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Am. J. Respir. Crit. Care Med., Volume 164, Number 7, October 2001, 1171-1181

Predicting Survival in Idiopathic Pulmonary Fibrosis
Scoring System and Survival Model

TALMADGE E. KING JR., JANET A. TOOZE, MARVIN I. SCHWARZ, KEVIN R. BROWN, and REUBEN M. CHERNIACK

Departments of Medicine and Divisions of Pulmonary and Critical Care Medicine, San Francisco General Hospital, and the University of California, San Francisco, San Francisco, California; National Jewish Medical and Research Center, and the University of Colorado Health Sciences Center, Denver, Colorado

Our purpose was to identify clinical, radiological and physiological (CRP) determinants of survival and to develop a CRP scoring system that predicts survival in newly diagnosed cases of idiopathic pulmonary fibrosis (IPF). The study population consisted of 238 patients with biopsy confirmed usual interstitial pneumonia. For each patient, clinical manifestations, chest radiographs, and pulmonary physiology were prospectively assessed. We used Cox proportional-hazards models to assess the effect of these parameters on survival. The effects of age and smoking were included in the analysis. Survival was related to age, smoking status (longer in current smokers), clubbing, the extent of interstitial opacities and presence of pulmonary hypertension on the chest radiograph, reduced lung volume, and abnormal gas exchange during maximal exercise. A mathematical CRP score for predicting survival was derived from these parameters. We showed that this CRP score correlated with the extent and severity of the important histopathologic features of IPF, i.e., fibrosis, cellularity, the granulation/connective tissue deposition, and the total pathologic derangement. Using these models, clinicians are in a better position to provide prognostic information to patients with IPF and to improve the selection of the most appropriate patients for lung transplantation or other standard or novel therapeutic interventions.




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A. Azuma, T. Nukiwa, E. Tsuboi, M. Suga, S. Abe, K. Nakata, Y. Taguchi, S. Nagai, H. Itoh, M. Ohi, et al.
Double-blind, Placebo-controlled Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis
Am. J. Respir. Crit. Care Med., May 1, 2005; 171(9): 1040 - 1047.
[Abstract] [Full Text] [PDF]

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 ThoraxHome page
J J Egan, F J Martinez, A U Wells, and T Williams
Lung function estimates in idiopathic pulmonary fibrosis: the potential for a simple classification
Thorax, April 1, 2005; 60(4): 270 - 273.
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 Am. J. Respir. Crit. Care Med.Home page
I. Ito, S. Nagai, M. Kitaichi, A. G. Nicholson, T. Johkoh, S. Noma, D. S. Kim, T. Handa, T. Izumi, and M. Mishima
Pulmonary Manifestations of Primary Sjogren's Syndrome: A Clinical, Radiologic, and Pathologic Study
Am. J. Respir. Crit. Care Med., March 15, 2005; 171(6): 632 - 638.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
Y. Jegal, D. S. Kim, T. S. Shim, C.-M. Lim, S. Do Lee, Y. Koh, W. S. Kim, W. D. Kim, J. S. Lee, W. D. Travis, et al.
Physiology Is a Stronger Predictor of Survival than Pathology in Fibrotic Interstitial Pneumonia
Am. J. Respir. Crit. Care Med., March 15, 2005; 171(6): 639 - 644.
[Abstract] [Full Text] [PDF]

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 ChestHome page
S. D. Nathan
Lung Transplantation: Disease-Specific Considerations for Referral
Chest, March 1, 2005; 127(3): 1006 - 1016.
[Abstract] [Full Text] [PDF]

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 Am. J. Pathol.Home page
J. M. Pilewski, L. Liu, A. C. Henry, A. V. Knauer, and C. A. Feghali-Bostwick
Insulin-Like Growth Factor Binding Proteins 3 and 5 Are Overexpressed in Idiopathic Pulmonary Fibrosis and Contribute to Extracellular Matrix Deposition
Am. J. Pathol., February 1, 2005; 166(2): 399 - 407.
[Abstract] [Full Text] [PDF]

