RSNA Education Exhibits

Nonspecific Interstitial Pneumonia: Radiologic, Clinical, and Pathologic Considerations

Published Online:Jan 1 2009https://doi.org/10.1148/rg.291085096

Abstract

Nonspecific interstitial pneumonia (NSIP) has variable clinical, pathologic, and radiologic manifestations. Cellular and fibrotic NSIP are the two main histologic subtypes and differ from one another in the degree of inflammation and fibrosis. It is important to differentiate NSIP from other diffuse lung diseases, especially usual interstitial pneumonia and hypersensitivity pneumonitis, owing to differences in prognosis and treatment. At high-resolution computed tomography, the most common findings suggestive of NSIP are lower lobe peripherally predominant ground-glass opacity with reticular abnormality, traction bronchiectasis, and lower lobe volume loss. Nodules, cysts, and areas of low attenuation are uncommon and should point one toward other diagnoses. Because many cases of NSIP are associated with collagen vascular diseases, it is important to look for associated findings that may suggest an underlying collagen vascular disease. Given the difficulty clinicians, pathologists, and radiologists experience in making the diagnosis of NSIP, a group approach in which these specialists work together to reach a consensus diagnosis has the highest likelihood of achieving the correct diagnosis.

References

1 CollardHR, King TE Jr. Demystifying idiopathic interstitial pneumonia. Arch Intern Med2003; 163: 17–29.
Medline Google Scholar
2 ElliotTL, Lynch DA, Newell JD Jr, et al. High-resolution computed tomography features of nonspecific interstitial pneumonia and usual interstitial pneumonia. J Comput Assist Tomogr2005; 29: 339–345.
Medline Google Scholar
3 JeongYJ, Lee KS, Muller NL, et al. Usual interstitial pneumonia and non-specific interstitial pneumonia: serial thin-section CT findings correlated with pulmonary function. Korean J Radiol2005; 6: 143–152.
Medline Google Scholar
4 JohkohT, Muller NL, Cartier Y, et al. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology1999; 211: 555–560.
Google Scholar
5 MacDonaldSL, 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.
Google Scholar
6 NagaiS, Kitaichi M, Itoh H, Nishimura K, Izumi T, Colby TV. Idiopathic nonspecific interstitial pneumonia/fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP. Eur Respir J1998; 12: 1010–1019.
Medline Google Scholar
7 NishiyamaO, Kondoh Y, Taniguchi H, et al. Serial high resolution CT findings in nonspecific interstitial pneumonia/fibrosis. J Comput Assist Tomogr2000; 24: 41–46.
Medline Google Scholar
8 SumikawaH, Johkoh T, Ichikado K, et al. Usual interstitial pneumonia and chronic idiopathic interstitial pneumonia: analysis of CT appearance in 92 patients. Radiology2006; 241: 258–266.
Google Scholar
9 TsubamotoM, Muller NL, Johkoh T, et al. Pathologic subgroups of nonspecific interstitial pneumonia: differential diagnosis from other idiopathic interstitial pneumonias on high-resolution computed tomography. J Comput Assist Tomogr2005; 29: 793–800.
Medline Google Scholar
10 CaminatiA, Harari S. Smoking-related interstitial pneumonias and pulmonary Langerhans cell histiocytosis. Proc Am Thorac Soc2006; 3: 299–306.
Medline Google Scholar
11 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.
Medline Google Scholar
12 KimEA, Lee KS, Johkoh T, et al. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. RadioGraphics2002; 22(spec issue): S151–S165.
Google Scholar
13 LynchDA, Travis WD, Muller NL, et al. Idiopathic interstitial pneumonias: CT features. Radiology2005; 236: 10–21.
Google Scholar
14 TravisWD, 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.
Medline Google Scholar
15 TravisWD, Hunninghake G, King TE Jr, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med2008; 177: 1338–1347.
Medline Google Scholar
16 FlahertyKR, Toews GB, Travis WD, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J2002; 19: 275–283.
Medline Google Scholar
17 JegalY, Kim DS, Shim TS, et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med2005; 171: 639–644.
Medline Google Scholar
18 NicholsonAG, 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 Med2000; 162: 2213–2217.
Medline Google Scholar
19 RubinowitzAN, Moon M, Homer R. A 34-year-old man presenting with gradual onset of shortness of breath and interstitial lung disease. Chest2008; 133: 1041–1047.
Medline Google Scholar
20 CottinV, Donsbeck AV, Revel D, Loire R, Cordier JF. Nonspecific interstitial pneumonia: individualization of a clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med1998; 158: 1286–1293.
Medline Google Scholar
21 KimTS, Lee KS, Chung MP, et al. Nonspecific interstitial pneumonia with fibrosis: high-resolution CT and pathologic findings. AJR Am J Roentgenol1998; 171: 1645–1650.
Medline Google Scholar
22 ParkCS, Jeon JW, Park SW, et al. Nonspecific interstitial pneumonia/fibrosis: clinical manifestations, histologic and radiologic features. Korean J Intern Med1996; 11: 122–132.
Medline Google Scholar
23 ParkJS, Lee KS, Kim JS, et al. Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology1995; 195: 645–648.
Google Scholar
24 HartmanTE, Swensen SJ, Hansell DM, et al. Nonspecific interstitial pneumonia: variable appearance at high-resolution chest CT. Radiology2000; 217: 701–705.
Google Scholar
25 JohkohT, 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.
Google Scholar
26 KimHY, Shim YM, Lee KS, Han J, Yi CA, Kim YK. Persistent pulmonary nodular ground-glass opacity at thin-section CT: histopathologic comparisons. Radiology2007; 245: 267–275.
Google Scholar
27 FlahertyKR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax2003; 58: 143–148.
Medline Google Scholar
28 KimEY, 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.
Medline Google Scholar
29 ParkIN, Kim DS, Shim TS, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest2007; 132: 214–220.
Medline Google Scholar
30 SilvaCI, Muller NL, Lynch DA, et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology2008; 246: 288–297.
Google Scholar
31 FlahertyKR, Travis WD, Colby TV, et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med2001; 164: 1722–1727.
Medline Google Scholar
32 NicholsonAG, Addis BJ, Bharucha H, et al. Inter-observer variation between pathologists in diffuse parenchymal lung disease. Thorax2004; 59: 500–505.
Medline Google Scholar
33 HunninghakeGW, Lynch DA, Galvin JR, et al. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Chest2003; 124: 1215–1223.
Medline Google Scholar
34 ScreatonNJ, Hiorns MP, Lee KS, et al. Serial high resolution CT in non-specific interstitial pneumonia: prognostic value of the initial pattern. Clin Radiol2005; 60: 96–104.
Medline Google Scholar
35 SilvaCI, 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. Radiology2008; 247: 251–259.
Google Scholar
36 SilvaCI, Flint JD, Levy RD, Muller NL. Diffuse lung cysts in lymphoid interstitial pneumonia: high- resolution CT and pathologic findings. J Thorac Imaging2006; 21: 241–244.
Medline Google Scholar
37 HartmanTE, Primack SL, Swensen SJ, Hansell D, McGuinness G, Muller NL. Desquamative interstitial pneumonia: thin-section CT findings in 22 patients. Radiology1993; 187: 787–790.
Google Scholar
38 FlahertyKR, King TE Jr, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med2004; 170: 904–910.
Medline Google Scholar

Article History

Published in print: Jan 2009

Vol. 29, No. 1

Metrics

Altmetric Score

PDF download