What is Idiopathic Pulmonary Fibrosis

All patients with suspected IPF should undergo high-resolution computed tomography (HRCT) evaluation.^1-3 HRCT allows a detailed examination of the lung parenchyma.²

An HRCT for evaluating ILD should follow guidelines for improving diagnostic results.³

• Non-contrast
• Thin collimation (≤/2 cm), short rotation time, highest pitch for motion-free scan
• Number of acquisitions in proper position (3):
  1. Supine, sustained end-inspiration
  2. Supine, entire thorax at sustained end-expiration after prolonged expiration (volumetric or sequential acquisition)
  3. Volumetric or sequential image (can be limited to lower lobes) to clear position-induced changes
• Scan for new ground-glass changes, acute pulmonary embolism via CT angiography with/without non contrast HRCT

UIP PATTERN

There are 4 categories of usual interstitial pneumonia (UIP) pattern based on HRCT findings.

The 2018 ATS/ERS/JRS/ALAT guidelines define 4 categories for diagnosing UIP based on the presence or absence of specific HRCT features.³

The HRCT criteria were devised to convey a level of confidence for a radiologic diagnosis of UIP in a patient with clinical features that suggest a diagnosis of IPF.⁴

HRCT in IPF

The distribution and predominance of specific features on HRCT scans are key factors in determining UIP patterns.⁵

UIP: Criteria

HRCT criteria for usual interstitial pneumonia. The features necessary for a UIP diagnosis are:^3,6

• Subpleural and basal predominance (distribution is often heterogeneous)
  AND
• Honeycombing with or without peripheral traction bronchiectasis/bronchiolectasis

UIP: Example

UIP shows:^3,6

• Subpleural and basal predominant distribution
• Honeycombing
• Traction bronchiectasis

The distribution of UIP is characteristically basal and peripheral, though often patchy.^1,3,5,6

Image courtesy of and used with permission from Robert Suh, MD.

Honeycombing is critical for an HRCT diagnosis of UIP without surgical biopsy.^1,3,5-7

In cases of UIP, ground-glass opacities (GGO) may be present but should be less extensive than the reticulation.^1,3,6 Mediastinal lymphadenopathy may be present.³

PROBABLE UIP

Criteria

Criteria for a diagnosis of probable UIP:^3,6

• Subpleural and basal predominance (often heterogeneous distribution)
  AND
• Reticular pattern with peripheral traction bronchiectasis/bronchiolectasis
  AND
• May have mild ground-glass opacities

Example

Lacking honeycombing, probable UIP exhibits bronchiectasis and may have histopathologic signs of UIP upon biopsy. Surgical biopsy therefore may aid in IPF diagnosis.³

Note the lack of honeycombing in this image.

HRCT image of possible usual interstitial pneumonia. Reprinted from Fishman‘s Pulmonary Diseases and Disorders. 4th edition 2007. Meltzer EB and Noble PW: Chapter 70, Idiopathic Pulmonary Fibrosis.¹⁰ Used with permission from McGraw-Hill Companies. Inc. Copyright © 2007 McGraw-Hill Companies. Inc.

Surgical lung biopsy is not always possible. A surgical biopsy may be deferred due to underlying comorbidities, poor pulmonary function, or patient preference.^1,3

INDETERMINATE FOR UIP

Criteria

Criteria for a diagnosis of indeterminate for UIP:^3,6

• Subpleural and basal predominance
  AND
• Subtle reticulation, may have ground-glass opacity (mild) or distortion (“early UIP pattern”)
  AND
• Distribution or features of fibrosis not consistent with specific etiology (“truly indeterminate”)

Example

When characteristics suggestive of fibrosis are visible on HRCT scans but do not meet the criteria of UIP or probable UIP, the images should be classified as indeterminate for UIP.⁵ UIP pattern may be found on histological testing of patients with HRCT patterns indeterminate for UIP.^3,6

Image courtesy of and used with permission from Robert Suh, MD.

