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Am. J. Respir. Crit. Care Med., Volume 162, Number 6, December 2000, 2213-2217

The Prognostic Significance of the Histologic Pattern of Interstitial Pneumonia in Patients Presenting with the Clinical Entity of Cryptogenic Fibrosing Alveolitis

ANDREW G. NICHOLSON, THOMAS V. COLBY, ROLAND M. DUBOIS, DAVID M. HANSELL, and ATHOL U. WELLS

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

Lone cryptogenic fibrosing alveolitis (CFA) is a progressive interstitial lung disease, with a median survival of 3 to 6 yr from the onset of dyspnea. CFA can be subdivided into prognostically significant histopathologic patterns, including nonspecific interstitial pneumonia (NSIP). We reviewed 78 patients with a clinicopathologic diagnosis of CFA, biopsied between 1978 and 1989, to evaluate the prevalence and prognostic significance of these histopathologic patterns, in particular NSIP. Biopsy appearances were reclassified by two pulmonary histopathologists as usual interstitial pneumonia (UIP) (47%), NSIP (36%), or desquamative interstitial pneumonia (DIP)/respiratory bronchiolitis-associated interstitial lung disease (RBILD) (17%). The kappa coefficient of agreement between pathologists was 0.49. In 67 cases, follow-up was complete to death or 10 yr after biopsy, with 50 deaths during a median follow-up of 42 mo (UIP, 89%; NSIP, 61%, DIP/RBILD, 0%). Survival was highest in DIP/RBILD and higher in NSIP than UIP, p < 0.0005. When analysis was confined to patients with UIP or NSIP, the mortality of UIP remained higher, p < 0.01, but the 5-yr survival in patients with fibrotic NSIP was only 45%, indicating that this histologic appearance is often associated with a poor outcome. A response to treatment was more frequent in DIP/RBILD than in NSIP (p < 0.01) or UIP (p < 0.0005). This study confirms the prognostic value of subclassifying patients with CFA according to histopathologic pattern. However, in patients with clinically typical CFA, a histologic diagnosis of fibrotic NSIP needs to be interpreted with caution and does not necessarily denote a good outcome.




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K. R. Flaherty, T. V. Colby, W. D. Travis, G. B. Toews, J. Mumford, S. Murray, V. J. Thannickal, E. A. Kazerooni, B. H. Gross, J. P. Lynch III, et al.
Fibroblastic Foci in Usual Interstitial Pneumonia: Idiopathic versus Collagen Vascular Disease
Am. J. Respir. Crit. Care Med., May 15, 2003; 167(10): 1410 - 1415.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
A. U. Wells, S. R. Desai, M. B. Rubens, N. S. L. Goh, D. Cramer, A. G. Nicholson, T. V. Colby, R. M. du Bois, and D. M. Hansell
Idiopathic Pulmonary Fibrosis: A Composite Physiologic Index Derived from Disease Extent Observed by Computed Tomography
Am. J. Respir. Crit. Care Med., April 1, 2003; 167(7): 962 - 969.
[Abstract] [Full Text] [PDF]

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 Arch Intern MedHome page
H. R. Collard and T. E. King Jr
Demystifying Idiopathic Interstitial Pneumonia
Arch Intern Med, January 13, 2003; 163(1): 17 - 29.
[Abstract] [Full Text] [PDF]

