What Every Radiologist Should Know about Idiopathic Interstitial Pneumonias
Abstract
The American Thoracic Society–European Respiratory Society classification of idiopathic interstitial pneumonias (IIPs), published in 2002, defines the morphologic patterns on which clinical-radiologic-pathologic diagnosis of IIPs is based. IIPs include seven entities: idiopathic pulmonary fibrosis, which is characterized by the morphologic pattern of usual interstitial pneumonia (UIP); nonspecific interstitial pneumonia (NSIP); cryptogenic organizing pneumonia (COP); respiratory bronchiolitis–associated interstitial lung disease (RB-ILD); desquamative interstitial pneumonia (DIP); lymphoid interstitial pneumonia (LIP); and acute interstitial pneumonia (AIP). The characteristic computed tomographic findings in UIP are predominantly basal and peripheral reticular opacities with honeycombing and traction bronchiectasis. In NSIP, basal ground-glass opacities tend to predominate over reticular opacities, with traction bronchiectasis only in advanced disease. COP is characterized by patchy peripheral or peribronchovascular consolidation. RB-ILD and DIP are smoking-related diseases characterized by centrilobular nodules and ground-glass opacities. LIP is characterized by ground-glass opacities, often in combination with cystic lesions. AIP manifests as diffuse lung consolidation with ground-glass opacities, which usually progress to fibrosis in patients who survive the acute phase of the disease. Correct diagnosis of IIPs can be achieved only by means of interdisciplinary consensus and stringent correlation of clinical, imaging, and pathologic findings.
© RSNA, 2007
References
- 1
, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med1998; 157: 1301–1315.Katzenstein AL - 2
. New concepts and entities in pulmonary disease. Monogr Pathol1968; 8: 322–365.Liebow A - 3 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. [Published correction appears in Am J Respir Crit Care Med 2002;166:426.]
- 4
Jr, Schwarz MI, Brown K, et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med2001; 164: 1025–1032.King TE - 5
, Wells AU. Cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis. Eur Respir J Suppl2001; 32: 43s–55s.du Bois RM - 6
, Franquet T, Gimenez A, Bordes R, Pineda R, Madrid M. Smoking-related interstitial lung diseases: radiologic-pathologic correlation. Eur Radiol2006; 16: 2463–2470.Hidalgo A - 7
, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease? Proc Am Thorac Soc2006; 3: 293–298.Taskar VS - 8
, Aguayo SM, Roman J. Idiopathic pulmonary fibrosis: a practical approach for diagnosis and management. Chest2000; 118: 788–794.Michaelson JE - 9
, Sakamoto S, Kawabata M, et al. Cyclosporin treatment in steroid-resistant and acutely exacerbated interstitial pneumonia. Intern Med2005; 44: 1144–1150.Homma S - 10
, Mal H, Castier Y, et al. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis. J Thorac Cardiovasc Surg2003; 126: 469–475.Thabut G - 11
, Wells AU, Colby TV, du Bois RM, Hansell DM, Nicholson AG. Prognostic implications of histologic patterns in multiple surgical lung biopsies from patients with idiopathic interstitial pneumonias. Chest2004; 125: 522–526.Monaghan H - 12
, Travis WD, Colby TV, et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med2001; 164: 1722–1727.Flaherty KR - 13
, Miller RR, Webb WR, Evans KG, Ostrow DN. Fibrosing alveolitis: CT-pathologic correlation. Radiology1986; 160: 585–588.Muller NL - 14
, Shin MS, Friedman SE, Myers JL, Katzenstein AL. Radiographic manifestations of bronchiolitis obliterans with organizing pneumonia vs usual interstitial pneumonia. AJR Am J Roentgenol1986; 147: 899–906.Chandler PW - 15
, Lynch DA, Galvin JR, et al. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Chest2003; 124: 1215–1223.Hunninghake GW - 16
, Muller NL, Cartier Y, et al. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology1999; 211: 555–560.Johkoh T - 17
, Rubens MB, Hansell DM, et al. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology2001; 221: 600–605.MacDonald SL - 18
, Mageto YN, Lockhart D, Schmidt RA, Wood DE, Godwin JD. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study. Chest1999; 116: 1168–1174.Raghu G - 19
, Zimmerman MB, Schwartz DA, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med2001; 164: 193–196.Hunninghake GW - 20
, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax2003; 58: 143–148.Flaherty KR - 21
, 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 Pathol2000; 24: 19–33.Travis WD - 22
, 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 J2002; 19: 1114–1118.Riha RL - 23
, du Bois RM, Nicholson AG, et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med2003; 168: 531–537.Latsi PI - 24
. Idiopathic interstitial pneumonias: usual interstitial pneumonia versus nonspecific interstitial pneumonia. Proc Am Thorac Soc2006; 3: 81–95.Martinez FJ - 25
, Gilchrist FC, Nicholson AG, et al. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med1999; 160: 899–905.Daniil ZD - 26
, Lee KS, Johkoh T, et al. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. RadioGraphics2002; 22(spec no): S151–S165.Kim EA - 27
, Erasmus JJ, McAdams HP, Sporn TA, Goodman PC. Pulmonary drug toxicity: radiologic and pathologic manifestations. RadioGraphics2000; 20: 1245–1259.Rossi SE - 28
, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis: histologic features and clinical significance. Am J Surg Pathol1994; 18: 136–147.Katzenstein AL - 29
, Muller NL, Colby TV, et al. Nonspecific interstitial pneumonia: correlation between thin-section CT findings and pathologic subgroups in 55 patients. Radiology2002; 225: 199–204.Johkoh T - 30
, Inoue G, Yamamoto S, Sakatani M. Non-specific interstitial pneumonia: findings on sequential CT scans of nine patients. Thorax2000; 55: 854–859.Akira M - 31
, Lee KS, Chung MP, Kwon OJ, Kim TS, Hwang JH. Nonspecific interstitial pneumonia with fibrosis: serial high-resolution CT findings with functional correlation. AJR Am J Roentgenol1999; 173: 949–953.Kim EY - 32
, Desai SR, Rubens MB, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med2003; 167: 962–969.Wells AU - 33
, Veeraraghavan S, Hansell DM, et al. CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology2004; 232: 560–567.Desai SR - 34
, Lee JS, Colby TV, Kitaichi M, Kim DS. Nonspecific interstitial pneumonia versus usual interstitial pneumonia: differences in the density histogram of high-resolution CT. J Comput Assist Tomogr2005; 29: 544–548.Do KH - 35
, Giraud F, Remy J, Copin MC, Gosselin B, Duhamel A. Importance of ground-glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation. Radiology1993; 189: 693–698.Remy-Jardin M - 36
. Organising pneumonia. Thorax2000; 55: 318–328.Cordier JF - 37
, Loire R, Brune J. Idiopathic bronchiolitis obliterans organizing pneumonia: definition of characteristic clinical profiles in a series of 16 patients. Chest1989; 96: 999–1004.Cordier JF - 38
, Vandevenne A, Pelletier A, Leclerc P, Court-Fortune I, Cordier JF. Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. The Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P). Am J Respir Crit Care Med2000; 162: 571–577.Lazor R - 39
, Colby TV. Pathologic manifestations of bronchiolitis, constrictive bronchiolitis, cryptogenic organizing pneumonia, and diffuse panbronchiolitis. Clin Chest Med1993; 14: 611–622.Myers JL - 40
, Guerry-Force ML, Staples CA, et al. Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. Radiology1987; 162: 151–156.Muller NL - 41
, Kullnig P, Hartman TE, Muller NL. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR Am J Roentgenol1994; 162: 543–546.Lee KS - 42
, Kitaichi M, Nishimura K, Nagai S. Bronchiolitis obliterans organizing pneumonia: clinical features and differential diagnosis. Chest1992; 102: 715–719.Izumi T - 43
, Yamamoto S, Sakatani M. Bronchiolitis obliterans organizing pneumonia manifesting as multiple large nodules or masses. AJR Am J Roentgenol1998; 170: 291–295.Akira M - 44
, Kuhlman JE, Ball WC Jr, Hruban RH, Askin FB, Siegelman SS. CT findings in bronchiolitis obliterans organizing pneumonia (BOOP) with radiographic, clinical, and histologic correlation. J Comput Assist Tomogr1993; 17: 352–357.Bouchardy LM - 45
, Vizcaya M, Texido A, Aguilar X, Arevalo M. Idiopathic bronchiolitis obliterans organizing pneumonia with multiple cavitary lung nodules. Eur Respir J1995; 8: 1975–1977.Haro M - 46
, du Bois RM, Colby TV, Hansell DM, Nicholson AG. Clinical significance of respiratory bronchiolitis on open lung biopsy and its relationship to smoking related interstitial lung disease. Thorax1999; 54: 1009–1014.Moon J - 47
