Natural history of idiopathic pulmonary fibrosis

Research output: Contribution to journalReview article

44 Citations (Scopus)

Abstract

Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. IPF has a poor prognosis, with a median survival time of 2-3 years from diagnosis, but varying from a few months to a decade. The natural history of IPF is highly variable and the course of disease in an individual patient is difficult to predict. Some patients with IPF experience rapid decline, others progress much more slowly, and some patients show periods of relative stability interspersed with acute deteriorations in respiratory function. Many clinical, radiographic, serologic, and histopathologic variables have been shown to predict mortality in IPF. However, the accuracy of these predictors varies due to the retrospective nature of some of the studies and variations in study design. The ability to identify clinical characteristics that predict disease progression and survival would be useful for counseling patients, treatment decision-making, and prompt consideration for lung transplantation. A number of indices for predicting mortality in patients with IPF are available, but they require further validation. As high-resolution computed tomography scans become more widely available and patients with IPF are diagnosed earlier, survival times following diagnosis will improve. Early referral to interstitial lung disease specialty centers is important for accurate diagnosis and may be associated with improved outcomes. The goal of this review is to examine the natural history of IPF, discuss predictors of mortality, and highlight the importance of prompt diagnosis and referral for patients with IPF.

Original languageEnglish (US)
Pages (from-to)661-670
Number of pages10
JournalRespiratory Medicine
Volume109
Issue number6
DOIs
StatePublished - Jun 1 2015

Fingerprint

Idiopathic Pulmonary Fibrosis
Survival
Mortality
Referral and Consultation
Aptitude
Lung Transplantation
Interstitial Lung Diseases
Lung Diseases
Disease Progression
Counseling
Decision Making
Fibrosis
Tomography

Keywords

  • Acute exacerbations
  • Idiopathic pulmonary fibrosis
  • Natural history
  • Predictors of mortality
  • Progression

Cite this

Natural history of idiopathic pulmonary fibrosis. / Kim, Hyun J; Perlman, David M; Tomic, Rade.

In: Respiratory Medicine, Vol. 109, No. 6, 01.06.2015, p. 661-670.

Research output: Contribution to journalReview article

@article{01771510f46a4ffbba87a08e291bc5ae,
title = "Natural history of idiopathic pulmonary fibrosis",
abstract = "Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. IPF has a poor prognosis, with a median survival time of 2-3 years from diagnosis, but varying from a few months to a decade. The natural history of IPF is highly variable and the course of disease in an individual patient is difficult to predict. Some patients with IPF experience rapid decline, others progress much more slowly, and some patients show periods of relative stability interspersed with acute deteriorations in respiratory function. Many clinical, radiographic, serologic, and histopathologic variables have been shown to predict mortality in IPF. However, the accuracy of these predictors varies due to the retrospective nature of some of the studies and variations in study design. The ability to identify clinical characteristics that predict disease progression and survival would be useful for counseling patients, treatment decision-making, and prompt consideration for lung transplantation. A number of indices for predicting mortality in patients with IPF are available, but they require further validation. As high-resolution computed tomography scans become more widely available and patients with IPF are diagnosed earlier, survival times following diagnosis will improve. Early referral to interstitial lung disease specialty centers is important for accurate diagnosis and may be associated with improved outcomes. The goal of this review is to examine the natural history of IPF, discuss predictors of mortality, and highlight the importance of prompt diagnosis and referral for patients with IPF.",
keywords = "Acute exacerbations, Idiopathic pulmonary fibrosis, Natural history, Predictors of mortality, Progression",
author = "Kim, {Hyun J} and Perlman, {David M} and Rade Tomic",
year = "2015",
month = "6",
day = "1",
doi = "10.1016/j.rmed.2015.02.002",
language = "English (US)",
volume = "109",
pages = "661--670",
journal = "Respiratory Medicine",
issn = "0954-6111",
publisher = "W.B. Saunders Ltd",
number = "6",

}

TY - JOUR

T1 - Natural history of idiopathic pulmonary fibrosis

AU - Kim, Hyun J

AU - Perlman, David M

AU - Tomic, Rade

PY - 2015/6/1

Y1 - 2015/6/1

N2 - Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. IPF has a poor prognosis, with a median survival time of 2-3 years from diagnosis, but varying from a few months to a decade. The natural history of IPF is highly variable and the course of disease in an individual patient is difficult to predict. Some patients with IPF experience rapid decline, others progress much more slowly, and some patients show periods of relative stability interspersed with acute deteriorations in respiratory function. Many clinical, radiographic, serologic, and histopathologic variables have been shown to predict mortality in IPF. However, the accuracy of these predictors varies due to the retrospective nature of some of the studies and variations in study design. The ability to identify clinical characteristics that predict disease progression and survival would be useful for counseling patients, treatment decision-making, and prompt consideration for lung transplantation. A number of indices for predicting mortality in patients with IPF are available, but they require further validation. As high-resolution computed tomography scans become more widely available and patients with IPF are diagnosed earlier, survival times following diagnosis will improve. Early referral to interstitial lung disease specialty centers is important for accurate diagnosis and may be associated with improved outcomes. The goal of this review is to examine the natural history of IPF, discuss predictors of mortality, and highlight the importance of prompt diagnosis and referral for patients with IPF.

AB - Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. IPF has a poor prognosis, with a median survival time of 2-3 years from diagnosis, but varying from a few months to a decade. The natural history of IPF is highly variable and the course of disease in an individual patient is difficult to predict. Some patients with IPF experience rapid decline, others progress much more slowly, and some patients show periods of relative stability interspersed with acute deteriorations in respiratory function. Many clinical, radiographic, serologic, and histopathologic variables have been shown to predict mortality in IPF. However, the accuracy of these predictors varies due to the retrospective nature of some of the studies and variations in study design. The ability to identify clinical characteristics that predict disease progression and survival would be useful for counseling patients, treatment decision-making, and prompt consideration for lung transplantation. A number of indices for predicting mortality in patients with IPF are available, but they require further validation. As high-resolution computed tomography scans become more widely available and patients with IPF are diagnosed earlier, survival times following diagnosis will improve. Early referral to interstitial lung disease specialty centers is important for accurate diagnosis and may be associated with improved outcomes. The goal of this review is to examine the natural history of IPF, discuss predictors of mortality, and highlight the importance of prompt diagnosis and referral for patients with IPF.

KW - Acute exacerbations

KW - Idiopathic pulmonary fibrosis

KW - Natural history

KW - Predictors of mortality

KW - Progression

UR - http://www.scopus.com/inward/record.url?scp=84930673517&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84930673517&partnerID=8YFLogxK

U2 - 10.1016/j.rmed.2015.02.002

DO - 10.1016/j.rmed.2015.02.002

M3 - Review article

C2 - 25727856

AN - SCOPUS:84930673517

VL - 109

SP - 661

EP - 670

JO - Respiratory Medicine

JF - Respiratory Medicine

SN - 0954-6111

IS - 6

ER -