ALTERNATIVE DIAGNOSIS

The presence of any of these features should lead to the consideration of an alternative diagnosis.^3,6

CT Features:

• Mosaic attenuation
• Predominant GGO
• Consolidation
• Cysts, nodules (profuse or centrilobular)

Predominant Distribution:

• Peribronchovascular
• Perilymphatic
• Upper or mid-lung

Other:

• Pleural plaques (consider asbestosis)
• Extensive lymph node enlargement
• Distal clavicular erosions (consider rheumatoid arthritis)
• Dilated esophagus, pleural effusions, pleural thickening (consider connective tissue disease)

Upper-lung predominance

Image courtesy of and used with permission from David A Lynch, MD

Peribronchovascular predominance

Image courtesy of and used with permission from David A Lynch, MD

Extensive ground-glass abnormalities

Image courtesy of and used with permission from David A Lynch, MD

Profuse perilymphatic micronodules

Image courtesy of and used with permission from David A Lynch, MD

Discrete cysts

Image courtesy of and used with permission from Robert Suh, MD

Diffuse mosaic attenuation/air trapping

Image courtesy of and used with permission from David A Lynch, MD

Consolidation in bronchopulmonary segment(s)/lobe(s)

Image courtesy of and used with permission from David A Lynch, MD

HRCT is not always enough to diagnose IPF

Patients who are clinically suspected of having IPF with HRCT features of either probable UIP, indeterminate UIP, or alternative diagnosis should undergo additional diagnostic evaluation.³

Only a subset of possible IPF cases are candidates for a lung biopsy.

Patients whose HRCT results are non-diagnostic for IPF may undergo a surgical lung biopsy to reach a definite IPF diagnosis.^5,6,17 Surgical lung biopsy is not indicated in patients at high risk for intra-, peri-, or postoperative complications (e.g., severe hypoxemia at rest and/or severe pulmonary hypertension with a diffusion capacity <25% after correction for hematocrit). A multidisciplinary discussion is needed before a diagnosis of IPF is made.

The decision to pursue lung biopsy must take into account underlying comorbidities, pulmonary function, and patient preference.^5,6

Choosing the right biopsy method is crucial for making an accurate diagnosis.

A biopsy should take samples from multiple lobes, preferably via video-assisted thoracoscopic approaches to obtain adequate tissue for histologic diagnosis.^5,6,17

Transbronchial biopsies via fiberoptic bronchoscopy are not recommended in diagnosing IPF due to low yield.^6,10,17

Recommendations differ based on HRCT evidence of UIP or the potential for something other than UIP:⁵

• Surgical or transbronchial lung biopsies are not recommended for patients with a pattern of UIP on HRCT^5,6,17
• The 2018 ATS/ERS/JRS/ALAT guidelines recommend surgical biopsy for patients with an HRCT pattern of probable UIP, indeterminate UIP, or alternative diagnosis⁵
• However, the 2018 Fleischner Society White Paper suggests surgical lung biopsy is only needed in cases where HRCT patterns are indeterminate for UIP or suggest an alternative diagnosis¹⁷

THERE ARE 4 CATEGORIES OF UIP PATTERN IN HISTOPATHOLOGY.

The histopathologic features of UIP fall into 1 of 4 categories:^5,17

• UIP
• Probable UIP
• Indeterminate for UIP
• Alternative Diagnosis

UIP PATTERN^5,17

Evidence of dense fibrosis with architectural distortion (i.e., honeycombing and/or destructive scarring) in a predominantly subpleural/paraseptal distribution
AND
Predominant subpleural and/or paraseptal distribution of fibrosis
AND
Presence of patchy involvement of lung parenchyma by fibrosis
AND
Presence of fibroblast foci
AND
Absence of features to suggest an alternate diagnosis (see Alternate Diagnosis section)

The chief diagnostic criterion of UIP is patchy dense fibrosis at low magnification.⁵

UIP shows alternating areas of patchy pulmonary parenchymal fibrosis with scarring and honeycomb changes and less affected or normal parenchyma.^57,58

Histopathologic features of usual interstitial pneumonia. Reprinted from Fishman's Pulmonary Diseases and Disorders, 4th edition 2007. Meltzer, EB and Noble, PW: Chapter 70, Idiopathic Pulmonary Fibrosis. Used with permission from McGraw-Hill Companies, Inc. Copyright © 2007 McGraw-Hill Companies, Inc.¹⁰

Histopathologic changes in IPF often affect the subpleural and paraseptal parenchyma most severely.^5,17

The involved areas of the lung show complete distortion of normal architecture, with sheets of dense collagen replacing normal lung tissue and occasional microscopic honeycomb cysts.¹⁰

Histopathologic features of usual interstitial pneumonia. Reprinted from Fishman's Pulmonary Diseases and Disorders, 4th edition 2007. Meltzer, EB and Noble, PW: Chapter 70, Idiopathic Pulmonary Fibrosis. Used with permission from McGraw-Hill Companies, Inc. Copyright © 2007 McGraw—Hill Companies, Inc.¹⁰

At high magnification, the detailed features of definite UIP are visible.

