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Clinical Assessment of Lung Disease

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Scleroderma

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Abstract

For a variety of reasons, the epidemiology of systemic sclerosis-associated interstitial lung disease (SSc-ILD) has been difficult to accurately define. SSc is not a common disease (with an estimated prevalence of 50–300 per one million), and it may follow a clinically heterogeneous course, often with widely varying pulmonary manifestations. SSc-ILD may range from limited, non-progressive lung involvement to major pulmonary inflammation and fibrosis progressing to respiratory failure and death. The accuracy of epidemiological data has been further hampered by methodological differences in case ascertainment and differences in the definition of pulmonary disease. Recently, many of these shortcomings have been addressed.

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Author information

Authors and Affiliations

  1. Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK

    Gregory J. Keir MBBS, FRACP

  2. Medical University of South Carolina, Charleston, SC, USA

    Richard M. Silver MD

  3. Interstitial Lung Disease Unit, National Heart & Lung Institute, London, UK

    Athol U. Wells MD, FRCP

Authors
  1. Gregory J. Keir MBBS, FRACP
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  2. Richard M. Silver MD
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  3. Athol U. Wells MD, FRCP
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Corresponding author

Correspondence to Gregory J. Keir MBBS, FRACP .

Editor information

Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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Keir, G.J., Silver, R.M., Wells, A.U. (2012). Clinical Assessment of Lung Disease. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_33

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  • DOI: https://doi.org/10.1007/978-1-4419-5774-0_33

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