MD Conference Express ATS 2013 - (Page 30)
S E L E C T E D U P D A T E S O N I D I O PA T H I C P U L M O N A R Y F I B R O S I S
Adding Insult to Injury: Complications of
Idiopathic Pulmonary Fibrosis
Written by Phil Vinall
Gregory Tino, MD, University of Pennsylvania School
of Medicine, Philadelphia, Pennsylvania, USA, defined
idiopathic pulmonary fibrosis (IPF) as a distinctive form of
chronic interstitial lung disease occurring primarily in older
adults, more often male [Raghu G et al. Am J Respir Crit Care
Med 2011]. The estimated prevalence of IPF is 14 to 43 per
100,000 and an estimated incidence is 7 to 16 per 100,000,
depending on how it is defined [Raghu G et al. Am J Respir
Crit Care Med 2006]. IPF is often associated with smoking,
environmental factors, viral factors, and chronic aspiration
associated with gastroesophageal reflux disease (GERD),
although its etiology is not well understood. Mortality is
high, increasing, and most often due to pulmonary fibrosis,
cardiovascular disease, lung cancer, or pneumonia (Figure
1). Prognosis can be predicted by the radiographic extent
of the disease, amount of fibrosis, presence of pulmonary
hypertension, and comorbidities (emphysema, lung cancer,
and coronary artery disease [CAD]).The median time to
death after diagnoses is 3.2 years [Ley B et al. Ann Intern
Med 2012], based on the GAP (Gender, Age and Physiology)
staging system, a simple scoring model that uses commonly
measured clinical and physiologic variables to predict
mortality in patients with IPF.
Figure 1. IPF Epidemiology: Mortality Rates
Number of deaths (men)
Number of deaths (women)
Age-adjusted mortality rate (men)
Age-adjusted mortality rate (women)
75
8000
70
7000
65
6000
60
5000
55
4000
50
3000
45
2000
40
1000
35
0
1992
Mortality Rate per 1,000,000 Population
80
9000
Number of Deaths
10,000
30
1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003
Year
Reprinted with permission of the American Thoracic Society. Olson AL et al. Mortality from
Pulmonary Fibrosis Increased in the United States from 1992 to 2003. Am J Respir Crit Care
Med . 2006;176:277-284. Official journal of the American Thoracic Society.
Acute exacerbations of IPF are common and frequently
fatal, but not well understood. Harold R. Collard, MD,
University of California, San Francisco, San Francisco,
California, USA, reported that acute exacerbations of
IPF represent a distinct entity defined as acute, clinically
30
July 2013
significant deteriorations of unidentifiable cause in
patients with underlying IPF. Proposed diagnostic criteria
include subjective worsening over ≤30 days, new bilateral
radiographic opacities, the absence of microbiological
evidence of infection, and exclusion of other causes of
acute worsening [Collard HR et al. Am J Respir Crit Care
Med 2007]. Acute exacerbation is a significant predictor of
poor survival, and appears to be more common in patients
with low forced vital capacity and diffusing capacity of the
lung for carbon monoxide [Song JW et al. Eur Respir J 2011].
Potential triggers of acute exacerbation are viral
infection, surgical procedures, microaspiration, and ambient
pollution. Preventive measures, including vaccination,
avoidance of unnecessary surgery and pollutant exposures,
and evaluation and management of GERD, may have a
role. Treatment is largely supportive but most patients with
acute exacerbation will be treated with antibiotics and
corticosteroids [Collard HC et al. Resp Med 2007]. There is a
>50% mortality at 90 days in most cohorts of patients postacute exacerbation with a median survival of 2.2 months
[Song JW et al. Eur Respir J 2011] .
CAD has a high prevalence in patients with IPF and
has a significant impact on mortality (Figure 2). In a recent
study, patients with IPF were more significantly likely
to also have CAD compared with chronic obstructive
pulmonary disease (COPD; 65.8% vs 46.1%; p<0.028).
Unsuspected significant CAD was found in 18% of IPF
patients versus 10.9% of those with COPD (p<0.004)
[Nathan SD et al. Respir Med 2010]. In his presentation
Steven D. Nathan, MD, George Mason University, Virginia
Commonwealth University, Falls Church, Virginia, USA,
speculated that IPF might promote atherosclerosis
through either cytokine cross talk or plaque instability
leading to hypoxia. Although not significant, IPF status
appeared predictive of the presence of CAD (adjusted
OR, 1.67; 95% CI, 0.59 to 4.78) [Nathan SD et al.
Respir Med 2010] as well as for the occurrence of firsttime coronary syndromes [Hubbard RB et al. Am J Respir
Crit Care Med 2008].
Dr. Nathan stated that coronary calcification, assessed
by routine CT of the chest, is a good technique for predicting
underlying significant CAD in patients with IPF. The sensitivity
of moderate to severe calcification was 81%, while the specificity
was 85%, with an associated OR of 25.2 (4.64 to 166, p <0.005)
with excellent radiologist and pulmonologist agreement in
the grading of the coronary calcification [Nathan SD et al.
Respirology 2011; Weir N et al. Am J Respir Crit Care Med 2010].
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Table of Contents for the Digital Edition of MD Conference Express ATS 2013
MD Conference Express ATS 2013
Contents
Prevention and Early Treatment of Acute Lung Injury
Nocturnal Noninvasive Ventilation Improves Outcomes in Multiple Disorders
Hospital Readmissions: Challenges and Opportunities
EBUS-TBNA: Accurate and Safe for Detecting Sarcoidosis
Data Link Obstructive Sleep Apnea and Type 2 Diabetes
Statin Use Improves Respiratory-Related Mortality in Patients With COPD
Addition of Spironolactone to Ambrisentan May Be a Novel Treatment Strategy to Improve Outcome in Patients With PAH
Haloperidol Does Not Prevent Delirium in Ventilated ICU Patients
Beraprost Plus Sildenafil Effective in Pulmonary Arterial Hypertension
Dupilumab Is Safe and Effective for Controlling Asthma Attacks
Once-Daily QVA149 Improves Breathlessness in COPD Patients
CPAP in CVD and OSA Does Not Significantly Improve Cardiovascular Biomarkers
CPAP Reduces BP in Patients With Resistant Hypertension and Obstructive Sleep Apnea
Effects of Obesity on COPD
Pulmonary Embolism
Ventilator-Associated Pneumonia
Lung Cancer Screening
Idiopathic Pulmonary Fibrosis
Non-Small-Cell Lung Cancer
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