Areas of honeycomb change are composed of cystic fibrotic airspaces that are frequently lined by bronchiolar epithelium and filled with mucus and inflammatory cells.⁵⁸

Histopathologic features of usual interstitial pneumonia. Reprinted from Fishman's Pulmonary Diseases and Disorders, 4th edition 2007. Meltzer, EB and Noble, PW: Chapter 70, Idiopathic Pulmonary Fibrosis. Used with permission from McGraw-Hill Companies, Inc. Copyright © 2007 McGraw-Hill Companies, Inc.¹⁰

As the region of scarred lung tissue encroaches upon the areas of normal lung tissue, the advancing edge of the young fibrosis contains specialized structures known as fibroblast foci.^10,57,58

Fibroblastic foci may reflect a reticulum of scar tissue.

Fibrosis may occur as a reticulum of scar tissue that extends from the pleura to the central portions of lung rather than existing as discrete areas of fibrosis.⁵⁹

These findings, observed together, are diagnostic of UIP, assuming atypical findings are absent.^5,6

Histopathologic features of usual interstitial pneumonia. Reprinted from Fishman's Pulmonary Diseases and Disorders, 4th edition 2007. Meltzer, EB and Noble, PW: Chapter 70, Idiopathic Pulmonary Fibrosis. Used with permission from McGraw—Hill Companies, Inc. Copyright © 2007 McGraw—Hill Companies, Inc.

UIP is not limited to IPF.

The UIP pattern can be found in several other diseases, including:^10,17

• Connective tissue diseases
• Asbestosis
• Chronic hypersensitivity pneumonitis
• Hermansky-Pudlak syndrome
• Drug toxicities

Clinical history is essential for distinguishing IPF from other disorders that also produce a UIP pattern on biopsy.⁶

UIP may occur simultaneously with patterns of other lung diseases.

• It is important to recognize that UIP may be present along with patterns consistent with other lung diseases, such as emphysema^60,61
• In these cases, changes characteristic of each disease are expected to be present⁶¹

PROBABLE UIP PATTERN

The following features are suggestive of a probable UIP pattern:^5,17

Honeycomb Changes
OR
Some features of UIP, but precludes a definitive diagnosis of UIP/IPF
AND
Absence of features suggestive of an alternative diagnosis

INDETERMINATE UIP PATTERN

An indeterminate UIP pattern includes all of the following criteria:^5,17

Fibrosis with or without architectural distortion with features supporting a pattern other than UIP or UIP secondary to another cause^a
AND
Features of UIP along with features suggesting an alternative diagnosis^b

ALTERNATIVE DIAGNOSIS

Presence of the following features is considered indicative of an alternative diagnosis and should lead to the consideration of other diseases.^5,17

• All biopsies have features of other histologic patterns of IIPs (absence of fibroblast foci, loose fibrosis)
• Histologic findings indicative of other diseases – sarcoidosis, Langerhans cell histiocytosis, hypersensitivity pneumonitis, lymphangioleiomyomatosis (LAM)

SHOULD ALL PATIENTS WITH IPF BE TREATED WITH ANTIACID MEDICATIONS?

In 2011, the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association published evidence-based clinical practice guidelines for the diagnosis and treatment of IPF.¹

Recently, these guidelines were updated based on new evidence reported since publication of the prior guidelines, and treatment recommendations were updated accordingly.² Addressed specifically was the question, “Should patients with IPF be treated with antiacid medications?”

Gastroesophageal reflux (GER) is very common among patients with IPF, occurring in over 90% of patients, even in the absence of classical reflux symptoms.³ GER is a risk factor for aspiration and microaspiration, which could lead to pneumonitis.²

It is postulated that this could cause or worsen IPF. Accordingly, the risk for microaspiration-associated lung injury or damage may be decreased with the use of antiacid treatments, such as proton pump inhibitors (PPIs) or histamine-2 blocker receptor antagonists (H2RAs).

WHAT THEY DID

Pertinent literature was obtained through a search strategy using medical subject heading keywords and text words.² The Ovid platform was used to search MEDLINE, EMBASE, Cochrane Registry of Controlled Trials, Health Technology Assessment, and the Database of Abstracts of Reviews of Affects for May 2010 through May 2014.