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 RadiologyHome page
T. Johkoh, N. L. Muller, T. V. Colby, K. Ichikado, H. Taniguchi, Y. Kondoh, K. Fujimoto, M. Kinoshita, H. Arakawa, H. Yamada, et al.
Nonspecific Interstitial Pneumonia: Correlation between Thin-Section CT Findings and Pathologic Subgroups in 55 Patients
Radiology, October 1, 2002; 225(1): 199 - 204.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
A. G. Nicholson, L. G. Fulford, T. V. Colby, R. M. du Bois, D. M. Hansell, and A. U. Wells
The Relationship between Individual Histologic Features and Disease Progression in Idiopathic Pulmonary Fibrosis
Am. J. Respir. Crit. Care Med., July 15, 2002; 166(2): 173 - 177.
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 Am. J. Respir. Crit. Care Med.Home page
R. G. Crystal, P. B. Bitterman, B. Mossman, M. I. Schwarz, D. Sheppard, L. Almasy, H. A. Chapman, S. L. Friedman, T. E. King Jr., L. A. Leinwand, et al.
Future Research Directions in Idiopathic Pulmonary Fibrosis: Summary of a National Heart, Lung, and Blood Institute Working Group
Am. J. Respir. Crit. Care Med., July 15, 2002; 166(2): 236 - 246.
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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
H. Matsuoka, T. Arai, M. Mori, S. Goya, H. Kida, H. Morishita, H. Fujiwara, I. Tachibana, T. Osaki, and S. Hayashi
A p38 MAPK inhibitor, FR-167653, ameliorates murine bleomycin-induced pulmonary fibrosis
Am J Physiol Lung Cell Mol Physiol, July 1, 2002; 283(1): L103 - L112.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
T. E. King Jr.
Nonspecific Interstitial Pneumonia and Systemic Sclerosis
Am. J. Respir. Crit. Care Med., June 15, 2002; 165(12): 1578 - 1579.
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 Am. J. Respir. Crit. Care Med.Home page
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.
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 Eur Respir JHome page
R.L. Riha, E.E. Duhig, B.E. Clarke, R.H. Steele, R.E. Slaughter, and P.V. Zimmerman
Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia
Eur. Respir. J., June 1, 2002; 19(6): 1114 - 1118.
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 Eur Respir JHome page
K.R. Flaherty, G.B. Toews, W.D. Travis, T.V. Colby, E.A. Kazerooni, B.H. Gross, A. Jain, R.L. Strawderman III, R. Paine, A. Flint, et al.
Clinical significance of histological classification of idiopathic interstitial pneumonia
Eur. Respir. J., February 1, 2002; 19(2): 275 - 283.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
M. J. TOBIN
Tuberculosis, Lung Infections, and Interstitial Lung Disease in AJRCCM 2000
Am. J. Respir. Crit. Care Med., November 15, 2001; 164(10): 1774 - 1788.
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 Am. J. Respir. Crit. Care Med.Home page
A. G. Nicholson, A. U. Wells, D. o. H. R. Medicine, R. B. Hospital, London, and UK
Nonspecific Interstitial Pneumonia-Nobody Said It's Perfect
Am. J. Respir. Crit. Care Med., November 1, 2001; 164(9): 1553 - 1554.
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 Am. J. Respir. Crit. Care Med.Home page
K. R. FLAHERTY, W. D. TRAVIS, T. V. COLBY, G. B. TOEWS, E. A. KAZEROONI, B. H. GROSS, A. JAIN, R. L. STRAWDERMAN III, A. FLINT, J. P. LYNCH III, et al.
Histopathologic Variability in Usual and Nonspecific Interstitial Pneumonias
Am. J. Respir. Crit. Care Med., November 1, 2001; 164(9): 1722 - 1727.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
W. W. DOUGLAS, H. D. TAZELAAR, T. E. HARTMAN, R. P. HARTMAN, P. A. DECKER, D. R. SCHROEDER, and J. H. RYU
Polymyositis-Dermatomyositis-associated Interstitial Lung Disease
Am. J. Respir. Crit. Care Med., October 1, 2001; 164(7): 1182 - 1185.
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 RadiologyHome page
S. L. S. MacDonald, M. B. Rubens, D. M. Hansell, S. J. Copley, S. R. Desai, R. M. du Bois, A. G. Nicholson, T. V. Colby, and A. U. Wells
Nonspecific Interstitial Pneumonia and Usual Interstitial Pneumonia: Comparative Appearances at and Diagnostic Accuracy of Thin-Section CT
Radiology, December 1, 2001; 221(3): 600 - 605.
[Abstract] [Full Text] [PDF]

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 RadiologyHome page
D. A. Lynch
Nonspecific Interstitial Pneumonia: Evolving Concepts
Radiology, December 1, 2001; 221(3): 583 - 584.
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