Skip to main content
Log in

High resolution computed tomography for the evaluation of lung involvement in 101 patients with scleroderma

  • Originals
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Summary

The aim of the study was to investigate the pulmonary parenchymal abnormalities by high resolution computed tomography (HRCT) in patients with various forms of scleroderma. Three scans were performed sequentially in all cases: one at the level of aortic arch, one at the tracheal carina and one 1–2 cm above the diaphragm. Seventy cases with limited, 21 patients with diffuse cutaneous systemic sclerosis and 10 cases with circumscribed scleroderma were investigated. The 21 patients with diffuse scleroderma included three normal HRCT scan (14%), three with ground glass attenuation (14%), one with ground glass attenuation with fibrosis, three with fibrosis (14%), six with subpleural and five with diffuse honeycombing. The majority of cases with extensive honeycombing of the lungs had anti-Scl 70 autoantibody which was the characteristic antibody of the diffuse scleroderma subset. The 70 cases with limited cutaneous systemic sclerosis were characterized by normal HRCT (43%), ground glass opacity with or without fibrosis (18.6%), whereas fibrosis was detected in 22.9%, subpleural or diffuse honeycombing in 15.7% of the patients. Six of the ten cases with circumscribed scleroderma also showed a fibrosis. Cases with diffuse scleroderma are characterized by the presence of advanced fibrosis, whereas the extent of fibrosis is far less pronounced in limited scleroderma. Mild fibrosis may also be present in circumscribed scleroderma forms.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Czirják, L., Nagy, Z., Szegedi, G. Survival analysis of 118 patients with systemic sclerosis. J Int Med 1993, 234, 335–337.

    Google Scholar 

  2. Nakata, H., Kimoto, T., Nakayama, T., Kido, M., Miyazaki, N., Harada, S. Diffuse peripheral lung disease: evaluation by highresolution computed tomography. Radiology 1985, 157, 181–185.

    PubMed  Google Scholar 

  3. Bergin, C.J., Muller, N.L., CT in the diagnosis of interstitial lung disease. Am J Roentgenol 1985, 145, 505–510.

    Google Scholar 

  4. Schurawitzki, H., Stiglbauer, R., Graninger, W., Herold, C., Polzleitner, D., Burghuber, O.C., Tscholakoff, D. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 1990, 176, 755–759.

    PubMed  Google Scholar 

  5. Mathieson, J.R., Mayo, J.R., Staples, C.A., Muller, N.L. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology 1989, 171, 111116.

    Google Scholar 

  6. Wells, A.U., Hansell, D.M., Rubens, M.B., Cullinan, P., Black, C.M., du Bois, R.M. The predictive value of appearances on thinsection computed tomography in fibrosing alveolitis. Am Rev Respir Dis 1993, 148, 1076–1082.

    PubMed  Google Scholar 

  7. Warrick, J.H., Bhalla, M., Schabel, S.I., Silver, R.M., High resolution computed tomography in early scleroderma lung disease. J Rheumatol 1991, 18, 1520–1528.

    PubMed  Google Scholar 

  8. Garber, S.J., Wells, A.U., du Bois, R.M., Hansell D.M. Enlarged mediastinal lymph nodes in the fibrosing alveolitis of systemic sclerosis. Br J Radiol 1992, 65, 983–986.

    PubMed  Google Scholar 

  9. Harrison, N.K., Glanville, A.R., Strickland, B., Haslam, P.L., Corrin, B., Addis, B.J., Lawrence, R., Millar, A.B., Black, C.M., Turner Warwick, M. Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99m Tc-DTPA clearance. Respir Med 1989, 83, 403–14.

    PubMed  Google Scholar 

  10. Wells, A.U., Hansell, D.M., Corrin, B., Harrison, N.K., Goldstraw, P., Black, C.M., du Bois, R.M. High resolution computed tomography as a predictor of lung histology in systemic, sclerosis. Thorax 1992, 47, 738–742.

    PubMed  Google Scholar 

  11. Strickland, B., Strickland, N.H. The value of high definition, narrow section computed tomography in fibrosing alveolitis., Clin Radiol 1988, 39, 589–594.

    PubMed  Google Scholar 

  12. Czirják, L., Szolnoki, E., Nagy, Z., Szegedi, G. Changes in pulmonary function in systemic sclerosis. Postgrad Med J 1992, 68, 388.

    Google Scholar 

  13. Potente, G., Bellelli, A., Nardis, P. Specific diagnosis by CT and HRCT in six chronic lung diseases. Comput Med Imaging Graph 1992, 16, 277–282.

