11/5/2016

Radiology Approach to Radiology ILD

Brett M. Elicker, MD University of Pathology Clinical California, San Francisco

Normal PFTs Role of HRCT

• Detection

Scleroderma: nonspecific interstitial pneumonia

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End stage constrictive bronchiolitis Inspiration Expiration HRCT may show reduced sensitivity for:

• Small airways diseases – Constrictive broncholitis – Hypersensitivity pneumonitis – Asthma • Emphysema • Pulmonary hypertension Markedly reduced FEV1

NSIP + pulmonary hypertension Role of HRCT

• Detection

• Diagnosis

Markedly reduced DLCO

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Confidence in diagnosis

Definitive HRCT HRCT + clinical: Nonspecific HRCT pattern diagnostic

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Pathology: the gold standard Clinical Context Adenocarcinoma Scar

• Bird exposure -> hypersensitivity pneumonitis • Smoker -> respiratory bronchiolitis • Connective tissue disease -> follicular bronchiolitis • Iron welder -> siderosis • Acute symptoms -> viral infection

Pathology: NSIP Diagnosis: HP Role of HRCT

• Detection

• Diagnosis

• Fibrosis vs. inflammation

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Fibrosis vs. Inflammation Role of HRCT No GGO- fibrosis GGO- inflammation GGO- fibrosis

• Detection

• Diagnosis

• Fibrosis vs. inflammation

• Next step in management

HRCT guides further work-up Role of HRCT Bronchoscopy Sputum VATS

• Detection

• Diagnosis

• Fibrosis vs. inflammation

• Next step in management

• Serial changes

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HRCT: follow-up after tx Clinical/PFT deterioration 6 months later Initial

Acute exacerbation of IPF Role of HRCT

• Detection

• Diagnosis

• Fibrosis vs. inflammation

• Next step in management

• Serial changes

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Idiopathic Clinical Histologic Pattern Associated Diseases ? diagnosis Syndrome Usual interstitial Idiopathic pulmonary Connective tissue disease pneumonia fibrosis (CTD), drugs, asbestosis CTD, drugs, Nonspecific interstitial Idiopathic NSIP hypersensitivity pneumonia (NSIP) pneumonitis (HP) Desquamative interstitial Smoking, CTD, drugs, toxic Idiopathic DIP pneumonia (DIP) inhalation

CTD, drugs, infections, Organizing pneumonia Cryptogenic OP chronic eosinophilic (OP) pneumonia, HP

Post-viral, CTD, drugs, Constrictive bronchiolitis Idiopathic CB graft vs. host disease, lung (CB) transplant rejection Infection, aspiration, Acute interstitial Diffuse alveolar damage trauma, sepsis, pneumonia pancreatitis, etc.

Idiopathic Clinical Histologic Pattern Associated Diseases Radiology <-> Pathology Syndrome Usual interstitial Idiopathic pulmonary Connective tissue disease pneumonia fibrosis (CTD), drugs, asbestosis CTD, drugs, Nonspecific interstitial Idiopathic NSIP hypersensitivity pneumonia (NSIP) pneumonitis (HP) Desquamative interstitial Smoking, CTD, drugs, toxic Idiopathic DIP pneumonia (DIP) inhalation

CTD, drugs, infections, Organizing pneumonia Cryptogenic OP chronic eosinophilic (OP) pneumonia, HP

Post-viral, CTD, drugs, Constrictive bronchiolitis Idiopathic CB graft vs. host disease, lung (CB) transplant rejection Infection, aspiration, Acute interstitial Diffuse alveolar damage trauma, sepsis, pneumonia pancreatitis, etc.

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Definite UIP: IPF Usual interstitial pneumonia (HRCT) • Honeycombing features Raghu et al. Am J Respir Crit Care Med 2011; 183: 788 – Air density Inconsistent with Definite UIP Possible UIP UIP – Round with fairly thick wall – Always involves subpleural Irregular reticulation Irregular reticulation Honeycombing NO honeycombing lung Mid-upper lung – Associated signs of fibrosis Subpleural, basilar Subpleural, basilar distribution OR not distribution distribution subpleural distribution OR presence of Absence of features Absence of features features inconsistent inconsistent with UIP inconsistent with UIP with UIP

UIP: non-idiopathic causes What % of patients have IPF? Raghu et al. Am J Respir Crit Care Med 2011; 183: 788

Asbestosis Drug 95% 65% 25% Inconsistent with Definite UIP Possible UIP UIP Irregular reticulation Irregular reticulation Honeycombing (HC) NO honeycombing Mid-upper lung Subpleural, basilar Subpleural, basilar distribution OR not distribution distribution subpleural distribution OR presence of Rheumatoid Absence of features Absence of features features inconsistent inconsistent with UIP inconsistent with UIP with UIP

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Nonspecific interstitial pneumonia Findings inconsistent with UIP (NSIP)

• 1. Ground glass opacity Subpleural sparing • 2. Mosaic perfusion/air trapping ( ≥3 lobes) • 3. Profuse micronodules • 4. Discrete cysts • 5. Consolidation • 6. Mid-upper lung predominance • 7. Peribronchovascular predominance

Nonspecific interstitial pneumonia

Bronchiectasis

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Desquamative interstitial pneumonia Desquamative interstitial pneumonia

Cystic abnormality

VATS: UIP Explant: HP Lymphoid interstitial pneumonia

Cysts Mosaic + nodules perfusion ≥3 lobes

Definitely not “possible UIP”

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Idiopathic pulmonary fibrosis Familial Interstitial Lung Disease Leslie et al. Arch Pathol Lab Med 2012; 136: 1366 Lee et al. Chest 2012; 142: 1577

• Genetic mutation (e.g. telomerase) or idiopathic • 2-20% cases of IPF • Earlier age of onset (<50 years old) • Pathology – Unclassifiable fibrosis: 60% Ground glass opacity – UIP: 40% • Radiology Inconsistent with UIP – Definite/possible UIP (22%) – Honeycombing (32%)

Radiology Approach to Familial: ILD surfactant protein C mutation Brett M. Elicker, MD University of California, San Francisco

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