A Case of Lung Involvement Showing Multiple Lung Cysts in Primary Sjögren's Syndrome

다발성 폐 낭종을 보인 쇼그렌 증후군의 폐 침범 1예

  • Kim, Ji Yon (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Hwang, Hyun Gyu (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Choi, Jae Sung (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Seo, Ki Hyun (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Kim, Yong Hoon (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Oh, Mee Hye (Department of Pathology, Soonchunhyang University College of Medicine) ;
  • Na, Ju Ock (Department of Internal Medicine, Soonchunhyang University College of Medicine)
  • 김지연 (순천향대학교 의과대학 천안병원 내과학교실) ;
  • 황현규 (순천향대학교 의과대학 천안병원 내과학교실) ;
  • 최재성 (순천향대학교 의과대학 천안병원 내과학교실) ;
  • 서기현 (순천향대학교 의과대학 천안병원 내과학교실) ;
  • 김용훈 (순천향대학교 의과대학 천안병원 내과학교실) ;
  • 오미혜 (순천향대학교 의과대학 천안병원 병리학교실) ;
  • 나주옥 (순천향대학교 의과대학 천안병원 내과학교실)
  • Received : 2008.01.20
  • Accepted : 2008.02.22
  • Published : 2008.03.30

Abstract

We described herein the first case of primary $Sj{\ddot{o}}gren^{\prime}s$ syndrome in Korea which presented with multiple cysts caused by only peribronchiolar lymphocytic infiltration, a rare pulmonary manifestation in $Sj{\ddot{o}}gren^{\prime}s$ syndrome, and was confirmed by surgical lung biopsy. A brief review of the literature has been included.

쇼그렌 증후군은 림프구 침윤과 관련된 만성적인 염증성 자가면역 질환으로 아직 정확한 병태생리학적 기전은 밝혀지지 않았다. 45세 여자 환자가 내원 2년 전 전신 쇠약 및 피로감으로 입원하여 혈청 검사에서 anti-Ro/La antibody 양성, 흉부 단순방사선 및 컴퓨터 촬영에서 양 폐야의 다낭성 병변이 관찰되어 비디오 흉강경을 이용한 폐 생검 시행 결과 세기관지 주위에 림프구 침윤 및 다양한 크기의 폐낭종들이 관찰되어 쇼그렌 증후군의 폐 침범 의심하에 추가 검사 시행하려 하였으나 추적 관찰 되지 않았다. 2년 후 폐렴으로 입원하였으며, 다시 시행한 흉부 컴퓨터 단층촬영에서 다발성 낭성 변화는 큰 차이를 보이지 않았다. 쇼그렌 증후군의 폐 침범은 다양한 형태로 나타나는데, 단순히 세기관지 주위에 림프구 침윤에 의한 다낭성 폐 질환에 대한 보고는 극히 드물다. 따라서 본 저자들은 일차성 쇼그렌 증후군 환자에서 비디오 흉강경을 이용한 폐 생검으로 진단된 세기관지 주위에 림프구 침윤을 동반한 다낭성 폐 질환 1예를 경험하였기에 보고하는 바이다.

Keywords

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