Etiologic Distribution, Diagnostic Tests and Treatment in Prospectively Registered Patients with DILD for Two Years in a Tertiary Medical Center

한 3차 병원에서 2년간 전향적으로 등록된 DILD 환자의 원인, 진단 수기 및 치료 현황

  • Kwon, Yong Soo (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Chung, Man Pyo (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jeon, Gyeong Man (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lyu, Yeon Ju (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Yu, Chang Min (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Choi, Jae Chul (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kang, Eun Hae (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Koh, Won-Jung (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Suh, Gee Young (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Hojoong (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kwon, O Jung (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 권용수 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 정만표 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 전경만 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 류연주 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 유창민 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 최재철 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 강은해 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 고원중 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 서지영 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 김호중 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과) ;
  • 권오정 (성균관대학교 의과대학 내과학교실 삼성서울병원 호흡기내과)
  • Received : 2005.03.08
  • Accepted : 2005.05.02
  • Published : 2005.06.30

Abstract

Introduction : Diffuse interstitial lung diseases (DILD) comprise of a large group of lung diseases with diverse etiologies. They are classified into four categories based on the etiology and pathological findings. In Korea, epidemiological data on DILD has never been reported in a prospective manner. Method : From May 2002 to April 2004, total 487 patients with DILD were prospectively registered at Samsung Medical Center. The prospective observational analysis of the etiologies, its classification based on 2002 ATS/ERS (American Thoracic Society/European Respiratory Society) guidelines, as well as diagnostic tests and the retrospective analysis of the treatment modalities were carried out. Any infectious and malignant causes were excluded. Results : 1) The patients were classified into idiopathic interstitial pneumonia (IIP) in 269 patients (55.2%), known causes of DILD in 168 patients (34.5%), sarcoidosis in 27 patients (5.5%), other forms of DILD in 14 patients (2.9%), and undetermined DILD in 9 patients (1.9%). 2) The diagnostic test showed that most patients had undergone chest high resolution computed tomography (HRCT) and pulmonary function test (PFT) (97%, 89%). Transbronchial lung biopsy (TBLB) and surgical lung biopsy (SLB) were performed in limited patients (38%, 29%). 3) Among 269 patients with IIP, 220 (82%) had idiopathic pulmonary fibrosis (IPF) while 23 (9%) had nonspecific interstitial pneumonia. SLB was carried out in 36% of patients with IIP. 4) Symptomatic supportive care was given to 67% of IPF, but specific medical treatment including corticosteroids was administered to 89% of non-IPF patients. Conclusion : A nationwide registry of DILD patients is required to determine the annual incidence, etiology, and practice pattern of diagnosis and treatment in Korea.

배 경 : 국내에서 전향적으로 DILD를 등록하여 원인 분류한 보고가 없고 진단 수기 및 치료에 대한 보고도 전무하여, 한 3차 대학병원에서 2년 동안 전향적으로 진단된 DILD의 원인, 진단 수기 및 치료현황에 대한 실태를 파악하고자 본 연구를 시행하였다. 방 법 : 2002년 5월부터 2004년 4월까지 2년간 삼성서울병원에서 전향적으로 등록된 DILD 환자 487명을 대상으로, 2002년 ATS/ERS 분류에 따른 원인, 진단에 이용된 수기, 치료 방법에 대해 후향적 조사를 시행하였다. 결 과 : 1) 전체 487명중 DILD of known causes 168명, IIP 269명, granulomatous DILD 27명, other forms of DILD 14명이었고, 분류가 불확실한 환자 9명이었다. 2) 진단 수기는 HRCT가 97%, 폐기능검사가 89%, BAL이 44%, TBLB가 38%, 외과적 폐생검이 29%에서 이용 되었다. 3) 269명의 IIP 환자 중 IPF가 222명(82%), NSIP 23명(9%)이었고, 진단수기는 HRCT가 97%, BAL이 42%, 외과적 폐생검이 36%에서 받았다. 4) IIP 환자 중 폐생검을 받은 환자는 받지 않은 환자에 비해 평균연령이 더 낮고(57.8세 vs 68.2세, p<0.05) 심장질환 등의 동반 질환이 적었으며(16.5% vs 69%, p<0.001), HRCT 소견상 non-IPF인 경우가 더 많았다(33.7% vs 9.2%, p<0.001). 5) IPF 환자는 67%에서 대증적 치료만을 받았으나 non-IPF 환자에서는 89%에서 스테로이드 등의 약물치료를 받았다. 결 론 : 국내에서도 DILD 질환 등록 사업 및 적절한 검사를 통한 정확한 진단이 필요하고 이에 따른 치료가 이루어져야 할 것으로 사료된다.

