Tuberculosis and Respiratory Diseases

  • 제59권1호
  • /
  • Pages.77-85
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  • 2005
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  • 1738-3536(pISSN)
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  • 2005-6184(eISSN)
대한결핵및호흡기학회 (The Korean Academy of Tuberculosis and Respiratory Diseases)
권호 표지

임상적으로 진단된 특발성 폐섬유화증의 임상경과

Clinical Course of Probable Idiopathic Pulmonary Fibrosis

  • 경선영 (가천의과대학교 길병원 호흡기내과) ;
  • 박철희 (가천의과대학교 길병원 호흡기내과) ;
  • 임영희 (가천의과대학교 길병원 호흡기내과) ;
  • 안창혁 (가천의과대학교 길병원 호흡기내과) ;
  • 이상표 (가천의과대학교 길병원 호흡기내과) ;
  • 박정웅 (가천의과대학교 길병원 호흡기내과) ;
  • 전경만 (성균관대학교 의과대학 삼성서울병원 호흡기내과) ;
  • 이병훈 (성균관대학교 의과대학 삼성서울병원 호흡기내과) ;
  • 정만표 (성균관대학교 의과대학 삼성서울병원 호흡기내과) ;
  • 정성환 (가천의과대학교 길병원 호흡기내과)
  • Kyung, Sun Young (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center) ;
  • Park, Cheul Hee (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center) ;
  • Lim, Young-Hee (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center) ;
  • An, Chang Hyeok (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center) ;
  • Lee, Sang Pyo (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center) ;
  • Park, Jeong Woong (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center) ;
  • Jeon, Kyeongman (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center) ;
  • Lee, Byoung-Hoon (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center) ;
  • Chung, Man Pyo (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center) ;
  • Jeong, Sung Hwan (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
  • 투고 : 2005.04.04
  • 심사 : 2005.07.11
  • 발행 : 2005.07.30
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초록

연구배경 : 2002년 ATS/ERS consensus에서 제시된 진단 기준에 의하면 수술적 폐 생검 없이도 임상적으로 IPF를 진단할 수 있으며 이를 probable IPF라고 정의하였다. 이에 본 저자들은 probable IPF의 진단 기준을 만족하는 환자들의 임상 경과를 알아보고자 하였다. 방 법 : 1995년 3월부터 2002년 8월까지가천의대길병원과 성균관의대 삼성서울병원에서 수술적 폐 생검을 시행하지 않고 방사선 및 임상적 판단에 의해 IPF로 추정되었던 환자 149례 (길병원 28례, 삼성서울병원 121례) 중에서 probable IPF의 진단 기준을 만족하는 36명의 환자를 후향적으로 분석하였다. 결 과 : 대상 환자의 평균 연령은 65.6세 였고 남녀비는 2.6대 1이었다. 임상 증상은 호흡곤란 92% (33/36례)이 가장 많았고, 17% (6/33례)에서 발열이 동반되었으며, 평균 증상 기간은 16개월이었다. 수술적 폐 생검을 실시하지 않은 이유는 70세 이상의 고령인 경우가 가장 많았고(42%), 진단 기준을 만족한 경우(27%), 환자가 거부한 경우(14%), 동반 질환이 심각한 경우(11%) 순이었다. 대상 환자들의 1년 누적 생존률은 82.4%, 3년 누적 생존률은 50.3%였고, 생존기간 중앙값은 42개월이었다. 사망 원인은 IPF의 진행에 의한 호흡부전(59%), 호흡기 감염(14%)이 주된 원인이었다. 사망군이 생존군에 비해 BAL액의 총 세포수가 증가되어 있었다(p<0.05). 치료군과 비치료군 간에 생존률의 차이는 없었다. 결 론 : Probable IPF의 임상 경과는 조직학적으로 확진된 UIP/IPF의 알려진 임상경과와 유사하며, 임상적 진단 기준을 만족하지 못하는 경우 반드시 수술적 폐 생검을 통한 적극적인 진단 노력이 필요할 것으로 생각된다.

Background : According to the 2002 consensus report of the American Thoracic Society/European Respiratory Society (ATS/ERS), idiopathic pulmonary fibrosis (IPF) was classified as biopsy proven or probable IPF. Probable IPF is defined as those with distinctive features that allow for a confident diagnosis of IPF/usual interstitial pneumonia (UIP) within an appropriate clinical setting. The determination of the clinical course of probable IPF, as diagnosed by the ATS/ERS criteria, was studied. Methods : Between March 1995 and August 2002, 36 patients with probable IPF, from two tertiary referral hospitals, were enrolled in this study. The clinical characteristics, prognostic factors and treatment efficacy of these patients were retrospectively evaluated. Results : The mean age of the subjects was $65{\pm}6$ years. The one and 3 year survival rates were 82.4 and 50.3%, respectively, and a median survival period of 42.0 months. The total cell count of bronchoalveolar lavage was higher in the death than the survival group (p<0.05). No survival benefits were found in the cytoxan and steroid treatment groups compared with other treatment groups. Conclusion : These results suggest that the clinical course of probable IPF may be similar to that of biopsy-proven UIP. However, atypical patients must undergo an open lung biopsy for confirmation of the diagnosis.

키워드

참고문헌

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