Idiopathic Pulmonary Fibrosis Follow-up

  • Author: Amanda Godfrey, MD; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: May 23, 2012
 

Further Inpatient Care

The clinical course of patients with idiopathic pulmonary fibrosis is generally marked by a decline in pulmonary function over time. Increasingly, patients have been recognized as having an acute, and often fatal, clinical deterioration, termed an acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF).

The following are diagnostic criteria for an AE-IPF:[51]

  • Previous or concurrent diagnosis of idiopathic pulmonary fibrosis
  • Unexplained worsening or development of dyspnea within 30 days
  • High-resolution computed tomography (HRCT) scan with new bilateral ground-glass abnormality and/or consolidation superimposed on a background reticular or honeycomb pattern consistent with a usual interstitial pneumonia pattern
  • Worsening hypoxemia from a known baseline arterial blood gas measurement
  • No evidence of pulmonary infection by endotracheal aspiration or bronchoalveolar lavage (BAL)
  • Exclusion of alternative causes, including left-sided heart failure, pulmonary embolism, and an identifiable cause of acute lung injury

Patients with idiopathic pulmonary fibrosis who develop an acute clinical deterioration often require hospitalization. These patients should undergo HRCT imaging of the chest to document the interval development of significant ground-glass opacities, which are suggestive of an AE-IPF. Additionally, a BAL should be completed to examine the possibility of infectious etiologies. Support with supplemental oxygen should be given to alleviate hypoxemia.[51]

Once infection has been acceptably ruled out and other alternative causes of the acute deterioration have been excluded, treatment with intravenous methylprednisolone (Solu-Medrol) at 2 mg/kg/d for 2 weeks followed by a prolonged taper can be given.[51] However, no randomized controlled trials support a particular therapy for an AE-IPF. If a patient with an AE-IPF develops respiratory failure and requires invasive mechanical ventilation, plateau pressures should be maintained at less than 30 cm water.[51] Patients with idiopathic pulmonary fibrosis who require mechanical ventilation have a poor prognosis.

In a retrospective review, of 461 patients with idiopathic pulmonary fibrosis, 20.8% of all subjects experienced an AE-IPF during the median follow-up period of 22.9 months. Approximately 50% of patients hospitalized for an AE-IPF died during the hospitalization. The 1-year and 5-year survival rates from the initial diagnosis of an AE-IPF were 56.2% and 18.4%, respectively.[52] Therefore, an acute exacerbation of idiopathic pulmonary fibrosis has a serious impact on the overall survival of patients with idiopathic pulmonary fibrosis.

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Further Outpatient Care

The rate of decline and progression to death in patients with idiopathic pulmonary fibrosis may take several clinical forms, including slow physiologic deterioration with worsening severity of dyspnea, rapid deterioration and progression to death, or periods of relative stability interposed with periods of acute respiratory decline sometimes manifested by hospitalizations for respiratory failure.[4] Therefore, all patients with idiopathic pulmonary fibrosis should be seen by a pulmonologist on a regular basis for a complete history and physical examination. Patients must undergo disease-specific monitoring with serial assessments of lung physiology, gas exchange, exercise performance, and HRCT to further refine prognosis and management decisions. Patients must be asked about adverse medication effects.

Any patient with idiopathic pulmonary fibrosis who is a current smoker should be encouraged to quit and offered pharmacologic therapy if needed.

Vaccination against influenza and pneumococcal infection should be encouraged in all patients with idiopathic pulmonary fibrosis.

Patients with idiopathic pulmonary fibrosis should be evaluated for pulmonary rehabilitation and should be encouraged to participate in regular exercise to maintain a maximal degree of musculoskeletal conditioning.

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Transfer

Lung transplantation for idiopathic pulmonary fibrosis has been shown to confer a survival benefit over medical therapy. Any patient diagnosed with idiopathic pulmonary fibrosis or probable idiopathic pulmonary fibrosis should be referred to a lung transplantation center for lung transplant evaluation, regardless of the vital capacity.[36]

Patients with idiopathic pulmonary fibrosis should be referred to institutions where they can be counseled regarding enrollment in a trial of an investigational agent for the treatment of idiopathic pulmonary fibrosis.

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Complications

The following are complications that can be seen in patients with idiopathic pulmonary fibrosis:

  • Pulmonary hypertension
  • Acute exacerbation of pulmonary fibrosis
  • Respiratory infection
  • Acute coronary syndrome
  • Thromboembolic disease
  • Adverse medication effects
  • Lung cancer
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Prognosis

A worse prognosis can be expected based on various clinical parameters, physiologic factors, radiographic findings, histopathologic findings, laboratory findings, and bronchoalveolar lavage findings. du Bois et al evaluated a scoring system to predict individual risk of mortality. They used a Cox proportional hazards model and data from two clinical trials (n = 1,099) to identify independent predictors of 1-year mortality among patients with IPF. The findings demonstrated that 4 readily ascertainable predictors (age, history of respiratory hospitalization within the previous 24 weeks, percent predicted FVC, and 24-week change in FVC) could be used in a scoring system to estimate 1-year mortality. However, this scoring system needs to be validated in other populations of patients with IPF.[53]

Ley et al used competing risks regression modeling to retrospectively screen potential predictors of mortality in a derivation cohort of patients with IPF (n = 228). They identified a model consisting of 4 predictors (sex, age, % predicted FVC, and % predicted DLCO). Based on these 4 predictors, they developed a simple point-score model and staging system that was retrospectively validated in a separate cohort of patients with IPF (n = 330).[54]

