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) [1], 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.
CITATION STYLE
Keir, G. J., Silver, R. M., & Wells, A. U. (2012). Clinical assessment of lung disease. In Scleroderma: From Pathogenesis to Comprehensive Management (pp. 403–413). Springer US. https://doi.org/10.1007/978-1-4419-5774-0_33
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