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American Journal of Respiratory and Critical Care Medicine Vol 166. pp. 173-177, (2002)
© 2002 American Thoracic Society

Original Article

The Relationship between Individual Histologic Features and Disease Progression in Idiopathic Pulmonary Fibrosis

Andrew G. Nicholson, Laura G. Fulford, Thomas V. Colby, Roland M. du Bois, David M. Hansell and Athol U. Wells

Departments of Histopathology, Radiology, and Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom; and Department of Pathology, Mayo Clinic, Scottsdale, Arizona

Correspondence and requests for reprints should be addressed to Dr. A. G. Nicholson, Department of Histopathology, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. E-mail: a.nicholson{at}rbh.nthames.nhs.uk

We have retrospectively studied 53 patients with idiopathic pulmonary fibrosis and a histologic diagnosis of usual interstitial pneumonia and evaluated the prognostic significance of four individual histologic features (fibroblastic foci [FF], interstitial mononuclear cell infiltrate, established fibrosis, and intra-alveolar macrophages) using a semiquantitative scale of 0–6. An objective count of FF was also undertaken. Using weighted kappa coefficients, interobserver agreement between pathologists was moderate to good (0.56–0.76). Subjective and objective FF scores were strongly associated (RS = 0.88, < 0.00005). Mortality was independently linked to a high FF score, p = 0.006, and a low percent predicted carbon monoxide diffusing capacity (DLCO), p = 0.01. For pulmonary function, on univariate analysis, the strongest correlations were observed between increasing interstitial mononuclear cell infiltrate or FF scores and greater declines in forced vital capacity (FVC) or DLCO at 6 months. Multivariate models revealed that increasing FF scores were independently associated with greater declines in FVC and DLCO at both 6 and 12 months. Increasing interstitial mononuclear cell infiltrate scores were also independently linked to functional decline, but only at 6 months. These data suggest a reproducible method on biopsy for predicting rate of disease progression in patients with idiopathic pulmonary fibrosis.

Key Words: pulmonary fibrosis • prognosis • usual interstitial pneumonia




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