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Am. J. Respir. Crit. Care Med., Volume 160, Number 3, September 1999, 899-905

A Histologic Pattern of Nonspecific Interstitial Pneumonia Is Associated with a Better Prognosis Than Usual Interstitial Pneumonia in Patients with Cryptogenic Fibrosing Alveolitis

ZOE D. DANIIL, FRANCES C. GILCHRIST, ANDREW G. NICHOLSON, DAVID M. HANSELL, JESSICA HARRIS, THOMAS V. COLBY, and ROLAND M. du BOIS

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

This study aimed to investigate whether there was a difference in outcome related to histologic pattern in cryptogenic fibrosing alveolitis (CFA) and to see whether there were correlations between clinical and radiologic findings and histology. One hundred thirteen lung biopsies from consecutive patients taken for the diagnosis of diffuse lung disease were reviewed and reclassified using the Katzenstein and Myers criteria for interstitial pneumonias. Patients lacking full investigational data at presentation and those with conditions predisposing to lung fibrosis were excluded, leaving 15 patients diagnosed with nonspecific interstitial pneumonia (NSIP) and 15 with usual interstitial pneumonia (UIP). Clinical and radiologic findings at presentation and serial lung function information and survival status in November 1998 were compared for the two groups. Survival was found to be significantly greater in the NSIP group compared with the UIP group (p < 0.001). This could not be explained by differences in treatment. Patients with UIP showed a progressive deterioration in lung function whereas those with NSIP remained stable. CT scans of patients with UIP showed more fibrosis than those of patients with NSIP (p < 0.011). A histologic diagnosis of NSIP is associated with a better prognosis than UIP. This subclassification of CFA is clinically useful.




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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
T. E. KING JR., J. A. TOOZE, M. I. SCHWARZ, K. R. BROWN, and R. M. CHERNIACK
Predicting Survival in Idiopathic Pulmonary Fibrosis . Scoring System and Survival Model
Am. J. Respir. Crit. Care Med., October 1, 2001; 164(7): 1171 - 1181.
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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.
[Abstract] [Full Text] [PDF]


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Am. J. Respir. Crit. Care Med.Home page
T. E. KING JR., M. I. SCHWARZ, K. BROWN, J. A. TOOZE, T. V. COLBY, J. A. WALDRON JR., A. FLINT, W. THURLBECK, and R. M. CHERNIACK
Idiopathic Pulmonary Fibrosis . Relationship between Histopathologic Features and Mortality
Am. J. Respir. Crit. Care Med., September 15, 2001; 164(6): 1025 - 1032.
[Abstract] [Full Text] [PDF]


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Eur Respir JHome page
R.M. du Bois and A.U. Wells
Cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis
Eur. Respir. J., July 1, 2001; 18(32_suppl): 43S - 55s.
[Abstract] [Full Text] [PDF]


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Eur Respir JHome page
U. Costabel and T.E. King
International consensus statement on idiopathic pulmonary fibrosis
Eur. Respir. J., February 1, 2001; 17(2): 163 - 167.
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Eur Respir JHome page
J.H. Ryu, T.V. Colby, T.E. Hartman, and R. Vassallo
Smoking-related interstitial lung diseases: a concise review
Eur. Respir. J., January 1, 2001; 17(1): 122 - 132.
[Abstract] [Full Text] [PDF]


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Am. J. Respir. Crit. Care Med.Home page
A. G. NICHOLSON, T. V. COLBY, R. M. DUBOIS, D. M. HANSELL, and A. U. WELLS
The Prognostic Significance of the Histologic Pattern of Interstitial Pneumonia in Patients Presenting with the Clinical Entity of Cryptogenic Fibrosing Alveolitis
Am. J. Respir. Crit. Care Med., December 1, 2000; 162(6): 2213 - 2217.
[Abstract] [Full Text]


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Am. J. Respir. Crit. Care Med.Home page
H. MIKI, T. MIO, S. NAGAI, Y. HOSHINO, T. NAGAO, M. KITAICHI, and T. IZUMI
Fibroblast Contractility . Usual Interstitial Pneumonia and Nonspecific Interstitial Pneumonia
Am. J. Respir. Crit. Care Med., December 1, 2000; 162(6): 2259 - 2264.
[Abstract] [Full Text]


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ThoraxHome page
M. Akira, G. Inoue, S. Yamamoto, and M. Sakatani
Non-specific interstitial pneumonia: findings on sequential CT scans of nine patients
Thorax, October 1, 2000; 55(10): 854 - 859.
[Abstract] [Full Text]


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NEJMHome page
R.M. du Bois
Interferon Gamma-1b for the Treatment of Idiopathic Pulmonary Fibrosis
N. Engl. J. Med., October 21, 1999; 341(17): 1302 - 1304.
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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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