Jointly provided by The Potomac Center for Medical Education and Rockpointe

This activity is supported by an independent educational grant from

Boehringer Ingelheim Pharmaceuticals, Inc.

Steering Committee

Jonathan H. Chung, MDAssociate Professor of Radiology

Associate Section Chief, Thoracic Radiology

The University of Chicago Medicine

Chicago, IL

Mary M. Salvatore, MD, MBAAssistant Professor of Thoracic Radiology

Mount Sinai Hospital

New York, NY

Disclosures

Steering Committee and Program Faculty

The steering committee and faculty reported the following relevant financial relationships that they or their spouse/partner have with commercial interests:

Jonathan H. Chung, MD – Consultant: ACI Clinical, Boehringer Ingelheim, Genentech, Veracyte; Speaker: Elsevier

Mary M. Salvatore, MD, MBA – Speaker's Bureau: Boehringer Ingelheim, Genentech

Non-faculty Content Contributors

Non-faculty content contributors and/or reviewers reported the following relevant financial relationships that they or their spouse/partner have with commercial interests:

Jeanelle Spencer, PhD; Blair St. Amand; Jay Katz, CHCP; Lindsay Scott, PT, DPT, ATC:Nothing to disclose

Polling Question

Please rate your level of confidence in determining definite, possible, and inconsistent UIP patterns in HRCT images?

1. Not confident

2. Slightly confident

3. Confident

4. Very confident

5. Expert

Polling Question

How familiar are you with non-radiological features of IPF?

1. Not familiar

2. Slightly familiar

3. Familiar

4. Very familiar

5. Expert

Learning Objectives

• Recognize IPF-specific patterns and differentiate usual interstitial pneumonia (UIP) categories (definite, possible, and inconsistent) in HRCT images, distinguishing IPF from other pulmonary conditions

• Identify barriers to effective communication with referring physicians and initiate strategies to enhance the quality of the communication to gather relevant histories, discuss analyses of HRCTs, and ensure accurate diagnoses

Interstitial Lung Disease

Known causes

• Hypersensitivity pneumonitis

• Asbestosis

• Drug reactions

• Connective tissue disease

Idiopathic

UIP Usual interstitial pneumonia

NSIP Nonspecific interstitial

pneumonia

COP Cryptogenic organizing

pneumonia

AIP Acute interstitial pneumonitis

RBILD Respiratory bronchiolitis-

associated interstitial lung

disease

DIP Desquamative interstitial

pneumonia

LIP Lymphocytic interstitial

pneumonia

PPFE Pleuroparenchymal

fibroelastosis

Factors which increase a person’s risk for pulmonary fibrosis

Pulmonary fibrosis

Male

Genetic

Drugs

Occupation

Smoking

Older age

Idiopathic Pulmonary Fibrosis (IPF) 101

• Idiopathic pulmonary fibrosis (IPF) most common and deadly type of pulmonary fibrosis

• Similar survival as compared to non-small cell lung cancer

• Usual interstitial pneumonitis (UIP) is the imaging pattern and histology in IPF

• Conversely, UIP almost always IPF (>95%)

• In 2014, FDA-approved new therapies for the treatment of patients with IPF

Xia, Meng, et al. American Thoracic Society, 2016;A2697-A2697.

Recognizing the IPF Pattern

Secondary Pulmonary Lobule

Interlobular

septa

Bronchus

Artery

Alveoli

ATS Guidelines for UIP

• Subpleural basilar predominant fibrosis

• Reticulations

• Honeycombing

• Absence of features that would suggest and alternative diagnosis

Raghu G, et al. Am J Respir Crit Care Med. 2011;183:788-824.

CANNOT B UIP

• Consolidation

• Air trapping

• Nodules

• Non-solid or ground glass

• O is for holes or cysts

• T is for top

• B is for bronchovascular

Polling Question

What key radiological feature is depicted in this image?

1. Honeycombing

2. Bronchiectasis

3. Cysts

4. Ground-glass opacities

5. Nodules

Honeycombing vs Bronchiectasis

Polling Question

The pattern seen in this HRCT scan meets the ATS Guidelines criteria for…

1. IPF

2. Definite UIP

3. Possible UIP

4. Inconsistent with UIP

5. NSIP

ATS Guidelines for Possible UIP

• Subpleural basilar predominant fibrosis

• Reticulations

• Honeycombing

• Absence of features that would suggest and alternative diagnosis

Possible UIP

Subpleural and basilar

predominant

Possible UIP

Reticulations

Pre-honeycombing to Honeycombing

Reticulations Honeycombing

ATS Guidelines for Inconsistent with UIP

• Inconsistent with UIP pattern (any of the seven features)

– Upper or mid-lung predominance

– Peribronchovascular predominance

– Extensive ground-glass abnormality (extent>reticular abnormality)

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

– Profuse micronodules (bilateral, predominantly upper lobes)

– Discrete cysts (multiple, bilateral, away from areas of honeycombing)

– Diffuse mosaic attenuation/air-trapping (bilateral, in three or more lobes)

Distribution

Increased

density

Decreased

density

Raghu G, et al. Am J Respir Crit Care Med., 2011;183:788-824.

Upper Lobe Fibrosis

• Upper lobe fibrosis with volume loss

• Relative sparing of the lower lobes

• Centrilobular nodules

Ground-glass Opacity

• Ground-glass opacity and traction bronchiectasis in NSIP

Polling Question

55-year-old female with dyspnea – What is the most likely diagnosis?

