Idiopathic pulmonary fibrosis: Relationship between histopathologic features and mortality

Talmadge E. King, Marvin I. Schwarz, Kevin Brown, Janet A. Tooze, Thomas V. Colby, James A. Waldron, Andrew Flint, William Thurlbeck, Reuben M. Cherniack

Research output: Contribution to journalArticle

473 Citations (Scopus)

Abstract

It is hypothesized that the extent and severity of fibrosis and cellularity found on lung biopsy determine the prognosis and response to therapy in idiopathic pulmonary fibrosis (IPF). The objective of this study was to determine which histopathologic features predict survival in IPF. We prospectively studied 87 patients with usual interstitial pneumonia (UIP) confirmed by surgical lung biopsy. Four pathologists independently graded the extent and severity of specific histopathologic features. We used Cox proportional-hazards models to assess the effect of histopathologic patterns on patients' survival. The effects of age, sex, and smoking were also included in the analysis. Sixty-three patients died during the 17-yr study period. Survival was longer in subjects with lesser degrees of granulation/connective tissue deposition (fibroblastic foci). The degree of alveolar space cellularity, alveolar wall fibrosis, and cellularity did not affect survival. A history of cigarette smoking, the level of dyspnea, and the degree of lung stiffness at presentation were also shown to be independent factors predicting survival. The extent of fibroblastic foci present on lung biopsy predicts survival in IPF. These findings support the hypothesis that the critical pathway to end-stage fibrosis is not "alveolitis" but rather the ongoing epithelial damage and repair process associated with persistent fibroblastic proliferation. Controlling these processes, rather than stopping inflammation, appears most important in preventing progressive disease and the fatal outcome common in IPF.

Original languageEnglish (US)
Pages (from-to)1025-1032
Number of pages8
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume164
Issue number6
StatePublished - Sep 15 2001
Externally publishedYes

Fingerprint

Idiopathic Pulmonary Fibrosis
Survival
Mortality
Lung
Fibrosis
Biopsy
Smoking
Critical Pathways
Fatal Outcome
Granulation Tissue
Proportional Hazards Models
Connective Tissue
Dyspnea
Inflammation

Keywords

  • Idiopathic pulmonary fibrosis
  • Prospective studies
  • Pulmonary fibrosis physiopathology
  • Smoking physiopathology
  • Survival rate
  • Usual interstitial pneumonia

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

King, T. E., Schwarz, M. I., Brown, K., Tooze, J. A., Colby, T. V., Waldron, J. A., ... Cherniack, R. M. (2001). Idiopathic pulmonary fibrosis: Relationship between histopathologic features and mortality. American Journal of Respiratory and Critical Care Medicine, 164(6), 1025-1032.

Idiopathic pulmonary fibrosis : Relationship between histopathologic features and mortality. / King, Talmadge E.; Schwarz, Marvin I.; Brown, Kevin; Tooze, Janet A.; Colby, Thomas V.; Waldron, James A.; Flint, Andrew; Thurlbeck, William; Cherniack, Reuben M.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 164, No. 6, 15.09.2001, p. 1025-1032.

Research output: Contribution to journalArticle

King, TE, Schwarz, MI, Brown, K, Tooze, JA, Colby, TV, Waldron, JA, Flint, A, Thurlbeck, W & Cherniack, RM 2001, 'Idiopathic pulmonary fibrosis: Relationship between histopathologic features and mortality', American Journal of Respiratory and Critical Care Medicine, vol. 164, no. 6, pp. 1025-1032.
King TE, Schwarz MI, Brown K, Tooze JA, Colby TV, Waldron JA et al. Idiopathic pulmonary fibrosis: Relationship between histopathologic features and mortality. American Journal of Respiratory and Critical Care Medicine. 2001 Sep 15;164(6):1025-1032.
King, Talmadge E. ; Schwarz, Marvin I. ; Brown, Kevin ; Tooze, Janet A. ; Colby, Thomas V. ; Waldron, James A. ; Flint, Andrew ; Thurlbeck, William ; Cherniack, Reuben M. / Idiopathic pulmonary fibrosis : Relationship between histopathologic features and mortality. In: American Journal of Respiratory and Critical Care Medicine. 2001 ; Vol. 164, No. 6. pp. 1025-1032.
@article{136d32566b4b4a1a84310c0ad6cb57d0,
title = "Idiopathic pulmonary fibrosis: Relationship between histopathologic features and mortality",
abstract = "It is hypothesized that the extent and severity of fibrosis and cellularity found on lung biopsy determine the prognosis and response to therapy in idiopathic pulmonary fibrosis (IPF). The objective of this study was to determine which histopathologic features predict survival in IPF. We prospectively studied 87 patients with usual interstitial pneumonia (UIP) confirmed by surgical lung biopsy. Four pathologists independently graded the extent and severity of specific histopathologic features. We used Cox proportional-hazards models to assess the effect of histopathologic patterns on patients' survival. The effects of age, sex, and smoking were also included in the analysis. Sixty-three patients died during the 17-yr study period. Survival was longer in subjects with lesser degrees of granulation/connective tissue deposition (fibroblastic foci). The degree of alveolar space cellularity, alveolar wall fibrosis, and cellularity did not affect survival. A history of cigarette smoking, the level of dyspnea, and the degree of lung stiffness at presentation were also shown to be independent factors predicting survival. The extent of fibroblastic foci present on lung biopsy predicts survival in IPF. These findings support the hypothesis that the critical pathway to end-stage fibrosis is not {"}alveolitis{"} but rather the ongoing epithelial damage and repair process associated with persistent fibroblastic proliferation. Controlling these processes, rather than stopping inflammation, appears most important in preventing progressive disease and the fatal outcome common in IPF.",
keywords = "Idiopathic pulmonary fibrosis, Prospective studies, Pulmonary fibrosis physiopathology, Smoking physiopathology, Survival rate, Usual interstitial pneumonia",
author = "King, {Talmadge E.} and Schwarz, {Marvin I.} and Kevin Brown and Tooze, {Janet A.} and Colby, {Thomas V.} and Waldron, {James A.} and Andrew Flint and William Thurlbeck and Cherniack, {Reuben M.}",
year = "2001",
month = "9",
day = "15",
language = "English (US)",
volume = "164",
pages = "1025--1032",
journal = "American Journal of Respiratory and Critical Care Medicine",
issn = "1073-449X",
publisher = "American Thoracic Society",
number = "6",

