Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
권호 표지
DOI QR코드

DOI QR Code

2008 National Survey of Idiopathic Interstitial Pneumonia in Korea

2008년도 특발성 간질성 폐렴(IIP) 전국실태조사보고

  • Received : 2009.01.09
  • Accepted : 2009.02.09
  • Published : 2009.02.28
Download PDF
⟨ Previous Next ⟩

Abstract

Background: There is limited data on the epidemiology and relative frequency of idiopathic interstitial pneumonia (IIP) worldwide. This survey was performed to assess the epidemiology and relative frequency of IIP in Korea. Methods: The patients with IIP and who were confirmed by lung biopsy, except those patients with idiopathic pulmonary fibrosis, (IPF) over a 5 year period (from Jan. $1^{st}$, 2003 to Dec. $31^{st}$, 2007) were registered by a web-base questionnaire. Results: A total of 3,156 cases were registered, but 970 cases were excluded due to duplicative registration, inadequate data and the unmet ATS/ERS diagnostic criteria. A total of 2,186 cases were analyzed. The male to female ratio was about 2 : 1 and their mean age was 65 (range: 11-94). The most frequent disease was IPF (77.1%), followed in decreasing order by nonspecific interstitial pneumonia (NSIP) (11.9%), cryptogenic organizing pneumonia (COP) (8.5%), acute interstitial pneumonia (AIP) (1.1%), desquamative interstitial pneumonia (DIP) (0.9%), respiratory bronchiolitis-interstitial lung disease (RB-ILD) (0.4%) and lymphocytic interstitial pneumonia (LIP) (0.1%). The mean age of the patients with IPF, NSIP and COP was 67.8, 57.1 and 57.7 years old, respectively. The most frequent symptom was dyspnea on exertion (69%) followed by coughing (61%) and sputum (33%) for the whole population. The three year survival rate was 62% for the patients with IPF and the five year survival rate was 85% in both the NSIP and COP patients. Conclusion: This survey provides helpful information for the management of IIP and to produce management guidelines for this illness in Korea.

연구배경: 과거 국내외 특발성 간질성 폐렴(IIP)의 역학 조사는 제한적이었다. 간질성 폐질환에 대한 관심이 증가하고 있고 국내 IIP 환자들의 분포 및 역학에 대해서 알려진 바 없어 이를 알아보고자 하였다. 방 법: 2003년 1월 1일부터 2007년 12월 31일 사이에 진단된 환자들을 대상으로 하였으며, 진단은 ATS/ERS 기준에 따랐다. 결 과: 입력된 환자는 3,156명이었으나, 중복, 진단기준 미달, 부정확한 자료로 970명이 제외되어 총 2,186명을 분석하였다. 남녀 비는 약 2 : 1이었고 이들의 평균 나이는 65세(11-94세)였다. 빈도는 특발성 폐 섬유화증(IPF) 1,685명(77.1%), 비특이적 간질성 폐렴(NSIP) 261명(11.9%), 특발성 기질화 폐렴(COP) 186명(8.5%), 급성 간질성 폐렴(AIP) 24명(1.1%), 박리성 간질성 폐렴(DIP) 19명(0.9%), 호흡성 세기관지염-간질성 폐질환(RB-ILD) 9명(0.4%), 림프구성 간질성 폐렴(LIP) 2명(0.1%)였다. 평균 나이는 IPF 67.8세, COP 57.7세, NSIP 57.1세로 IPF 환자의 나이가 가장 많았다. 전체 환자를 대상으로 하였을 때, 운동 시 호흡곤란은 68.9%, 기침 60.8%, 객담 32.5%, 흉통 6.8%, 객혈 2.2%, 무증상 4.5%로 운동 시 호흡곤란의 증상이 가장 많았다. IPF의 3년 생존율은 62%였고, NSIP와 COP의 5년 생존율은 모두 85%였다. 결 론: 이 조사는 국내의 IIP 치료 및 진료 지침을 제작에 유용한 도움을 줄 것으로 판단된다.