1. CHP

2. IPF

3. NSIP

4. Sarcoidosis

5. UIP

Polling Question

37-year-old with chronic cough – What is the most likely diagnosis?

1. CHP

2. IPF

3. NSIP

4. Sarcoidosis

5. UIP

Other Common Non-UIP Diagnoses

Mimickers of Fibrosis

Fibrosis caused

by osteophyte

Reversible

atelectasis

SarcoidCHP

NSIPUIP

NSIP

• Lower lobe fibrosis

• Follows bronchovasculardistribution

• Homogeneous

• Ground-glass opacity

• Dilated esophagus

Sasaki, Shinichi, et al. American Thoracic Society, 2016. A4999-A4999.

NSIP

• Lower lobe fibrosis

• Follows bronchovasculardistribution

• Homogeneous

• Ground-glass opacity

• Dilated esophagus

Chronic Hypersensitivity Pneumonitis

• Upper lobe predominant

• Bronchovasculardistribution

• Air trapping

Silva C, et al. Radiology. 2008;246:288-297.

Chronic Hypersensitivity Pneumonitis

Centrilobular nodules and early fibrosis

Stage 4 Sarcoidosis

• Upper lobe predominant fibrosis

• Bronchovasculardistribution

Brauner MW, et al. Radiology 1989;172:467-471.

Stage 4 Sarcoidosis

Upper lobe predominant fibrosis

that follows bronchovascular

distribution

Important Non-pulmonary Findings

Hiatal Hernia

Hiatal hernia and gastro-esophageal reflux are associated

with fibrosis of the lung

Pulmonary Artery

• Pulmonary artery enlargement is associated with pulmonary hypertension and can be seen in patients with fibrosis

Enlarged Lymph Nodes

• Mediastinal lymphadenopathy can occur in patients with pulmonary fibrosis

Souza CA, al. Am J Roentgenol. 2006;186:995-999.

Combined Pulmonary Fibrosis and Emphysema

Honeycombing

Emphysema

Non-radiological Features that Point Toward IPF Diagnosis

• Older age

• Male sex

• History of smoking

• Basilar crackles

• Decreased DLCO, FVC

• Decreased performance on 6 minute walk test

Raghu G, et al. Am J Respir Crit Care Med. 2011;183:788-824. Rosewarne D, et al. Imaging. 2014;20:289-302.

Radiological Features that Point Toward IPF Diagnosis

• Lower lobe predominant fibrosis

• Sub-pleural fibrosis

• Reticulations and traction bronchiectasis

• Honeycombing

• Volume loss

• Absence of consolidation, air trapping, nodules

Raghu G, et al. Am J Respir Crit Care Med. 2011;183:788-824. Rosewarne D, et al. Imaging. 2014;20:289-302.

Case Review

Case 1: 55-year-old Female with Dyspnea

What is the most likely diagnosis?

Case 1: 55-year-old Female with Dyspnea

• NSIP

– Lower lobe predominant fibrosis

– Bronchovasculardistribution

– Absence of honeycombing

– Ground-glass opacity

Case 2: 73-year-old Female with Long-standing Dyspnea

What is the most likely diagnosis?

Case 2: 73-year-old Female with Long-standing Dyspnea

• CHP

– Upper lobe fibrosis

– Bronchovascular

– Air trapping

Case 3: 37-year-old with Chronic Cough

What is the most likely diagnosis?

Case 3: 37-year-old with Chronic Cough

• Stage 4 Sarcoid

– Upper lobe fibrosis

– Bronchovasculardistribution

– Cystic changes

Case 4: 45-year-old with Chronic Cough

What is the most likely diagnosis?

Case 4: 45-year-old with Chronic Cough

• Organizing pneumonia

– Consolidation

– Bronchovasculardistribution

Case 5: 68-year-old Female with Increasing Shortness of Breath and Cough

What is the most likely diagnosis?

Case 5: 68-year-old Female with Increasing Shortness of Breath and Cough

• Possible UIP

– Subpleuralbasilar predominant fibrosis

– Absence of honeycombing

– Reticulations and traction bronchiectasis

Case 6: 78-year-old Man with Shortness of Breath

What is the most likely diagnosis?

Case 6: 78-year-old Man with Shortness of Breath

• UIP

– Subpleural basilar predominant fibrosis

– Honeycombing

– Reticulations and traction bronchiectasis

Radiology and Pulmonary Communication

• Radiology report should provide supporting words for correct diagnosis

• Ancillary findings in report include pulmonary artery size, hiatal hernia, and liver cirrhosis

• Discussion with pulmonologist for first CT to review clinical information

• Multidisciplinary conference is gold standard

Post-activity Survey

Polling QuestionPost-activity Survey

What key radiological feature is depicted in this image?

1. Honeycombing

2. Bronchiectasis

3. Cysts

4. Ground-glass opacities

5. Nodules

Polling QuestionPost-activity Survey

The pattern seen in this HRCT scan meets the ATS Guidelines criteria for…

1. IPF

2. Definite UIP

3. Possible UIP

4. Inconsistent with UIP

5. NSIP

Polling QuestionPost-activity Survey

55-year-old female with dyspnea – What is the most likely diagnosis?

1. CHP

2. IPF

3. NSIP

4. Sarcoidosis

5. UIP

Polling QuestionPost-activity Survey

37-year-old with chronic cough – What is the most likely diagnosis?

1. CHP

2. IPF

3. NSIP

4. Sarcoidosis

5. UIP

Questions

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