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 Eur Respir JHome page
T. S. Hallstrand, L. J. Boitano, W. C. Johnson, C. A. Spada, J. G. Hayes, and G. Raghu
The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis
Eur. Respir. J., January 1, 2005; 25(1): 96 - 103.
[Abstract] [Full Text] [PDF]

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 ChestHome page
J. J. Swigris, W. G. Kuschner, J. L. Kelsey, and M. K. Gould
Idiopathic Pulmonary Fibrosis: Challenges and Opportunities for the Clinician and Investigator
Chest, January 1, 2005; 127(1): 275 - 283.
[Abstract] [Full Text] [PDF]

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 Mayo Clin Proc.Home page
H. F. Nadrous, J. L. Myers, P. A. Decker, and J. H. Ryu
Idiopathic Pulmonary Fibrosis in Patients Younger Than 50 Years
Mayo Clin. Proc., January 1, 2005; 80(1): 37 - 40.
[Abstract] [PDF]

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 ChestHome page
M. Bonay, C. Bancal, D. de Zuttere, F. Arnoult, G. Saumon, and F. Camus
Normal Pulmonary Capillary Blood Volume in Patients With Chronic Infiltrative Lung Disease and High Pulmonary Artery Pressure
Chest, November 1, 2004; 126(5): 1460 - 1466.
[Abstract] [Full Text] [PDF]

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 ImagingHome page
N J Screaton and T Koh
Emphysema and smoking-related lung diseases
Imaging, October 1, 2004; 16(1): 50 - 60.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
N. S. Hill
Brain Natriuretic Peptide: Is It Helpful in Detecting Pulmonary Hypertension in Fibrotic Lung Disease?
Am. J. Respir. Crit. Care Med., August 15, 2004; 170(4): 352 - 353.
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 Am. J. Respir. Crit. Care Med.Home page
H. H. Leuchte, C. Neurohr, R. Baumgartner, M. Holzapfel, W. Giehrl, M. Vogeser, and J. Behr
Brain Natriuretic Peptide and Exercise Capacity in Lung Fibrosis and Pulmonary Hypertension
Am. J. Respir. Crit. Care Med., August 15, 2004; 170(4): 360 - 365.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
G. P. Cosgrove, K. K. Brown, W. P. Schiemann, A. E. Serls, J. E. Parr, M. W. Geraci, M. I. Schwarz, C. D. Cool, and G. S. Worthen
Pigment Epithelium-derived Factor in Idiopathic Pulmonary Fibrosis: A Role in Aberrant Angiogenesis
Am. J. Respir. Crit. Care Med., August 1, 2004; 170(3): 242 - 251.
[Abstract] [Full Text] [PDF]

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 NEJMHome page
G. Raghu, K. K. Brown, W. Z. Bradford, K. Starko, P. W. Noble, D. A. Schwartz, T. E. King Jr., and the Idiopathic Pulmonary Fibrosis Study Group
A Placebo-Controlled Trial of Interferon Gamma-1b in Patients with Idiopathic Pulmonary Fibrosis
N. Engl. J. Med., January 8, 2004; 350(2): 125 - 133.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
V. N. Lama, K. R. Flaherty, G. B. Toews, T. V. Colby, W. D. Travis, Q. Long, S. Murray, E. A. Kazerooni, B. H. Gross, J. P. Lynch III, et al.
Prognostic Value of Desaturation during a 6-Minute Walk Test in Idiopathic Interstitial Pneumonia
Am. J. Respir. Crit. Care Med., November 1, 2003; 168(9): 1084 - 1090.
[Abstract] [Full Text] [PDF]

