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Published ahead of print on June 5, 2003, doi:10.1164/rccm.200210-1245OC

Am. J. Respir. Crit. Care Med., Volume 168, Number 5, September 2003, 531-537

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Submitted on October 30, 2002
Accepted on May 29, 2003

Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends

Panagiota I Latsi1, Roland M du Bois1, Andrew G Nicholson1, Thomas V Colby2, Danai Bisirtzoglou1, Ageliki Nikolakopoulou1, Srihari Veeraraghavan1, David M Hansell1, and Athol U Wells1*

1 Interstitial Lung Disease Unit, Radiology and Pathology, Royal Brompton Hospital, London, United Kingdom, 2 Pathology, Mayo Clinic, Scottsdale, Arizona, USA

* To whom correspondence should be addressed. E-mail: a.wells{at}rbh.nthames.nhs.uk.

Survival is linked to the histolopathologic distinction between usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP), the most commonly encountered fibrotic idiopathic interstitial pneumonias (IIP). We retrospectively compared the prognostic significance of histopathologic diagnoses, baseline pulmonary function indices, and serial trends in pulmonary function indices (DLco, FVC, FEV1, the recently defined composite physiologic index [CPI]) at six and 12 months in 104 patients (UIP, n=63; fibrotic NSIP, n=41). Survival was lower in UIP than in fibrotic NSIP, p=0.001, but not in patients with severe functional impairment; mortality during the first two years was linked solely to the severity of functional impairment at presentation; the CPI was the strongest determinant of outcome (p<0.001). At six months, serial DLco levels (p=0.003) and histopathologic diagnosis (p=0.002) were prognostically equivalent. At 12 months, serial pulmonary function trends were the only major prognostic determinant (p<0.0005 for all variables), with no independent significance associated with the distinction between UIP and fibrotic NSIP. We conclude that at 12 months, serial pulmonary function trends have considerable prognostic value in UIP and NSIP. Their histologic distinction provides no additional prognostic information when pulmonary function trends are clear-cut, or when functional impairment is severe.
Key words: Pulmonary function tests, usual interstitial pneumonia, non-specific interstitial pneumonia, prognosis




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