Keywords

References

  1. The diagnosis, assessment and treatment of diffuse parenchymal lung disease in adults: introduction. Thorax 1999;54(Suppl 1):S1-14 https://doi.org/10.1136/thx.54.1.1
  2. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS). Statement on sarcoidosis: Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999;160:736-55 https://doi.org/10.1164/ajrccm.160.2.ats4-99
  3. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement. Am J Respir Crit Care Med 2000;161:646-64 https://doi.org/10.1164/ajrccm.161.2.ats3-00
  4. du Bois RM, Wells AU. Cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis. Eur Respir J Suppl 2001;32:43s-55s
  5. American Thoracic Society/European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias: this joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002;165:277-304 https://doi.org/10.1164/ajrccm.165.2.ats01
  6. Raghu G. Interstitial lung disease: a diagnostic approach: are CT scan and lung biopsy indicated in every patient? Am J Respir Crit Care Med 1995;151:909-14 https://doi.org/10.1164/ajrccm/151.3_Pt_1.909
  7. Song JS. Diagnostic approach in idiopathic interstitial pneumonia. Tuberc Respir Dis 2003;54:251-9 https://doi.org/10.4046/trd.2003.54.3.251
  8. Thomeer MJ, Costabe U, Rizzato G, Poletti V, Demedts M. Comparison of registries of interstitial lung diseases in three European countries. Eur Respir J Suppl 2001;32:114s-8s
  9. Coultas DB, Zumwalt RE, Black WC, Sobonya RE. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 1994;150:967-72 https://doi.org/10.1164/ajrccm.150.4.7921471
  10. Coultas DB, Hughes MP. Accuracy of mortality data for interstitial lung diseases in New Mexico, USA. Thorax 1996;51:717-20 https://doi.org/10.1136/thx.51.7.717
  11. Kim JH, Choi SJ, Kim DS, You JH, Kang HM, You SH, et al. The etiology of the diffuse infiltrative disease of the lung in Korea. Tuberc Respir Dis 1991;38:1-7
  12. Lim GI, Lee KH, Jeong SW, Uh ST, Jin SY, Lee DH, et al. Clinical features of interstitial lung diseases. Korean J Intern Med 1996;11:113-21 https://doi.org/10.3904/kjim.1996.11.2.113
  13. Liscoet-Loheac N, Andre N, Couturaud F, Chenu E, Quiot JJ, Leroyer C. Hypersensitivity pneumonitis in a patient taking pravastatin. Rev Mal Respir 2001;18:426-8
  14. Rizzato G, Bariffi F. Inchiesta epidemiologica sulle interstiziopatic polmonari in Italia: dati e risultati a macchia di leopardo. L'Internista 1999;7:20-24
  15. Padley SP, Adler B, Muller NL. High-resolution computed tomography of the chest: current indications. J Thorac Imaging 1993;8:189-99 https://doi.org/10.1097/00005382-199322000-00004
  16. Padley SP, Hansell DM, Flower CD, Jennings P. Comparative accuracy of high resolution computed tomography and chest radiography in the diagnosis of chronic diffuse infiltrative lung disease. Clin Radiol 1991;44:222-6 https://doi.org/10.1016/S0009-9260(05)80183-7