Role of HRCT Detection Scleroderma: nonspecific interstitial - - PowerPoint PPT Presentation

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Role of HRCT Detection Scleroderma: nonspecific interstitial - - PowerPoint PPT Presentation

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11/5/2016 Radiology Radiology Approach to ILD Brett M. Elicker, MD University of Pathology Clinical California, San Francisco Normal PFTs Role of HRCT Detection Scleroderma: nonspecific interstitial pneumonia 1 11/5/2016 End

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11/5/2016 1

Radiology Approach to ILD

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

Radiology Pathology Clinical

Role of HRCT

  • Detection

Normal PFTs

Scleroderma: nonspecific interstitial pneumonia

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11/5/2016 2

End stage constrictive bronchiolitis

Inspiration

Markedly reduced FEV1

Expiration

HRCT may show reduced sensitivity for:

  • Small airways diseases

– Constrictive broncholitis – Hypersensitivity pneumonitis – Asthma

  • Emphysema
  • Pulmonary hypertension

NSIP + pulmonary hypertension

Markedly reduced DLCO

Role of HRCT

  • Detection
  • Diagnosis
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Confidence in diagnosis

Definitive HRCT pattern HRCT + clinical: diagnostic Nonspecific HRCT

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Clinical Context

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

Pathology: the gold standard

Adenocarcinoma Scar

Pathology: NSIP Diagnosis: HP

Role of HRCT

  • Detection
  • Diagnosis
  • Fibrosis vs. inflammation
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Fibrosis vs. Inflammation

No GGO- fibrosis GGO- inflammation GGO- fibrosis

Role of HRCT

  • Detection
  • Diagnosis
  • Fibrosis vs. inflammation
  • Next step in management

HRCT guides further work-up

Bronchoscopy Sputum VATS

Role of HRCT

  • 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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? diagnosis

Histologic Pattern Idiopathic Clinical Syndrome Associated Diseases Usual interstitial pneumonia Idiopathic pulmonary fibrosis Connective tissue disease (CTD), drugs, asbestosis Nonspecific interstitial pneumonia (NSIP) Idiopathic NSIP CTD, drugs, hypersensitivity pneumonitis (HP) Desquamative interstitial pneumonia (DIP) Idiopathic DIP Smoking, CTD, drugs, toxic inhalation Organizing pneumonia (OP) Cryptogenic OP CTD, drugs, infections, chronic eosinophilic pneumonia, HP Constrictive bronchiolitis (CB) Idiopathic CB Post-viral, CTD, drugs, graft vs. host disease, lung transplant rejection Diffuse alveolar damage Acute interstitial pneumonia Infection, aspiration, trauma, sepsis, pancreatitis, etc.

Radiology <-> Pathology

Histologic Pattern Idiopathic Clinical Syndrome Associated Diseases Usual interstitial pneumonia Idiopathic pulmonary fibrosis Connective tissue disease (CTD), drugs, asbestosis Nonspecific interstitial pneumonia (NSIP) Idiopathic NSIP CTD, drugs, hypersensitivity pneumonitis (HP) Desquamative interstitial pneumonia (DIP) Idiopathic DIP Smoking, CTD, drugs, toxic inhalation Organizing pneumonia (OP) Cryptogenic OP CTD, drugs, infections, chronic eosinophilic pneumonia, HP Constrictive bronchiolitis (CB) Idiopathic CB Post-viral, CTD, drugs, graft vs. host disease, lung transplant rejection Diffuse alveolar damage Acute interstitial pneumonia Infection, aspiration, trauma, sepsis, pancreatitis, etc. Histologic Pattern Idiopathic Clinical Syndrome Associated Diseases Usual interstitial pneumonia Idiopathic pulmonary fibrosis Connective tissue disease (CTD), drugs, asbestosis Nonspecific interstitial pneumonia (NSIP) Idiopathic NSIP CTD, drugs, hypersensitivity pneumonitis (HP) Desquamative interstitial pneumonia (DIP) Idiopathic DIP Smoking, CTD, drugs, toxic inhalation Organizing pneumonia (OP) Cryptogenic OP CTD, drugs, infections, chronic eosinophilic pneumonia, HP Constrictive bronchiolitis (CB) Idiopathic CB Post-viral, CTD, drugs, graft vs. host disease, lung transplant rejection Diffuse alveolar damage Acute interstitial pneumonia Infection, aspiration, trauma, sepsis, pancreatitis, etc.

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Usual interstitial pneumonia (HRCT)

Raghu et al. Am J Respir Crit Care Med 2011; 183: 788 Definite UIP Possible UIP Inconsistent with UIP Irregular reticulation Irregular reticulation Honeycombing NO honeycombing Subpleural, basilar distribution Subpleural, basilar distribution Mid-upper lung distribution OR not subpleural distribution Absence of features inconsistent with UIP Absence of features inconsistent with UIP OR presence of features inconsistent with UIP

Definite UIP: IPF

  • Honeycombing features

– Air density – Round with fairly thick wall – Always involves subpleural lung – Associated signs of fibrosis

UIP: non-idiopathic causes

Asbestosis Rheumatoid Drug

What % of patients have IPF?

Raghu et al. Am J Respir Crit Care Med 2011; 183: 788

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

95% 65% 25%

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Findings inconsistent with UIP

  • 1. Ground glass opacity
  • 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 (NSIP)

Subpleural sparing

Nonspecific interstitial pneumonia

Bronchiectasis

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Desquamative interstitial pneumonia

Cystic abnormality

Desquamative interstitial pneumonia VATS: UIP Explant: HP

Mosaic perfusion ≥3 lobes

Lymphoid interstitial pneumonia

Cysts + nodules Definitely not “possible UIP”

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Idiopathic pulmonary fibrosis

Inconsistent with UIP Ground glass opacity

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% – UIP: 40%

  • Radiology

– Definite/possible UIP (22%) – Honeycombing (32%)

Familial: surfactant protein C mutation Radiology Approach to ILD

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