial lung biopsy and mediastinal lymph node biopsy revealed noncaseating epithelioid granuloma without AFB. Slit lamp examination of the eyes showed severe anterior uveitis. Systemic steroid therapy was started due to progressive uveitis with antituberculous medication.

어머니가 Stage 2 폐유육종증으로 진단 받고 치료된 후 4년만에 친 딸에서 X선상 폐문종대와 전방 포도막염이 발생하여, 기관지 폐생검 및 종격동경으로 실시한 종격동 임파선 생검으로 유육종증으로 진단된 가족형 유육종증이 우리나라에서는 처음 발견되어 문헌 고찰과 함께 보고하는 바이다.

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