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Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 3. Idiopathic Nonspecific Interstitial Pneumonia

  • Lee, Jongmin (Division of Pulmonology and Allergy, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University School of Medicine) ;
  • Kim, Yong Hyun (Division of Allergy and Pulmonology, Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University School of Medicine) ;
  • Kang, Ji Young (Division of Pulmonology and Allergy, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University School of Medicine) ;
  • Jegal, Yangjin (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Ulsan University Hospital, Ulsan University College of Medicine) ;
  • Park, So Young (Department of Pulmonary and Critical Care Medicine, Chungnam National University Hospital) ;
  • Korean Interstitial Lung Diseases Study Group (Korean Interstitial Lung Diseases Study Group)
  • 투고 : 2018.12.21
  • 심사 : 2019.04.23
  • 발행 : 2019.10.31

초록

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.

키워드

참고문헌

  1. Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188:733-48. https://doi.org/10.1164/rccm.201308-1483ST
  2. 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
  3. 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
  4. 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
  5. Flaherty KR, Martinez FJ. Nonspecific interstitial pneumonia. Semin Respir Crit Care Med 2006;27:652-8. https://doi.org/10.1055/s-2006-957336
  6. Hyldgaard C, Hilberg O, Muller A, Bendstrup E. A cohort study of interstitial lung diseases in central Denmark. Respir Med 2014;108:793-9. https://doi.org/10.1016/j.rmed.2013.09.002
  7. Scientific Committee of the Korean Academy of Tuberculosis and Respiratory Diseases. 2008 National survey of idiopathic interstitial pneumonia in Korea. Tuberc Respir Dis 2009;66:141-51. https://doi.org/10.4046/trd.2009.66.2.141
  8. Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2018;198:e44-68. https://doi.org/10.1164/rccm.201807-1255ST
  9. Romagnoli M, Nannini C, Piciucchi S, Girelli F, Gurioli C, Casoni G, et al. Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders? Eur Respir J 2011;38:384-91. https://doi.org/10.1183/09031936.00094910
  10. Fujita J, Ohtsuki Y, Yoshinouchi T, Yamadori I, Bandoh S, Tokuda M, et al. Idiopathic non-specific interstitial pneumonia: as an "autoimmune interstitial pneumonia". Respir Med 2005;99:234-40. https://doi.org/10.1016/j.rmed.2004.07.001
  11. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, et al. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med 2007;176:691-7. https://doi.org/10.1164/rccm.200702-220OC
  12. Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, Lee SD, et al. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 2002;19:121-7.
  13. Collins B, Raghu G. Interstitial pneumonia with autoimmune features: the new consensus-based definition for this cohort of patients should be broadened. Eur Respir J 2016;47:1293-5. https://doi.org/10.1183/13993003.02084-2015
  14. Silva CI, Muller NL, Hansell DM, Lee KS, Nicholson AG, Wells AU. Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. Radiology 2008;247:251-9. https://doi.org/10.1148/radiol.2471070369
  15. Hartman TE, Swensen SJ, Hansell DM, Colby TV, Myers JL, Tazelaar HD, et al. Nonspecific interstitial pneumonia: variable appearance at high-resolution chest CT. Radiology 2000;217:701-5. https://doi.org/10.1148/radiology.217.3.r00nv31701
  16. Bna C, Zompatori M, Poletti V, Spaggiari E, Chetta A, Calabro E, et al. Differential diagnosis between usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP) assessed by high-resolution computed tomography (HRCT). Radiol Med 2005;109:472-87.
  17. Ryu YJ, Chung MP, Han J, Kim TS, Lee KS, Chun EM, et al. Bronchoalveolar lavage in fibrotic idiopathic interstitial pneumonias. Respir Med 2007;101:655-60. https://doi.org/10.1016/j.rmed.2006.06.003
  18. Veeraraghavan S, Latsi PI, Wells AU, Pantelidis P, Nicholson AG, Colby TV, et al. BAL findings in idiopathic nonspecific interstitial pneumonia and usual interstitial pneumonia. Eur Respir J 2003;22:239-44. https://doi.org/10.1183/09031936.03.00105202