    PubMed  Google Scholar 

  14. D'Angelo, W.A., Fries, J.F., Masi, A.T., Shulman L.E. 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.

    PubMed  Google Scholar 

  15. Romagnoli, R., Bertolani, M., Guicciardi, L., Buonaura, P.C. Diagnostic imaging of non infiltrative interstitial lung disease. Rays 1992, 17, 242–261.

    PubMed  Google Scholar 

  16. Pignone, A., Matucci Cerinic, M., Lombardi, A., Fedi, R., Fargnoli, R., De Dominicis, R., Cagnoni, M. High resolution computed tomography in systemic sclerosis. Real diagnostic utilities in the assessment of pulmonary involvement and comparison with other modalities of lung investigation. Clin Rheumatol 1992, 11, 465–472.

    PubMed  Google Scholar 

  17. Dansin, E., Wallaert, B., Remy Jardin, M., Perez, T., Hatron, P.Y., Remy, J., Tonnel, A.B. Study of high-resolution thoracic computerized tomography and bronchoalveolar lavage in 36 patients presenting with systemic disease and a normal throacic radiography. Rev Mal Respir 1991, 8, 551–558.

    PubMed  Google Scholar 

  18. Chanez, P., Lacoste, J.Y., Guillot, B., Giron, J., Barncon, G., Enander, I., Godard, P., Michel, F.B., Bousquet, J. Mast cells' contribution to the fibrosing alveolitis of the scleroderma lung. Am Rev Respir Dis 1993, 147, 1497–1502.

    PubMed  Google Scholar 

  19. LeRoy, E.C., Black, C., Fleischmajer R., Jablonska, S., Krieg, T., Medsger, T.A.J., Rowell, N., Wollheim, F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988, 15, 202–204.

    PubMed  Google Scholar 

  20. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for Scleroderma Criteria, of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee Arthritis Rheum 1980, 23, 581–590.

  21. Czirják, L., Bokk, A., Csontos, G., Lörincz, G., Szegedi, G. Clinical findings in 61 patients with progressive systemic sclerosis. Acta Derm Venereol (Stockh) 1989, 69, 533–536.

    Google Scholar 

  22. Czirják, L., Nagy, Z., Szegedi, G. Systemic sclerosis in the elderly. Clin Rheumatol 1992, 11, 483–485.

    PubMed  Google Scholar 

  23. Kerr, I.H. Interstitial lung disease: the role of the radiologist. Clin Radiol 1984, 35, 1–7.

    PubMed  Google Scholar 

  24. Mayo, J.R., Webb, W.R., Gould, R., Stein, M.G., Bass, I., Gamsu, G., Goldberg, H.I. High-resolution CT of the lungs: an optimal approach. Radiology 1987, 163, 507–510 25.

    PubMed  Google Scholar 

  25. Breit, S.N., Cairns, D., Szentirmay, A., Callaghan, T., Murray, D., Wacher, T., Gibson, P.G., Marks, G., Bryant, D.H., Yeates, M. The presence of Sjögren's syndrome is a major determinant of the pattern of interstitial lung disease in scleroderma and other connective tissue diseases. J Rheumatol 1989, 16, 1043–1049.

    PubMed  Google Scholar 

  26. Muller, N.L., Staples, C.A., Miller, R.R., Vedal, S., Thurlbeck, W.M., Ostrow, D.N. Disease activity, in idiopathic pulmonary fibrosis: CT and pathologic correlation. Radiology 1987, 165, 731–734.

    PubMed  Google Scholar 

  27. Remy Jardin, M., Remy, J., Wallaert, B., Bataille, D., Hatron, P.Y., Pulmonary involvement in progressive systemic sclerosis: sequential evaluation. Radiology 1993, 188, 499–506.

    PubMed  Google Scholar 

  28. Czirják, L., Ladányi, E., Schlammadinger, J., Szegedi, G. Signs of systemic disease in localized scleroderma. Arch Dermatol Res 1991, 283, 418.

    PubMed  Google Scholar 

  29. Konig, G., Luderschmidt, C., Hammer, C., Adelmann Grill, B.C., Braun Falco, O., Fruhmann, G. Lung involvement in scleroderma. Chest 1984, 85, 318–324.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Dévényi, K., Czirják, L. High resolution computed tomography for the evaluation of lung involvement in 101 patients with scleroderma. Clin Rheumatol 14, 633–640 (1995). https://doi.org/10.1007/BF02207928

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02207928

Key words

Navigation