Keywords

References

  1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Class ification 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
  2. Xaubet A, Ancochea J, Morell F, Rodriguez-Arias JM, Villena V, Blanquer R, et al. Report on the incidence of interstitial lung diseases in Spain. Sarcoidosis Vasc Diffuse Lung Dis 2004;21:64-70
  3. 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
  4. Roelandt M, Demedts M, Callebaut W, Coolen D, Slabbynck H, Bockaert J, et al. Epidemiology of interstitial lung disease (ILD) in flanders: registration by pneumologists in 1992-1994. Working group on ILD, VRGT. Vereniging voor Respiratoire Gezondheidszorg en Tuberculosebestrijding. Acta Clin Belg 1995;50: 260-8 https://doi.org/10.1080/17843286.1995.11718459
  5. Schweisfurth H, Kieslich C, Satake N, Loddenkemper R, Schonfeld N, Mader I, et al. How are interstitial lung diseases diagnosed in Germany? Results of the scientific registry for the exploration of interstitial lung diseases ('Fibrosis registry') of the WATL. Pneumologie 2003;57:373-82 https://doi.org/10.1055/s-2003-40557
  6. Tinelli C, De Silvestri A, Richeldi L, Oggionni T. The Italian register for diffuse infiltrative lung disorders (RIPID): a four-year report. Sarcoidosis Vasc Diffuse Lung Dis 2005;22 Suppl 1:S4-8
  7. 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
  8. Flint A, Martinez FJ, Young ML, Whyte RI, Toews GB, Lynch JP 3rd. Influence of sample number and biopsy site on the histologic diagnosis of diffuse lung disease. Ann Thorac Surg 1995;60:1605-7; discussion 1607-8 https://doi.org/10.1016/0003-4975(95)00895-0
  9. Nagai S, Kitaichi M, Itoh H, Nishimura K, Izumi T, Colby TV. Idiopathic nonspecific interstitial pneumonia/ fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP. Eur Respir J 1998;12:1010-9 https://doi.org/10.1183/09031936.98.12051010
  10. Flaherty KR, Travis WD, Colby TV, Toews GB, Kazerooni EA, Gross BH, et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med 2001;164:1722-7 https://doi.org/10.1164/ajrccm.164.9.2103074
  11. Enomoto T, Usuki J, Azuma A, Nakagawa T, Kudoh S. Diabetes mellitus may increase risk for idiopathic pulmonary fibrosis. Chest 2003;123:2007-11 https://doi.org/10.1378/chest.123.6.2007
  12. Bouros D, Hatzakis K, Labrakis H, Zeibecoglou K. Association of malignancy with diseases causing interstitial pulmonary changes. Chest 2002;121:1278-89 https://doi.org/10.1378/chest.121.4.1278
  13. Ma Y, Seneviratne CK, Koss M. Idiopathic pulmonary fibrosis and malignancy. Curr Opin Pulm Med 2001; 7:278-82 https://doi.org/10.1097/00063198-200109000-00005
  14. Kawasaki H, Nagai K, Yokose T, Yoshida J, Nishimura M, Takahashi K, et al. Clinicopathological characteristics of surgically resected lung cancer associated with idiopathic pulmonary fibrosis. J Surg Oncol 2001; 76:53-7 https://doi.org/10.1002/1096-9098(200101)76:1<53::AID-JSO1009>3.0.CO;2-T
  15. Oshikawa K, Sugiyama Y. Serum anti-p53 autoantibodies from patients with idiopathic pulmonary fibrosis associated with lung cancer. Respir Med 2000; 94:1085-91 https://doi.org/10.1053/rmed.2000.0917
  16. Uematsu K, Yoshimura A, Gemma A, Mochimaru H, Hosoya Y, Kunugi S, et al. Aberrations in the fragile histidine triad (FHIT) gene in idiopathic pulmonary fibrosis. Cancer Res 2001;61:8527-33
  17. Wells C, Mannino DM. Pulmonary fibrosis and lung cancer in the United States: analysis of the multiple cause of death mortality data, 1979 through 1991. South Med J 1996;89:505-10 https://doi.org/10.1097/00007611-199605000-00012
  18. Desai SR, Ryan SM, Colby TV. Smoking-related interstitial lung diseases: histopathological and imaging perspectives. Clin Radiol 2003;58:259-68 https://doi.org/10.1016/S0009-9260(02)00525-1
  19. Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1997;155:242-8 https://doi.org/10.1164/ajrccm.155.1.9001319
  20. Travis WD, Hunninghake G, King TE Jr, Lynch DA, Colby TV, Galvin JR, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med 2008; 177:1338-47 https://doi.org/10.1164/rccm.200611-1685OC
  21. Lynch DA, Newell JD, Logan PM, King TE Jr, Müller NL. Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis? AJR Am J Roent genol 1995;165:807-11 https://doi.org/10.2214/ajr.165.4.7676971
  22. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000;161:646-64 https://doi.org/10.1164/ajrccm.161.2.ats3-00
  23. Riha RL, Duhig EE, Clarke BE, Steele RH, Slaughter RE, Zimmerman PV. Survival of patients with biopsy- proven usual interstitial pneumonia and nonspecific interstitial pneumonia. Eur Respir J 2002;19:1114-8 https://doi.org/10.1183/09031936.02.00244002
  24. Flaherty KR, Toews GB, Travis WD, Colby TV, Kazerooni EA, Gross BH, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J 2002;19:275-83 https://doi.org/10.1183/09031936.02.00182002
  25. Jeon K, Chung MP, Lee KS, Chung MJ, Han J, Koh WJ, et al. Prognostic factors and causes of death in Korean patients with idiopathic pulmonary fibrosis. Respir Med 2006;100:451-7 https://doi.org/10.1016/j.rmed.2005.06.013
  26. Jegal Y, Kim DS, Shim TS, Lim CM, Do Lee S, Koh Y, et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005;171:639-44 https://doi.org/10.1164/rccm.200403-331OC
  27. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar HD, Schroeder DR, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;157:199-203 https://doi.org/10.1164/ajrccm.157.1.9704130
  28. Thomeer MJ, Costabe U, Rizzato G, Poletti V, Demedts M. Comparison of registries of interstitial lung diseases in three European countries. Eur Respir J 2001;32:114-8s
  29. Hodgson U, Laitinen T, Tukiainen P. Nationwide prevalence of sporadic and familial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland. Thorax 2002;57:338-42 https://doi.org/10.1136/thorax.57.4.338
  30. Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: clinical features and their influence on survival. Thorax 1980;35:171-80 https://doi.org/10.1136/thx.35.3.171
  31. Mapel DW, Hunt WC, Utton R, Baumgartner KB, Samet JM, Coultas DB. Idiopathic pulmonary fibrosis: survival in population based and hospital based cohorts. Thorax 1998;53:469-76 https://doi.org/10.1136/thx.53.6.469
  32. Hubbard R, Johnston I, Britton J. Survival in patients with cryptogenic fibrosing alveolitis: a populationbased cohort study. Chest 1998;113:396-400 https://doi.org/10.1378/chest.113.2.396
  33. Hubbard R, Johnston I, Coultas DB, Britton J. Mortality rates from cryptogenic fibrosing alveolitis in seven countries. Thorax 1996;51:711-6 https://doi.org/10.1136/thx.51.7.711
  34. King TE Jr, Tooze JA, Schwarz MI, Brown KR, Cherniack RM. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med 2001;164:1171-81 https://doi.org/10.1164/ajrccm.164.7.2003140
  35. Schwartz DA, Merchant RK, Helmers RA, Gilbert SR, Dayton CS, Hunninghake GW. The influence of cigarette smoking on lung function in patients with idiopathic pulmonary fibrosis. Am Rev Respir Dis 1991;144:504-6 https://doi.org/10.1164/ajrccm/144.3_Pt_1.504
  36. Panos RJ, Mortenson RL, Niccoli SA, King TE Jr. Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med 1990;88:396-404 https://doi.org/10.1016/0002-9343(90)90495-Y
  37. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994;18:136-47 https://doi.org/10.1097/00000478-199402000-00003
  38. Nicholson AG, Colby TV, du Bois RM, Hansell DM, Wells AU. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 2000;162:2213-7 https://doi.org/10.1164/ajrccm.162.6.2003049
  39. Travis WD, Matsui K, Moss J, Ferrans VJ. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol 2000;24:19-33 https://doi.org/10.1097/00000478-200001000-00003
  40. Bellomo R, Finlay M, McLaughlin P, Tai E. Clinical spectrum of cryptogenic organising pneumonitis. Thorax 1991;46:554-8 https://doi.org/10.1136/thx.46.8.554
  41. Yousem SA, Colby TV, Gaensler EA. Respiratory bronchiolitis- associated interstitial lung disease and its relationship to desquamative interstitial pneumonia. Mayo Clin Proc 1989;64:1373-80 https://doi.org/10.1016/S0025-6196(12)65379-8
  42. Johkoh T, Muller NL, Pickford HA, Hartman TE, Ichikado K, Akira M, et al. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology 1999;212:567-72 https://doi.org/10.1148/radiology.212.2.r99au05567
  43. Vourlekis JS, Brown KK, Cool CD, Young DA, Cherniack RM, King TE, et al. Acute interstitial pneumonitis. Case series and review of the literature. Medicine (Baltimore) 2000;79:369-78 https://doi.org/10.1097/00005792-200011000-00003