  19. Leslie KO. Historical perspective: a pathologic approach to the classification of idiopathic interstitial pneumonias. Chest 2005;128(5 Suppl 1):513S-9S. https://doi.org/10.1378/chest.128.5_suppl_1.513S
  20. 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
  21. Kligerman SJ, Groshong S, Brown KK, Lynch DA. Nonspecific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics 2009;29:73-87. https://doi.org/10.1148/rg.291085096
  22. Wells AU, Kokosi M, Karagiannis K. Treatment strategies for idiopathic interstitial pneumonias. Curr Opin Pulm Med 2014;20:442-8. https://doi.org/10.1097/MCP.0000000000000085
  23. Park IN, Jegal Y, Kim DS, Do KH, Yoo B, Shim TS, et al. Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J 2009;33:68-76. https://doi.org/10.1183/09031936.00158507
  24. Kim YJ, Park JW, Kyung SY, Lee SP, Chung MP, Kim YH, et al. Clinical characteristics of idiopathic pulmonary fibrosis patients with diabetes mellitus: the national survey in Korea from 2003 to 2007. J Korean Med Sci 2012;27:756-60. https://doi.org/10.3346/jkms.2012.27.7.756
  25. Watanabe K, Higuchi K, Ninomiya K, Ohshima T, Aritomi T, Kawabata Y, et al. Steroid treatment based on the findings of transbronchial biopsy in idiopathic interstitial pneumonia. Eur Respir J 2002;20:1213-9. https://doi.org/10.1183/09031936.02.00269302
  26. Tomassetti S, Ryu JH, Piciucchi S, Chilosi M, Poletti V. Nonspecific interstitial pneumonia: what is the optimal approach to management? Semin Respir Crit Care Med 2016;37:378-94. https://doi.org/10.1055/s-0036-1583176
  27. 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
  28. Nanki N, Fujita J, Yamaji Y, Maeda H, Kurose T, Kaji M, et al. Nonspecific interstitial pneumonia/fibrosis completely recovered by adding cyclophosphamide to corticosteroids. Intern Med 2002;41:867-70. https://doi.org/10.2169/internalmedicine.41.867
  29. Xu W, Xiao Y, Liu H, Qin M, Zheng W, Shi J. Nonspecific interstitial pneumonia: clinical associations and outcomes. BMC Pulm Med 2014;14:175. https://doi.org/10.1186/1471-2466-14-175
  30. Kinder BW. Nonspecific interstitial pneumonia. Clin Chest Med 2012;33:111-21. https://doi.org/10.1016/j.ccm.2011.11.003
  31. Kondoh Y, Taniguchi H, Yokoi T, Nishiyama O, Ohishi T, Kato T, et al. Cyclophosphamide and low-dose prednisolone in idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia. Eur Respir J 2005;25:528-33. https://doi.org/10.1183/09031936.05.00071004
  32. Corte TJ, Ellis R, Renzoni EA, Hansell DM, Nicholson AG, du Bois RM, et al. Use of intravenous cyclophosphamide in known or suspected, advanced non-specific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 2009;26:132-8.
  33. Keir GJ, Maher TM, Ming D, Abdullah R, de Lauretis A, Wickremasinghe M, et al. Rituximab in severe, treatment-refractory interstitial lung disease. Respirology 2014;19:353-9. https://doi.org/10.1111/resp.12214
  34. Poletti V, Romagnoli M, Piciucchi S, Chilosi M. Current status of idiopathic nonspecific interstitial pneumonia. Semin Respir Crit Care Med 2012;33:440-9. https://doi.org/10.1055/s-0032-1325155
  35. Latsi PI, du Bois RM, Nicholson AG, Colby TV, Bisirtzoglou D, Nikolakopoulou A, et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003;168:531-7. https://doi.org/10.1164/rccm.200210-1245OC
  36. Jegal Y, Kim DS, Shim TS, Lim CM, Lee SD, 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
  37. Belloli EA, Beckford R, Hadley R, Flaherty KR. Idiopathic nonspecific interstitial pneumonia. Respirology 2016;21:259-68. https://doi.org/10.1111/resp.12674
  38. Troy L, Corte TJ. Management of the idiopathic interstitial pneumonias. Aust Prescr 2012;35:202-6. https://doi.org/10.18773/austprescr.2012.087
  39. 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
  40. Park IN, Kim DS, Shim TS, Lim CM, Lee SD, Koh Y, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest 2007;132:214-20. https://doi.org/10.1378/chest.07-0323
  41. Sato T, Fujita J, Yamadori I, Ohtsuki Y, Yoshinouchi T, Bandoh S, et al. Non-specific interstitial pneumonia; as the first clinical presentation of various collagen vascular disorders. Rheumatol Int 2006;26:551-5. https://doi.org/10.1007/s00296-005-0028-9
  42. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J 2015;46:976-87. https://doi.org/10.1183/13993003.00150-2015

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