Am. J. Respir. Crit. Care Med., Volume 157, Number 5, May 1998, 1474-1482

Bronchoalveolar Lavage Cellularity
Lone Cryptogenic Fibrosing Alveolitis Compared with the Fibrosing Alveolitis of Systemic Sclerosis

ATHOL U. WELLS, DAVID M. HANSELL, PATRICIA L. HASLAM, MICHAEL B. RUBENS, JEREMY CAILES, CAROL M. BLACK, and ROLAND M. du BOIS

Departments of Interstitial Lung Disease and Radiology, Royal Brompton Hospital, Biology Unit, National Heart and Lung Institute, Department of Rheumatology, Royal Free Hospital, London, United Kingdom; and Department of Respiratory Medicine, Green Lane Hospital, Auckland, New Zealand

Lone cryptogenic fibrosing alveolitis (CFA) is histologically identical to fibrosing alveolitis associated with systemic sclerosis (FASSc), but it has a much worse prognosis after matching for disease severity at presentation. The aims of this study were to gain insights into possible pathogenetic mechanisms contributing to this prognostic difference, by comparing bronchoalveolar lavage (BAL) cellularity in the two diseases, and to evaluate the relationships between BAL findings and the regional and global extent of disease, quantified by thin-section computed tomography (CT) and lung function indices. Patients with CFA were distinguished by more extensive fibrosing alveolitis on CT (p < 0.02) and by higher counts of neutrophils (total per ml, p < 0.02; percentage p < 0.03) and eosinophils (total per ml, p < 0.002; percentages, p < 0.02) in BAL fluid. After adjustment for functional and morphologic measures of disease extent, eosinophil percentages and total counts were increased in CFA (p < 0.05 in all 12 multivariate models), but they were not independently related to regional or global disease severity. Neutrophil percentages and total counts were virtually identical in CFA and FASSc in disease of comparable severity, and they increased with increasingly extensive lobar disease and global disease, as judged by CT, p < 0.0005 in all analyses. Neutrophil levels were more closely linked to the extent of disease on CT than to the severity of functional impairment, on univariate and multivariate analysis. The higher BAL eosinophil levels seen in CFA, compared with those seen in FASSc, after adjustment for disease extent, indicate that an eosinophilic influx may be linked to the pathogenesis of fibrosing alveolitis. By contrast, BAL neutrophil levels increase with increasingly extensive disease on CT, but they do not differ independently between CFA and FASSc, suggesting that neutrophil degradation products are unlikely to account for the excess mortality in CFA, compared with that in FASSc.

This article has been cited by other articles:

				E. A. Renzoni, D. A. Walsh, M. Salmon, A. U. Wells, P. Sestini, A. G. Nicholson, S. Veeraraghavan, A. E. Bishop, H. M. Romanska, P. Pantelidis, et al. Interstitial Vascularity in Fibrosing Alveolitis Am. J. Respir. Crit. Care Med., February 1, 2003; 167(3): 438 - 443. [Abstract] [Full Text] [PDF]

				H Mukae, H Iiboshi, M Nakazato, T Hiratsuka, M Tokojima, K Abe, J Ashitani, J Kadota, S Matsukura, and S Kohno Raised plasma concentrations of {alpha}-defensins in patients with idiopathic pulmonary fibrosis Thorax, July 1, 2002; 57(7): 623 - 628. [Abstract] [Full Text] [PDF]

				D. Bouros, A. U. Wells, A. G. Nicholson, T. V. Colby, V. Polychronopoulos, P. Pantelidis, P. L. Haslam, D. A. Vassilakis, C. M. Black, and R. M. du Bois Histopathologic Subsets of Fibrosing Alveolitis in Patients with Systemic Sclerosis and Their Relationship to Outcome Am. J. Respir. Crit. Care Med., June 15, 2002; 165(12): 1581 - 1586. [Abstract] [Full Text] [PDF]

				J Fujita, T Yoshinouchi, Y Ohtsuki, M Tokuda, Y Yang, I Yamadori, S Bandoh, T Ishida, J Takahara, and R Ueda Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis Ann Rheum Dis, March 1, 2001; 60(3): 281 - 283. [Abstract] [Full Text] [PDF]

				M. SUGA, K. IYONAGA, T. OKAMOTO, Y. GUSHIMA, H. MIYAKAWA, T. AKAIKE, and M. ANDO Characteristic Elevation of Matrix Metalloproteinase Activity in Idiopathic Interstitial Pneumonias Am. J. Respir. Crit. Care Med., November 1, 2000; 162(5): 1949 - 1956. [Abstract] [Full Text]

				J.-i. Ashitani, H. Mukae, H. Taniguchi, T. Ihi, J.-i. Kadota, S. Kohno, and S. Matsukura Granulocyte-colony stimulating factor levels in bronchoalveolar lavage fluid from patients with idiopathic pulmonary fibrosis Thorax, November 1, 1999; 54(11): 1015 - 1020. [Abstract] [Full Text]

				A. Wells Clinical usefulness of high resolution computed tomography in cryptogenic fibrosing alveolitis Thorax, December 1, 1998; 53(12): 1080 - 1087. [Full Text]