Published ahead of print on March 27, 2008, doi:10.1164/rccm.200706-877OC Am. J. Respir. Crit. Care Med., Volume 177, Number 11, June 2008, 1248-1254 A more recent version of this article appeared on June 1, 2008
Submitted on June 16, 2007 Interstitial Lung Disease in Systemic Sclerosis: A Simple Staging SystemNicole SL Goh1,1 Royal Brompton Hospital and National Heart and Lung Institute, London, United Kingdom, 2 King's College Hospital, London, United Kingdom, 3 Hammersmith Hospital, London, United Kingdom, 4 Royal Free Hospital, London, United Kingdom * To whom correspondence should be addressed. E-mail: a.wells{at}rbht.nhs.uk.
Rationale: In interstitial lung disease complicating systemic sclerosis (SSc-ILD), the optimal prognostic use of baseline pulmonary function tests (PFT) and high resolution computed tomography (HRCT) is uncertain.
Objectives: To construct a readily applicable prognostic algorithm in SSc-ILD, integrating PFT and HRCT.
Methods: The prognostic value of baseline PFT and HRCT variables was quantified in patients with SSc-ILD (n=215) against survival and serial PFT data.
Results: Increasingly extensive disease on HRCT was a powerful predictor of mortality (p<0.0005), with an optimal extent threshold of 20%. In patients with HRCT extent 10-30% (termed indeterminate disease), an FVC threshold of 70% was an adequate prognostic substitute. Based upon these observations, SSc-ILD was staged as Limited disease (minimal disease on HRCT or, in indeterminate cases, FVC Key words: prognosis, HRCT, PFT, Limited, Extensive
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