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Rheumatology 2008 47(Supplement 5):v65-v67; doi:10.1093/rheumatology/ken313
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

This article appears in the following Rheumatology issue: Update in systemic sclerosis [View the issue table of contents]

Pulmonary function tests

J. Behr1 and D. E. Furst2

1Department of Internal Medicine, Grosshadern clinic, Ludwig Maximilians University of Munich, Munich, Germany and 2Geffen School of Medicine, University of California in Los Angeles, Los Angeles, CA, USA.

Correspondence to: J. Behr, Klinikum Grosshadern, Medizinische Klinik I, Marchioninistr. 15, 81377 Munich, Germany. E-mail: jbehr{at}med1.med.uni-muenchen.de


  Abstract

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is the leading cause of morbidity and mortality in SSc patients. In autopsy studies, up to 70% of the SSc patients show histological evidence of lung involvement. In surgical lung biopsies from SSc-ILD patients, a pattern of non-specific interstitial pneumonia is present in a majority, while usual interstitial pneumonia is found in a more variable proportion of patients. Up to 90% of the SSc patients have lung function abnormalities, especially with respect to gas transfer. Pulmonary function testing, therefore, is a major tool of investigation of lung involvement in SSc.

KEY WORDS: Systemic sclerosis, Pulmonary function tests, Spirometry, Body plethysmography, Diffusing capacity, CO transfer factor, Compliance, Exercise tests, Pulmonary hypertension

Submitted 1 May 2008; Accepted 3 July 2008


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