, Ward S, Lynch DA, Remy-Jardin M, Johkoh T, Muller NL. Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process? AJR Am J Roentgenol1999; 173: 1617–1622.Heyneman LE - 48
, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest2005; 127: 178–184.Ryu JH - 49
, Veal CF Jr, Shin MS, Katzenstein AL. Respiratory bronchiolitis causing interstitial lung disease: a clinicopathologic study of six cases. Am Rev Respir Dis1987; 135: 880–884.Myers JL - 50
, Colby TV, Hartman TE, Vassallo R. Smoking-related interstitial lung diseases: a concise review. Eur Respir J2001; 17: 122–132.Ryu JH - 51
, Remy J, Boulenguez C, Sobaszek A, Edme JL, Furon D. Morphologic effects of cigarette smoking on airways and pulmonary parenchyma in healthy adult volunteers: CT evaluation and correlation with pulmonary function tests. Radiology1993; 186: 107–115.Remy-Jardin M - 52
, Hansell DM, Wells AU, Nicholson AG, Flint JD, Muller NL. Follicular bronchiolitis: thin-section CT and histologic findings. Radiology1999; 212: 637–642.Howling SJ - 53
, Ko GJ, Kim JY, et al. Desquamative interstitial pneumonia associated with concurrent cytomegalovirus and Aspergillus pneumonia in a renal transplant recipient. Nephrol Dial Transplant2005; 20: 635–638.Sung SA - 54
, Kuhn C 3rd, Ely EW, et al. Flock worker’s lung: broadening the spectrum of clinico-pathology, narrowing the spectrum of suspected etiologies. Chest2000; 117: 251–259.Kern DG - 55
, Colby TV, Gaensler EA. Respiratory bronchiolitis-associated interstitial lung disease and its relationship to desquamative interstitial pneumonia. Mayo Clin Proc1989; 64: 1373–1380.Yousem SA - 56
, Shreesha U, Savici D, Katzenstein AL. Respiratory bronchiolitis: a clinicopathologic study in current smokers, ex-smokers, and never-smokers. Am J Surg Pathol2002; 26: 647–653.Fraig M - 57
, Friedman PJ. Chest radiography in desquamative interstitial pneumonitis: a review of 37 patients. AJR Am J Roentgenol1980; 134: 91–99.Feigin DS - 58
, Travis WD, Muller NL, et al. Idiopathic interstitial pneumonias: CT features. Radiology2005; 236: 10–21.Lynch DA - 59
, Yamamoto S, Hara H, Sakatani M, Ueda E. Serial computed tomographic evaluation in desquamative interstitial pneumonia. Thorax1997; 52: 333–337.Akira M - 60
, Berry GJ, Raffin TA, Kuschner WG. Lymphoid interstitial pneumonia: a narrative review. Chest2002; 122: 2150–2164.Swigris JJ - 61
, Ahmad D. Malignant lymphoma complicating lymphocytic interstitial pneumonia: a monoclonal B-cell neoplasm arising in a polyclonal lymphoproliferative disorder. Hum Pathol1982; 13: 780–782.Banerjee D - 62
, Hyjek E, Isaacson PG. Primary pulmonary lymphoma: a re-appraisal of its histogenesis and its relationship to pseudolymphoma and lymphoid interstitial pneumonia. Histopathology1988; 13: 1–17.Addis BJ - 63
, Muller NL, Pickford HA, et al. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology1999; 212: 567–572.Johkoh T - 64
, Kinoshita M, Koga T, Oizumi K, Fujimoto K, Hayabuchi N. Lung cyst formation in lymphocytic interstitial pneumonia: CT features. J Comput Assist Tomogr1994; 18: 745–748.Ichikawa Y - 65
, Hamada H, Sakatani M, Kobayashi C, Nishioka M, Yamamoto S. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. AJR Am J Roentgenol1997; 168: 79–83.Akira M - 66
, Brown KK, Cool CD, et al. Acute interstitial pneumonitis: case series and review of the literature. Medicine (Baltimore)2000; 79: 369–378.Vourlekis JS - 67
, Colby TV, Elliott CG. Hamman-Rich syndrome revisited. Mayo Clin Proc1990; 65: 1538–1548.Olson J - 68
, Katzenstein AL. Diffuse alveolar damage and recurrent respiratory failure: report of 6 cases. Hum Pathol2001; 32: 1398–1402.Savici D - 69
, Muller NL, Taniguchi H, et al. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology1999; 211: 859–863.Johkoh T - 70
, Cancellieri A, Chilosi M, et al. Acute interstitial pneumonia: report of a series. Eur Respir J2003; 21: 187–191.Bonaccorsi A - 71
. Computed tomography and pathologic findings in fulminant forms of idiopathic interstitial pneumonia. J Thorac Imaging1999; 14: 76–84.Akira M - 72
, Johkoh T, Ikezoe J, et al. Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AJR Am J Roentgenol1997; 168: 333–338.Ichikado K - 73
, Wells AU, Rubens MB, Evans TW, Hansell DM. Acute respiratory distress syndrome: CT abnormalities at long-term follow-up. Radiology1999; 210: 29–35.Desai SR