Table 1. Scoring for mortality risk in IPF. (Open Table in a new window)

PredictorPoints
SexFemale0
Male1
Age (years)≥600
61-651
>652
FVC (% predicted)>750
50-751
< 502
DLCO (% predicted)>550
36-551
≤352
Cannot perform3

Table 2. Staging and mortality risk for IPF. (Open Table in a new window)

StageIIIIII
Points0-34-56-8
Mortality
1-year5.616.239.2
2-year10.929.962.1
3-year16.342.176.8

The authors believe that the index and staging system provide clinicians with a framework for discussing prognosis, policy-makers with a tool for investigating stage-specific management options, and researchers with the ability to identify at-risk study populations that maximize the efficiency and power of clinical trials.[54]

Patients with idiopathic pulmonary fibrosis who have concomitant pulmonary hypertension have more dyspnea, greater impairment of their exercise capacity, and increased 1-year mortality compared with their counterparts without pulmonary hypertension.[3] Additionally, a multicenter prospective cohort study of 126 lung transplant procedures performed for idiopathic pulmonary fibrosis revealed elevated pulmonary artery pressure as a risk factor for primary graft dysfunction (PGD) following lung transplantation.[55] The mean pulmonary artery pressure (mPAP) for patients with PGD following lung transplantation was 38.5 ± 16.3 mm Hg compared with a mPAP of 29.6 ± 11.5 mm Hg in patients without PGD following lung transplantation.

Patients with UIP pattern on HRCT imaging have a worse prognosis compared with patients with biopsy-proven usual interstitial pneumonia and atypical changes of idiopathic pulmonary fibrosis on HRCT imaging.[24, 4]

Patients who have a greater than 10% decline in forced vital capacity (FVC) (percent predicted) over 6 months have a 2.4-fold increased risk of death. Additionally, in patients who do not desaturate to less than 88% during a 6-minute walk test (6MWT), the only strong predictor of mortality is a progressive decline in FVC (>10% after 6 mo).[26]

A baseline diffusion capacity of carbon monoxide (DLCO) below 35% is correlated with increased mortality. Additionally, a decline in DLCO greater than 15% over 1 year is also associated with increased mortality.[26]

Desaturation below the threshold of 88% during the 6MWT has been associated with an increased mortality.[26] Additionally, in patients with idiopathic pulmonary fibrosis who desaturate to less than 88% during a 6MWT, a progressive decline in DLCO (>15% after 6 mo) is a strong predictor of mortality.[28]

BAL fluid neutrophilia has been demonstrated to predict early mortality. One study demonstrated a linear relationship between increasing neutrophil percentage and the risk of mortality. Each doubling in baseline BAL fluid neutrophil percentage was associated with a 30% increased risk of death or transplantation in the first year after presentation.[32]

Serum surfactant protein A (SP-A) is a member of the collectin family. SP-A is secreted by type II pneumocytes, and the level of SP-A appears to be increased early after breakdown in the alveolar epithelium. SP-A has been shown to be present in abnormal amounts in the BAL fluid of patients with idiopathic pulmonary fibrosis.[56] In a cohort study, after controlling for known clinical predictors of mortality, each increase of 49 ng/mL in baseline serum SP-A level was associated with a 3.3-fold increased risk of mortality in the first year after presentation.[56] Therefore, serum SP-A is independently and strongly associated with death or lung transplantation 1 year after presentation.[56]

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Patient Education

Patients should be presented information regarding the full range of options available for treating idiopathic pulmonary fibrosis. The pros, cons, risks, benefits, and alternatives should be discussed in a balanced and comprehensive fashion. For patient education resources, see the Lung and Airway Center.

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Contributor Information and Disclosures
Author

Amanda Godfrey, MD  Fellow, Pulmonary and Critical Care Medicine, Henry Ford Hospital

Amanda Godfrey, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Thoracic Society, and Michigan State Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Ouellette, MD, FCCP  Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP  Professor of Genomics and Personalized Medicine Research, Internal Medicine, and Pediatrics, Associate Director, Center for Genomics and Personalized Medicine Research, Director of Research, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Wake Forest University School of Medicine

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, and Sigma Xi

Disclosure: See below for list of all activities None None

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Daniel R Ouellette, MD, FCCP  Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society

Disclosure: Nothing to disclose.

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Rajesh G. Patel, MD, and Javier I. Diaz, MD, to the development and writing of this article.

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Chest radiograph of a patient with idiopathic pulmonary fibrosis showing bilateral lower lobe reticular opacities (red circles).
Classic subpleural honeycombing (red circle) in a patient with a diagnosis of idiopathic pulmonary fibrosis.
A patient with IPF and a confirmed histologic diagnosis of usual interstitial pneumonia. Note the reticular opacities (red circle) distributed in both lung bases and the minimal ground-glass opacities (blue circle).
A patient with nonspecific interstitial pneumonia. Note the predominance of ground-glass opacities (blue circles) and a few reticular lines (red arrow).
Patchwork distribution of abnormalities in a classic example of usual interstitial pneumonia (low-magnification photomicrograph; hematoxylin and eosin stain; original magnification, X4). Courtesy of Chad Stone, MD.
Table 1. Scoring for mortality risk in IPF.
PredictorPoints
SexFemale0
Male1
Age (years)≥600
61-651
>652
FVC (% predicted)>750
50-751
< 502
DLCO (% predicted)>550
36-551
≤352
Cannot perform3
Table 2. Staging and mortality risk for IPF.
StageIIIIII
Points0-34-56-8
Mortality
1-year5.616.239.2
2-year10.929.962.1
3-year16.342.176.8
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