Cited by

  1. 다발성 경화 소견으로 발현된 림프구성 간질성 폐렴 1예 vol.67, pp.1, 2009, https://doi.org/10.4046/trd.2009.67.1.37
  2. 일차성 Sjogren씨 증후군에서 발생한 림프구성 간질성 폐렴 1예: 증례보고와 국내문헌고찰 vol.69, pp.5, 2010, https://doi.org/10.4046/trd.2010.69.5.375
  3. 간질성 폐질환 vol.71, pp.3, 2011, https://doi.org/10.4046/trd.2011.71.3.163
  4. ADAM33 Gene Polymorphisms are Associated with the Risk of Idiopathic Pulmonary Fibrosis vol.192, pp.4, 2009, https://doi.org/10.1007/s00408-014-9578-5
  5. Management of Idiopathic Interstitial Pneumonia Treated only with Traditional Korean Medicine: a Case Report vol.35, pp.4, 2009, https://doi.org/10.13048/jkm.14048
  6. 가습기살균제 참사의 진행과 교훈(Q&A) vol.43, pp.1, 2017, https://doi.org/10.5668/jehs.2017.43.1.1
  7. Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 3. Idiopathic Nonspecific Interstitial Pneumonia vol.82, pp.4, 2009, https://doi.org/10.4046/trd.2018.0092