Skip to main content

Lung Impairment in Scleroderma

  • Conference paper
  • First Online:
Book cover Respiratory Regulation - Clinical Advances

Abstract

Scleroderma typically manifests as fibrosis of the skin, but may also involve other organs, particularly the lungs. Interstitial lung disease and functional abnormalities are observed in the majority of patients. The aim of this study was to evaluate radiological changes in the lungs and their correlation with functional disorders in scleroderma patients. The study was conducted in 37 scleroderma patients (F/M-31/6). High resolution computed tomography (HRCT), Warrick score system and spirometry, body plethysmography, and lung diffusion examinations (DLco) were performed. The HRCT showed septal and subpleural lines in 70%, ground-glass opacities in 51%, and honeycomb lungs in 30% of the cases. The DLco values were decreased in 92% of the patients. Total lung capacity (TLC) showed a restrictive pattern in 24% of the patients, and only in 11% of them obstruction predominated. The Warrick score correlated inversely with both DLco (r=0.36; p>0.05). Interstitial lung disease often coexists with scleroderma and is accompanied by functional lung abnormalities.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Avouac, J., Kowal-Bielecka, O., Landewé, R., Chwiesko, S., Miniati, I., Czirjak, L., Clements, P., Denton, C., Farge, D., Fligelstone, K., Földvari, I., Furst, D. E., Müller-Ladner, U., Seibold, J., Silver, R. M., Takehara, K., Garay Toth, B., Tyndall, A., Valentini, G., van den Hoogen, F., Wigley, F., Zulian, F., Matucci-Cerinic, M., & EUSTAR Coauthors. (2009). European League Against Rheumatism (EULAR) Scleroderma Trial and Research group (EUSTAR) recommendations for the treatment of systemic sclerosis: Methods of elaboration and results of systematic literature research. Annals of the Rheumatic Diseases, 68, 629–634.

    Article  PubMed  CAS  Google Scholar 

  • Behr, J., & Furst, D. E. (2008). Pulmonary function tests. Rheumatology, 47, 65–67.

    Article  Google Scholar 

  • Bellia, M., Cannizarro, F., Scichilone, N., Riili, M., Triolo, G., Midiri, M., & Lagalla, R. (2009). HRCT and scleroderma: Semiquantative evaluation of lung damage and functional abnormalities. La Radiologia Medica, 114, 190–203.

    Article  PubMed  CAS  Google Scholar 

  • Bussone, G., & Mouthon, L. (2011). Interstitial lung disease in systemic sclerosis. Autoimmunity Reviews, 10, 248–255.

    Article  PubMed  Google Scholar 

  • Cottin, V. (2007). Interstitial lung disease in connective diseases. La Revue du Praticien, 57, 2235–2242.

    PubMed  Google Scholar 

  • Dougados, M., Betteridge, N., Burmester, G. R., Euller-Ziegler, L., Guillemin, F., Hirvonen, J., Lloyd, J., Ozen, S., Da Silva, J. A. P., Emery, P., Kalden, J. R., Kvien, T., Matucci-Cerinic, M., & Smolen, J. (2004). EULAR standarised operating procedures for the elaboration, evaluation, dissemination, and implementation of recommendations endorsed by EULAR standing committees. Annals of the Rheumatic Diseases, 63, 1172–1176.

    Article  PubMed  CAS  Google Scholar 

  • Goldin, J. G., Lynch, D. A., Strollo, D. C., Suh, R. D., Schraufnagel, D. E., Clements, P. J., Elashoff, R. M., Furst, D. E., Vasunilashorn, S., McNitt-Gray, M., Brown, M. S., Toth, M. D., & Taskin, D. P. (2008). High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest, 134, 358–367.

    Article  PubMed  Google Scholar 

  • Hansen, J. E., Sun, X. G., & Wasserman, K. (2007). Spirometric criteria for airway obstruction. Use percentage of FEV1/FVC ratio below the fifth percentile, not 70%. Chest, 131, 349–355.

