CT Features of Lung Disease in Patients with Systemic Sclerosis: Comparison with Idiopathic Pulmonary Fibrosis and Nonspecific Interstitial Pneumonia

PURPOSE: To evaluate computed tomographic (CT) patterns of lung disease in patients with systemic sclerosis (SSc) and compare them with CT appearance in patients with biopsy-proved idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP).

MATERIALS AND METHODS: The CT features of consecutive patients with SSc (n = 225; male patients, 44; female patients, 181; median age, 47 years; age range, 16–78 years), IPF (n = 40; men, 26; women, 14; median age, 54.5 years; age range, 36–77 years) and NSIP (n = 27; men, 18; women, nine; median age, 53 years; age range, 32–68 years) were quantified separately by two observers. The extent of interstitial lung disease, ground-glass opacification, emphysema, and the coarseness of a reticular pattern were quantified. Group comparisons were made nonparametrically with the Wilcoxon rank sum test. Differences in CT features were identified with multiple logistic regression analysis.

RESULTS: The coarseness of fibrosis was similar in patients with SSc and idiopathic NSIP but strikingly different between patients with SSc (median coarseness score, 5.5; range, 0.0–13.3) and IPF (median coarseness score, 8.8; range, 2.5–15.0) (P < .001). The proportion of ground-glass opacification at CT was similar in patients with SSc and idiopathic NSIP but differed significantly between patients with SSc (median proportion, 49.9%; range, 0.0%–100.0%) and IPF (median proportion, 23.5%; range, 0.0%–97.2%) (P < .001). At logistic regression analysis, there were no differences in the CT features between patients with SSc and those with NSIP after controlling for age, disease extent, and the percentage predicted forced vital capacity and carbon monoxide diffusing capacity.

CONCLUSION: Interstitial lung disease in patients with SSc is less extensive, less coarse, and characterized by a greater proportion of ground-glass opacification than that in patients with IPF. The CT features of lung disease in patients with SSc closely resemble those in patients with idiopathic NSIP.