Two reviewers screened titles and abstracts to identify articles for full review and evaluated the full text of articles deemed potentially relevant by either reviewer. Results from identified studies with the same treatment agent were pooled, and meta-analyses were reviewed.

WHAT THEY FOUND

A survival benefit for patients receiving antiacid medication (hazard ratio [HR], 0.47; 95% CI, 0.24-0.93; adjusted analysis) was suggested by 1 retrospective analysis of longitudinal cohorts.⁴

A separate aggregate analysis of 3 randomized, controlled trials of different pharmacologic therapies in patients with IPF examined all the patients who were randomized to the placebo groups. Researchers compared 124 patients receiving a PPI or H2RA at baseline (91% PPI, 9% H2RA) to 118 patients not receiving antiacid treatment and not receiving other study medications.

For those receiving antiacid treatment at baseline, a significantly smaller decrease in forced vital capacity during the study period was found (mean difference, 0.07 L; 95% CI, 0-0.14; P=.05).⁵ Of note, in patients who received antiacid treatment, there were no episodes of adjudicated acute exacerbations compared with placebo. However, there were no differences in all-cause mortality or all-cause hospitalization.

WHAT IT MEANS

Based on these data, the guidelines suggest that “clinicians use regular antiacid treatment for patients with IPF (conditional recommendation, very low confidence in estimates of effect).”² In making this recommendation, the guidelines committee placed a higher value on possible improved lung function and survival and the low cost of therapy, and a lower value on the potential increased risk for pneumonia with antiacid therapy.

Abstract

REFERENCES

Pulmonary rehabilitation (PR) is the cornerstone of management of individuals with chronic respiratory diseases and has been shown to improve symptoms, exercise tolerance, and quality of life in patients with a variety of respiratory diseases including interstitial lung disease (ILD).¹

Based on data from 2 controlled trials of PR in IPF, the 2011 ATS/ERS/JRS/ALAT IPF guidelines recommend that the majority of patients with IPF should be treated with PR.² PR programs consist of aerobic conditioning, strength and flexibility training, educational lectures, nutritional interventions, and psychosocial support.²

Of concern is the fact that the educational content of PR was developed for patients with chronic obstructive pulmonary disease (COPD) and covers topics that may not be applicable to patients with IPF, as the conditions are dissimilar in terms of the physiological basis of exercise imitations, symptoms, treatment, disease progression, and outcomes.³ In order to address this issue, Holland, Fiore, Goh, Symons, et al queried patients and clinicians to determine the educational topics that should be included in PR programs for patients with ILD.³

WHAT THEY DID

The authors conducted individual semi structured interviews with 18 patients with ILD, 9 of whom had IPF, and 14 clinicians who treat patients with ILD.³ Questions focused on the educational needs of people with ILD and drew upon a previous appraisal of PR information needs for patients with COPD along with suggested ILD education topics in a published expert review.

WHAT THEY FOUND

Most patients with IPF understood the serious and progressive nature of their condition.³ Across the patient interviews, the strongest theme was the wish for PR clinicians to be honest about their prognosis and provide information about what to expect over the course of the disease.

In addition, patients indicated a desire for information on end-of-life planning and felt comfortable addressing the topic in a group setting. Most patients indicated a willingness to attend standard PR education sessions but desired ILD-specific content. For example, patients noted that they would like information on managing cough, how to manage medications and their side effects, and strategies to limit disease progression.

Among clinicians, management of cough and use of oxygen therapy were topics consistently acknowledged as critical components of ILD education. Additionally, most clinicians noted that patients needed to understand the types of treatments available, including the role of clinical trials.

There was disagreement among clinicians regarding whether prognosis and advanced care planning should be discussed in PR. Some felt that discussion of these topics should be handled by the treating physician and/or that the topics were not appropriate for a group setting.

WHAT IT MEANS

Patients with ILD have specific educational needs that are not currently addressed in standard PR education programs.³

Abstract

REFERENCES

CURRENT GUIDELINES FOR MANAGING IPF

An Official ATS/ERS/JRS/ALAT Statement: Evidence-Based Guidelines For Managing IPF.

Excerpted from: Raghu G, Collard HR, Egan JJ et al. Am J Respir Crit Care Med. 2011;183(6)788-824.

Note: this document was published in 2011. A revised version was published in 2018. Certain aspects of this document may be out of date and caution should be used when applying these in clinical practice or other usages.