    Article  PubMed  Google Scholar 

  • Ioannidis, J. P. A., Vlachoyannopoulos, P. G., Haldich, A. B., Medsger, T. A., Jr., Lucas, M., Michet, C. J., Kuwana, M., Yasuoka, H., van den Hoogen, F., Te Boome, L., van Laar, J. M., Verbeet, N. L., Matucci-Cerinic, M., Georgountzos, A., & Moutsopoulos, H. M. (2005). Mortality in systemic sclerosis: An international meta-analysis of individual patient data. American Journal of Medicine, 118, 2–10.

    Article  PubMed  Google Scholar 

  • Kaloudi, O., Miniati, I., Alari, S., & Matucci-Cerinic, M. (2007). Interstitial lung disease in systemic sclerosis. Internal and Emergency Medicine, 4, 250–255.

    Article  Google Scholar 

  • Kowal-Bielecka, O., Landewé, R., Avouac, J., & EUSTAR Coauthors. (2009). EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR scleroderma trials and research group (EUSRAR). Annals of the Rheumatic Diseases, 68, 620–628.

    Article  PubMed  CAS  Google Scholar 

  • Launay, D., Remy-Jardin, M., Michon-Pasturel, U., Mastora, I., Hachulla, E., Lambert, M., Delannoy, V., Queyrel, V., Duhamel, A., Matran, R., De Grotte, P., & Hatron, P. Y. (2006). High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. Journal of Rheumatology, 33, 1789–1801.

    PubMed  Google Scholar 

  • MacIntyre, N., Crapo, R. O., Viegi, G., Johnson, D. C., van der Grinten, C. P. M., Brusasco, V., Burgos, F., Casaburi, R., Coates, A., Enright, P., Gustafsson, P., Hankinson, J., Jensen, R., McKay, R., Miller, M. R., Navajas, D., Pedersen, O. F., Pellegrino, R., & Wagner, J. (2005). Standardisation of the single-breath determination of carbon monoxide uptake in the lung. European Respiratory Journal, 26, 720–735.

    Article  PubMed  CAS  Google Scholar 

  • Miller, M. R., Hankinson, J., Brusasco, V., Burgos, F., Casaburi, R., Coates, A., Crapo, R., Enright, P., Ven der Grinten, C. P. M., Gustafsson, P., Jensen, R., Johnson, D. C., McIntyre, N., McKay, R., Navajas, D., Pedersen, O. F., Pellegrino, R., Viegi, G., & Wagner, J. (2005). Standardisation of spirometry. European Respiratory Journal, 26, 319–338.

    Article  PubMed  CAS  Google Scholar 

  • Pellegrino, R., Viegi, G., Brusasco, V., Crapo, R., Casaburi, R., Coates, A., Ven der Grinten, C. P. M., Gustafsson, P., Hankinson, J., Jensen, R., Johnson, D. C., McIntyre, N., McKay, R., Miller, M. R., Navajas, D., Pedersen, O. F., & Wagner, J. (2005). Interpretative strategies for lung function tests. European Respiratory Journal, 26, 948–968.

    Article  PubMed  CAS  Google Scholar 

  • Wagner, J., Clausen, J. L., Coates, A., Pedersen, O. F., Brusasco, V., Burgos, F., Casaburi, R., Crapo, R., Enright, P., Ven der Grinten, C. P. M., Gustafsson, P., Hankinson, J., Jensen, R., Johnson, D. C., McIntyre, N., McKay, R., Miller, M. R., Navajas, D., Pellegrino, R., & Viegi, G. (2005). Standardisation of the measurement of lung volumes. European Respiratory Journal, 26, 511–522.

    Article  Google Scholar 

  • Warrick, J. H., Bhalla, M., Schabel, S. J., & Silver, R. M. (1991). High resolution computed tomography in early scleroderma lung disease. Journal of Rheumatology, 18, 1520–1528.

    PubMed  CAS  Google Scholar 

  • Wells, A. U. (2008). High-resolution computed tomography and scleroderma lung disease. Rheumatology, 47, 59–61.

    Article  Google Scholar 

Download references

Conflicts of Interest: The authors declare no conflicts of interest in relation to this article.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to T. Piorunek .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media Dordrecht

About this paper

Cite this paper

Piorunek, T. et al. (2013). Lung Impairment in Scleroderma. In: Pokorski, M. (eds) Respiratory Regulation - Clinical Advances. Advances in Experimental Medicine and Biology, vol 755. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-4546-9_20

Download citation

Publish with us

Policies and ethics