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 ChestHome page
M. Selman
The Spectrum of Smoking-Related Interstitial Lung Disorders: The Never-Ending Story of Smoke and Disease
Chest, October 1, 2003; 124(4): 1185 - 1187.
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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
J. Cisneros-Lira, M. Gaxiola, C. Ramos, M. Selman, and A. Pardo
Cigarette smoke exposure potentiates bleomycin-induced lung fibrosis in guinea pigs
Am J Physiol Lung Cell Mol Physiol, October 1, 2003; 285(4): L949 - L956.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Cell Mol. Bio.Home page
N. Kaminski, J. A. Belperio, P. B. Bitterman, L. Chen, S. W. Chensue, A. M.K. Choi, S. Dacic, J. H. Dauber, R. M. du Bois, J. J. Enghild, et al.
Idiopathic Pulmonary Fibrosis
Am. J. Respir. Cell Mol. Biol., September 1, 2003; 29(3): S1 - 105.
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 Am. J. Respir. Crit. Care Med.Home page
H. R. Collard, T. E. King Jr., B. B. Bartelson, J. S. Vourlekis, M. I. Schwarz, and K. K. Brown
Changes in Clinical and Physiologic Variables Predict Survival in Idiopathic Pulmonary Fibrosis
Am. J. Respir. Crit. Care Med., September 1, 2003; 168(5): 538 - 542.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
K. R. Flaherty, J. A. Mumford, S. Murray, E. A. Kazerooni, B. H. Gross, T. V. Colby, W. D. Travis, A. Flint, G. B. Toews, J. P. Lynch III, et al.
Prognostic Implications of Physiologic and Radiographic Changes in Idiopathic Interstitial Pneumonia
Am. J. Respir. Crit. Care Med., September 1, 2003; 168(5): 543 - 548.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
P. I. Latsi, R. M. du Bois, A. G. Nicholson, T. V. Colby, D. Bisirtzoglou, A. Nikolakopoulou, S. Veeraraghavan, D. M. Hansell, and A. U. Wells
Fibrotic Idiopathic Interstitial Pneumonia: The Prognostic Value of Longitudinal Functional Trends
Am. J. Respir. Crit. Care Med., September 1, 2003; 168(5): 531 - 537.
[Abstract] [Full Text] [PDF]

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 J. Thorac. Cardiovasc. Surg.Home page
G. Thabut, H. Mal, Y. Castier, O. Groussard, O. Brugiere, R. Marrash-Chahla, G. Leseche, and M. Fournier
Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis
J. Thorac. Cardiovasc. Surg., August 1, 2003; 126(2): 469 - 475.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
F. Laghi and M. J. Tobin
Disorders of the Respiratory Muscles
Am. J. Respir. Crit. Care Med., July 1, 2003; 168(1): 10 - 48.
[Abstract] [Full Text] [PDF]

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 Am. J. Pathol.Home page
M. Chilosi, V. Poletti, A. Zamo, M. Lestani, L. Montagna, P. Piccoli, S. Pedron, M. Bertaso, A. Scarpa, B. Murer, et al.
Aberrant Wnt/{beta}-Catenin Pathway Activation in Idiopathic Pulmonary Fibrosis
Am. J. Pathol., May 1, 2003; 162(5): 1495 - 1502.
[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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 Am. J. Respir. Crit. Care Med.Home page
N. Mayer-Hamblett, M. Rosenfeld, J. Emerson, C. H. Goss, and M. L. Aitken
Developing Cystic Fibrosis Lung Transplant Referral Criteria Using Predictors of 2-Year Mortality
Am. J. Respir. Crit. Care Med., December 15, 2002; 166(12): 1550 - 1555.
[Abstract] [Full Text] [PDF]

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 ChestHome page
M. Selman
From Anti-inflammatory Drugs Through Antifibrotic Agents to Lung Transplantation : A Long Road of Research, Clinical Attempts, and Failures in the Treatment of Idiopathic Pulmonary Fibrosis
Chest, September 1, 2002; 122(3): 759 - 761.
[Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
M. R. Khoshnevis, T. E. King Jr., and J. Tooze
Histopathology and prediction of survival in usual interstitial pneumonia
Am. J. Respir. Crit. Care Med., May 15, 2002; 165(10): 1451 - 1452.
[Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
M. J. TOBIN
Tuberculosis, Lung Infections, Interstitial Lung Disease, and Socioeconomic Issues in AJRCCM 2001
Am. J. Respir. Crit. Care Med., March 1, 2002; 165(5): 631 - 641.
[Full Text] [PDF]


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