Note: text in italics are excerpts from Raghu G, Remy-Jardin M, Myers JL et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

MANAGING IPF¹

• IPF is a fatal lung disease whose natural history is variable and unpredictable
• Although nonpharmacologic strategies and comorbidity management have shown variable effect on patient outcomes, many of these approaches are recommended by the 2011 ATS/ERS/JRS/ALAT guidelines for patients with IPF

NONPHARMACOLOGIC TREATMENTS FOR IPF

OXYGEN THERAPY^1,2

Question: Should patients with IPF and resting hypoxemia receive long-term oxygen therapy?

There is no data that directly informs the use of long-term oxygen therapy in patients with IPF. One study has retrospectively compared survival in a cohort of patients with IPF many of whom (27%) received oxygen therapy. In multivariate analysis, no survival benefit was demonstrated with oxygen use. This study was limited by its retrospective design...

Recommendation: We recommend that patients with IPF and clinically significant resting hypoxemia should be treated with long-term oxygen therapy (strong recommendation, very low-quality evidence).

Values: This recommendation places a high value on evidence from other chronic lung diseases and a low value is an inconvenience...

The guidelines recommend long-term oxygen supplementation for patients with IPF and clinically significant hypoxemia.

LUNG TRANSPLANTATION^1,2

Question: Should appropriate patients with IPF undergo lung transplantation?

Five-year survival rates after lung transplantation in IPF are estimated at 50 to 56%. A single-center study of 46 patients referred for lung transplantation with IPF demonstrated a reduced risk of death at 5 years in patients receiving lung transplantation. Additional evidence suggests that patients with pulmonary fibrosis undergoing lung transplantation have favorable long-term survival compared with other disease indications...

Recommendation: We recommend that appropriate patients with IPF should undergo lung transplantation (strong recommendation, low-quality evidence).

Values: This recommendation places a high value on low-quality evidence showing a...

The guidelines recommend lung transplantation for appropriate patients with IPF.

MECHANICAL VENTILATION^1,2

Question: Should patients with respiratory failure due to IPF receive mechanical ventilation?

There are several small studies of mechanical ventilation in patients with IPF and respiratory failure, all of which show a high hospital mortality rate. The inclusion criteria varied among studies, with some only including patients with respiratory failure of unknown etiology...

Recommendation: The majority of patients with respiratory failure due to IPF should not receive mechanical ventilation, but mechanical ventilation may be a reasonable intervention in a minority (weak recommendation, low-quality evidence).

Values: This recommendation places a high value on the high mortality observed in this patient population and on reducing...

The guidelines do not recommend mechanical ventilation for the majority of patients with IPF and respiratory failure.

PULMONARY REHAB^1,2

Question: Should patients with IPF receive pulmonary rehabilitation ?

Pulmonary rehabilitation programs involve aerobic conditioning, strength and flexibility training, educational lectures, nutritional interventions, and psychosocial support. Pulmonary rehabilitation has recently been studied in patients with ILD. Two controlled trials of pulmonary rehabilitation in IPF have demonstrated an improvement in walk distance and symptoms or quality of life...

Recommendation: The majority of patients with IPF should be treated with pulmonary rehabilitation, but pulmonary rehabilitation may not be reasonable in a minority (weak recommendation, low-quality evidence).

Values: This recommendation places a high value on moderate-quality data demonstrating improvement in functional status and…

The guidelines recommend that the majority of patients with IPF receive pulmonary rehabilitation.

TREATING COMORBIDITIES^1,2

Treatment of Selected Complications and Comorbid Conditions

There is an increasing awareness of complications and comorbid conditions frequently associated with IPF. These include acute exacerbation of IPF, pulmonary hypertension, gastroesophageal reflux disease, obesity, emphysema, and obstructive sleep apnea. It is unknown if treating these comorbidities influences clinical outcomes. There is no data on which to make recommendations for treatment of obesity, emphysema, and obstructive sleep apnea in the setting of IPF.

Treating comorbidities may or may not affect outcomes for patients with IPF. Check the guidelines for recommendations on treating individual comorbidities.

PALLIATIVE CARE^1,2

Palliative care focuses on reducing symptoms and providing comfort to patients, rather than treating patients’ disease. Specific goals for palliative care include relief from physical and emotional suffering and consideration for psychological and spiritual support for patients and caregivers. Such care will need to be individualized. Palliative care should be considered an adjunct to disease-focused care. Worsening of symptoms such as cough and dyspnea are common and difficult to treat. Limited data suggest that corticosteroids and thalidomide may be beneficial for chronic cough in IPF. Chronic opioids may be used for severe dyspnea and cough; careful monitoring for side effects should be performed. Advanced directives and end-of-life care issues should be addressed...