© RSNA, 2004


  • 1 Piper WN, Helwig EB. Progressive systemic sclerosis: visceral manifestations in generalized scleroderma. AMA Arch Derm 1955; 72:535-546. Crossref, MedlineGoogle Scholar
  • 2 D’Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma): a study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med 1969; 46:428-440. Crossref, MedlineGoogle Scholar
  • 3 Harrison NK, Myers AR, Corrin B, et al. Structural features of interstitial lung disease in systemic sclerosis. Am Rev Respir Dis 1991; 144:706-713. Crossref, MedlineGoogle Scholar
  • 4 American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment—international consensus statement. American Thoracic Society (ATS) and the European Respiratory Society (ERS). I. Am J Respir Crit Care Med 2000; 161:646-664. Google Scholar
  • 5 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. Crossref, MedlineGoogle Scholar
  • 6 Fujita J, Yoshinouchi T, Ohtsuki Y, et al. Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis. Ann Rheum Dis 2001; 60:281-283. Crossref, MedlineGoogle Scholar
  • 7 Kim DS, Yoo B, Lee JS, et al. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 2002; 19:121-127. MedlineGoogle Scholar
  • 8 Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 2002; 165:1581-1586. Crossref, MedlineGoogle Scholar
  • 9 Hunninghake GW, Zimmerman MB, Schwartz DA, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001; 164:193-196. Crossref, MedlineGoogle Scholar
  • 10 Cherniack RM, Colby TV, Flint A, et al. Co-operative group steering committee, 1991: quantitative assessment of lung pathology in idiopathic pulmonary fibrosis. Am Rev Respir Dis 1991; 144:892-900. Crossref, MedlineGoogle Scholar
  • 11 Ashcroft T, Simpson JM, Timbrell V. Simple method of estimating severity of pulmonary fibrosis on a numerical scale. J Clin Pathol 1988; 41:467-470. Crossref, MedlineGoogle Scholar
  • 12 MacDonald SL, Rubens MB, Hansell DM, et al. Non-specific interstitial pneumonitis and usual interstitial pneumonitis: comparative appearances and diagnostic accuracy of high resolution computed tomography. Radiology 2001; 221:600-605. LinkGoogle Scholar
  • 13 Park JS, Lee KS, Kim JS, et al. Non-specific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology 1995; 195:645-648. LinkGoogle Scholar
  • 14 Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23:581-590. Crossref, MedlineGoogle Scholar
  • 15 Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: clinical features and their influence on survival. Thorax 1980; 35:171-180. Crossref, MedlineGoogle Scholar
  • 16 Austin JH, Müller NL, Friedman PJ, et al. Glossary of terms for computed tomography of the lungs: recommendations of the nomenclature committee of the Fleischner society. Radiology 1996; 200:327-331. LinkGoogle Scholar
  • 17 Müller NL, Miller RR, Webb WR, Evans KG, Ostrow DN. Fibrosing alveolitis: CT-pathologic correlation. Radiology 1986; 160:585-588. LinkGoogle Scholar
  • 18 Müller NL, Staples CA, Miller RR, Vedal S, Thurlbeck WM, Ostrow DN. Disease activity in idiopathic pulmonary fibrosis: CT and pathologic correlation. Radiology 1987; 165:731-734. LinkGoogle Scholar
  • 19 Collins CD, Wells AU, Hansell DM, et al. Observer variation in pattern type and extent of disease in fibrosing alveolitis on thin section computed tomography and chest radiography. Clin Radiol 1994; 49:236-240. Crossref, MedlineGoogle Scholar
  • 20 Wells AU, Hansell DM, Corrin B, et al. High resolution computed tomography as a predictor of lung histology in systemic sclerosis. Thorax 1992; 47:738-742. Crossref, MedlineGoogle Scholar
  • 21 Streiner DL, Norman GR. In: Streiner DL, Norman GR, eds Health measurement scales: a practical guide to the development and use. Oxford, England: Oxford University Press, 1989; 94-95. Google Scholar
  • 22 Brennan P, Silman A. Statistical methods for assessing observer variability in clinical measures. BMJ 1992; 304:1491-1494. Crossref, MedlineGoogle Scholar
  • 23 Chinn S. Repeatability and method comparison. Thorax 1991; 46:454-456. Crossref, MedlineGoogle Scholar
  • 24 Colby TV, Carrington CB. Interstitial lung disease. In: Thurlbeck WM, Churg AM, eds. Pathology of the lung. New York, NY: Thieme, 1995; 589-737. Google Scholar
  • 25 Young RH, Mark GJ. Pulmonary vascular changes in scleroderma. Am J Med 1978; 64:998-1004. Crossref, MedlineGoogle Scholar
  • 26 Silver RM, Miller KS. Lung involvement in systemic sclerosis. Rheum Dis Clin North Am 1990; 16:199-216. Crossref, MedlineGoogle Scholar
  • 27 Wells AU, Cullinan P, Hansell DM, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1994; 149:1583-1590. Crossref, MedlineGoogle Scholar
  • 28 Douglas WW, Tazelaar HD, Hartman TE, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001; 164:1182-1185. Crossref, MedlineGoogle Scholar
  • 29 Katzenstein AL, Fiorelli RF. Non-specific interstitial pneumonia/fibrosis: histologic features and clinical significance. Am J Surg Pathol 1994; 18:136-147. Crossref, MedlineGoogle Scholar
  • 30 Nicholson AG, Colby TV, Wells AU. Histopathological approach to patterns of interstitial pneumonias in patients with connective tissue disorders. Sarcoidosis Vasc Diffuse Lung Dis 2002; 19:10-17. MedlineGoogle Scholar
  • 31 Schurawitzki H, Stiglbauer R, Graninger W, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 1990; 176:755-759. LinkGoogle Scholar
  • 32 Johkoh T, Ikezoe J, Kohno N, et al. High-resolution CT and pulmonary function tests in collagen vascular disease: a comparison with idiopathic pulmonary fibrosis. Eur J Radiol 1994; 18:113-121. Crossref, MedlineGoogle Scholar
  • 33 Wells AU, Hansell DM, Rubens MB, et al. Fibrosing alveolitis in systemic sclerosis: bronchoalveolar lavage findings in relation to computed tomographic appearance. Am J Respir Crit Care Med 1994; 150:462-468. Crossref, MedlineGoogle Scholar
  • 34 Chan TY, Hansell DM, Rubens MB, du Bois RM, Wells AU. Cryptogenic fibrosing alveolitis and the fibrosing alveolitis of systemic sclerosis: morphological differences on computed tomographic scans. Thorax 1997; 52:265-270. Crossref, MedlineGoogle Scholar
  • 35 Wells AU, Hansell DM, Rubens MB, Cailes JB, Black CM, du Bois RM. Functional impairment in lone cryptogenic fibrosing alveolitis and fibrosing alveolitis associated with systemic sclerosis: a comparison. Am J Respir Crit Care Med 1997; 155:1657-1664. Crossref, MedlineGoogle Scholar
  • 36 Nicholson AG, Colby TV, du Bois RM, Hansell DM, Wells AU. The prognostic significance of the histologic pattern of interstitial pneumonia in patients with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 2000; 162:2213-2217. Crossref, MedlineGoogle Scholar
  • 37 Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 157:199-203. Crossref, MedlineGoogle Scholar
  • 38 Daniil ZD, Gilchrist FC, Nicholson AG, et al. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstital pneumonia in patients with cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1999; 160:899-905. Crossref, MedlineGoogle Scholar
  • 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. Crossref, MedlineGoogle Scholar
  • 40 King TE, Jr, Schwarz MI, Brown K, et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med 2001; 164:1025-1032. Crossref, MedlineGoogle Scholar

Article History

Published in print: Aug 2004