}

TY - JOUR

T1 - Idiopathic pulmonary fibrosis

T2 - Relationship between histopathologic features and mortality

AU - King, Talmadge E.

AU - Schwarz, Marvin I.

AU - Brown, Kevin

AU - Tooze, Janet A.

AU - Colby, Thomas V.

AU - Waldron, James A.

AU - Flint, Andrew

AU - Thurlbeck, William

AU - Cherniack, Reuben M.

PY - 2001/9/15

Y1 - 2001/9/15

N2 - It is hypothesized that the extent and severity of fibrosis and cellularity found on lung biopsy determine the prognosis and response to therapy in idiopathic pulmonary fibrosis (IPF). The objective of this study was to determine which histopathologic features predict survival in IPF. We prospectively studied 87 patients with usual interstitial pneumonia (UIP) confirmed by surgical lung biopsy. Four pathologists independently graded the extent and severity of specific histopathologic features. We used Cox proportional-hazards models to assess the effect of histopathologic patterns on patients' survival. The effects of age, sex, and smoking were also included in the analysis. Sixty-three patients died during the 17-yr study period. Survival was longer in subjects with lesser degrees of granulation/connective tissue deposition (fibroblastic foci). The degree of alveolar space cellularity, alveolar wall fibrosis, and cellularity did not affect survival. A history of cigarette smoking, the level of dyspnea, and the degree of lung stiffness at presentation were also shown to be independent factors predicting survival. The extent of fibroblastic foci present on lung biopsy predicts survival in IPF. These findings support the hypothesis that the critical pathway to end-stage fibrosis is not "alveolitis" but rather the ongoing epithelial damage and repair process associated with persistent fibroblastic proliferation. Controlling these processes, rather than stopping inflammation, appears most important in preventing progressive disease and the fatal outcome common in IPF.

AB - It is hypothesized that the extent and severity of fibrosis and cellularity found on lung biopsy determine the prognosis and response to therapy in idiopathic pulmonary fibrosis (IPF). The objective of this study was to determine which histopathologic features predict survival in IPF. We prospectively studied 87 patients with usual interstitial pneumonia (UIP) confirmed by surgical lung biopsy. Four pathologists independently graded the extent and severity of specific histopathologic features. We used Cox proportional-hazards models to assess the effect of histopathologic patterns on patients' survival. The effects of age, sex, and smoking were also included in the analysis. Sixty-three patients died during the 17-yr study period. Survival was longer in subjects with lesser degrees of granulation/connective tissue deposition (fibroblastic foci). The degree of alveolar space cellularity, alveolar wall fibrosis, and cellularity did not affect survival. A history of cigarette smoking, the level of dyspnea, and the degree of lung stiffness at presentation were also shown to be independent factors predicting survival. The extent of fibroblastic foci present on lung biopsy predicts survival in IPF. These findings support the hypothesis that the critical pathway to end-stage fibrosis is not "alveolitis" but rather the ongoing epithelial damage and repair process associated with persistent fibroblastic proliferation. Controlling these processes, rather than stopping inflammation, appears most important in preventing progressive disease and the fatal outcome common in IPF.

KW - Idiopathic pulmonary fibrosis

KW - Prospective studies

KW - Pulmonary fibrosis physiopathology

KW - Smoking physiopathology

KW - Survival rate

KW - Usual interstitial pneumonia

UR - http://www.scopus.com/inward/record.url?scp=0035883582&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0035883582&partnerID=8YFLogxK

M3 - Article

C2 - 11587991

AN - SCOPUS:0035883582

VL - 164

SP - 1025

EP - 1032

JO - American Journal of Respiratory and Critical Care Medicine

JF - American Journal of Respiratory and Critical Care Medicine

SN - 1073-449X

IS - 6

ER -