Individualized care should focus on relieving physical and emotional suffering, as well as psychological and spiritual support for patients and caregivers.

It should be used as an adjunct to disease-based care.

MONITORING DISEASE PROGRESSION^1,2

Monitoring the Clinical Course of Disease

Monitoring of patients with IPF is necessary to proactively identify patients with progressive disease, to appreciate worsening of symptoms and oxygenation, and to detect the development of disease or treatment complications. In addition, careful assessment of the clinical course is useful in helping patients understand their disease course and in initiating timely, appropriate therapeutic interventions, including consideration of lung transplantation.

Patients should be monitored regularly to detect worsening symptoms and oxygenation, as well as developing comorbidities (disease- or treatment- based).

Monitoring for Progressive Disease

Disease progression may be manifested by increasing respiratory symptoms, worsening pulmonary function test results, progressive fibrosis on high resolution computed tomography (HRCT), or acute respiratory decline. In the absence of another identifiable cause, the presence of any of the following changes is consistent with progressive disease:

• Progressive dyspnea (objectively assessed)
• Progressive, sustained decrease from baseline in absolute forced vital capacity (FVC)
• Progressive, sustained decrease from baseline in absolute diffusing capacity for carbon monoxide (DL_CO) (corrected for hemoglobin)
• Progression of fibrosis from baseline on HRCT
• Acute exacerbation
• Death from respiratory failure

These parameters were developed based on data from clinical trials (see STAGING AND PROGNOSIS). While progressive dyspnea is an important subjective variable, objective...

DISEASE PROGRESSION¹

Changes associated with disease progression include:

• Progressive dyspnea
• Progressive, sustained decrease from baseline in absolute FVC or DL_CO
• Progressive fibrosis from baseline on HRCT
• Acute exacerbation
• Death from respiratory failure

MONITORING FOR WORSENING SYMPTOMS^1,2

Identifying patients with worsening respiratory symptoms (e. g., dyspnea) has important management implications. Patients experiencing worsening respiratory symptoms require evaluation for progressive disease, assessment of oxygenation at rest and with exertion, and prompt detection of secondary complications (e. g., development of deep venous thrombosis and pulmonary embolus). In addition, some patients may benefit from symptom-based therapies. There are several research tools available for the quantification of dyspnea. It is unclear if any of these tools have clinical utility.

Identifying patients with worsening symptoms is important to properly manage their treatment.

MONITORING FOR OXYGENATION^1,2

Monitoring for Worsening Oxygenation

Oxygen saturation by pulse oximetry should be measured at rest and with exertion in all patients regardless of symptoms to assure adequacy of oxygenation and identify the need for supplemental oxygen at baseline and during follow-up evaluation. Careful attention to the pulse oximetry tracing and signal is required to overcome potential problems related to poor circulation and inadequate signal quality. Generally, desaturation below 88% during a formal 6-minute walk test (6MWT) or equivalent has been used to prescribe supplemental oxygen. Such measurements should be performed at baseline and during follow up at 3- to 6-month intervals. Formal cardiopulmonary exercise testing does not have a defined role and is not recommended for routine monitoring.

Monitoring oxygenation levels is critical to a timely prescription for supplemental oxygen.

• Oxygenation should be measured at rest and exertion at baseline and follow-up appointments
• Desaturation of ≤88% should generally lead to use of supplemental oxygen

MONITORING FOR COMORBIDITIES^1,2

Monitoring for Complications and Comorbidities

Comorbidities including pulmonary hypertension, pulmonary embolism, lung cancer, and coronary artery disease are known to occur in IPF. While the development of these comorbidities may influence survival, the role of routine screening to identify such complications in patients with IPF (e.g., annual HRCT for lung cancer surveillance) is unknown.

Thus, a recommendation for routine screening cannot be made. In patients demonstrating progressive disease, the identification of pulmonary hypertension may impact consideration for lung transplantation in eligible patients, and evaluation is indicated. Echocardiography is inaccurate in estimating pulmonary hemodynamics in patients with fibrotic lung disease and should not be relied upon to assess the presence and severity of...

The guidelines do not provide recommendations for routine screening for comorbidities.

• Acute respiratory worsening should lead to evaluation for acute exacerbation of IPF

IS DAILY HOME SPIROMETRY AN EFFECTIVE TOOL FOR DETECTING DISEASE PROGRESSION IN IPF?