5/9/2015 Multi-disciplinary Approach to Diffuse Lung Disease: The Imager’s Perspective No, I am not a pulmonologist! Radiology Pathology Clinical 1
5/9/2015 Everyone needs a CT Confidence in diagnosis � Definitive � HRCT + clinical: � Nonspecific HRCT pattern diagnostic HRCT pattern 2
5/9/2015 Clinical Context � Bird exposure -> hypersensitivity pneumonitis � Smoker -> respiratory bronchiolitis � Connective tissue disease -> follicular bronchiolitis � Iron welder -> siderosis � Acute symptoms -> viral infection, HP Pulmonary function tests vs. HRCT End stage IPF Markedly reduced TLC and DLCO 3
5/9/2015 End stage constrictive bronchiolitis HRCT may show reduced Inspiration Expiration sensitivity for: � Small airways diseases � Constrictive broncholitis � Hypersensitivity pneumonitis � Asthma � Emphysema � Pulmonary hypertension Markedly reduced FEV1 Fibrosis vs. Inflammation NSIP + pulmonary hypertension No GGO- fibrosis GGO- inflammation GGO- fibrosis Markedly reduced DLCO 4
5/9/2015 HRCT guides further work-up HRCT: follow-up after tx Bronchoscopy Sputum VATS Clinical/PFT deterioration Radiology <-> Pathology 6 months later Initial � 1. Microscopic honeycombing � 2. Collagenous fibrosis � 3. Fibroblastic foci � 4. Normal lung 5
5/9/2015 Idiopathic Pulmonary Fibrosis Idiopathic Clinical Idiopathic Clinical Histologic Pattern Histologic Pattern Associated Diseases Associated Diseases Syndrome Syndrome Usual interstitial Usual interstitial Idiopathic pulmonary Idiopathic pulmonary Connective tissue disease Connective tissue disease pneumonia pneumonia fibrosis fibrosis (CTD), drugs, asbestosis (CTD), drugs, asbestosis CTD, drugs, CTD, drugs, Nonspecific interstitial Nonspecific interstitial Idiopathic NSIP Idiopathic NSIP hypersensitivity hypersensitivity pneumonia (NSIP) pneumonia (NSIP) pneumonitis (HP) pneumonitis (HP) Desquamative interstitial Desquamative interstitial Smoking, CTD, drugs, Smoking, CTD, drugs, Idiopathic DIP Idiopathic DIP pneumonia (DIP) pneumonia (DIP) toxic inhalation toxic inhalation CTD, drugs, infections, CTD, drugs, infections, Organizing pneumonia Organizing pneumonia Cryptogenic OP Cryptogenic OP chronic eosinophilic chronic eosinophilic (OP) (OP) pneumonia, HP pneumonia, HP Post-viral, CTD, drugs, Post-viral, CTD, drugs, Constrictive bronchiolitis Constrictive bronchiolitis Idiopathic CB Idiopathic CB graft vs. host disease, lung graft vs. host disease, lung (CB) (CB) transplant rejection transplant rejection Infection, aspiration, Infection, aspiration, Acute interstitial Acute interstitial trauma, sepsis, pancreatitis, trauma, sepsis, pancreatitis, Diffuse alveolar damage Diffuse alveolar damage pneumonia pneumonia etc. etc. Definite UIP: IPF Usual interstitial pneumonia (HRCT) Raghu et al. Am J Respir Crit Care Med 2011; 183: 788 Inconsistent with Definite UIP Possible UIP UIP Irregular reticulation Irregular reticulation Honeycombing NO honeycombing Mid-upper lung 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 6
5/9/2015 UIP: non-idiopathic causes Inconsistent with UIP Drug Asbestosis Final diagnosis: IPF Rheumatoid What % of patients have IPF? Raghu et al. Am J Respir Crit Care Med 2011; 183: 788 95% 85% 25% Familial: Inconsistent with Definite UIP Possible UIP UIP surfactant Irregular reticulation Irregular reticulation protein C Honeycombing (HC) NO honeycombing mutation Mid-upper lung 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 7
5/9/2015 Familial Interstitial Lung Disease UIP (HRCT) Leslie et al. Arch Pathol Lab Med 2012; 136: 1366 Raghu et al. Am J Respir Crit Care Med 2011; 183: 788 Lee et al. Chest 2012; 142: 1577 � Genetic mutation (e.g. telomerase) or idiopathic Inconsistent with Definite UIP Possible UIP UIP � 2-20% cases of IPF Irregular reticulation Irregular reticulation � Earlier age of onset (<50 years old) Honeycombing (HC) NO honeycombing � Pathology Mid-upper lung Subpleural, basilar Subpleural, basilar distribution OR not � Unclassifiable fibrosis: 60% distribution distribution subpleural � UIP: 40% distribution � Radiology OR presence of Absence of features Absence of features features inconsistent � Definite/possible UIP (22%) inconsistent with UIP inconsistent with UIP with UIP � Honeycombing (32%) What diseases/patterns may Findings inconsistent with UIP mimic IPF on HRCT? � 1. Ground glass opacity � 2. Mosaic perfusion/air trapping ( � 3 lobes) � Hypersensitivity pneumonitis � 3. Profuse micronodules � 4. Discrete cysts � Nonspecific interstitial pneumonia (NSIP) � 5. Consolidation � 6. Mid-upper lung predominance � Desquamative interstitial pneumonia � 7. Peribronchovascular predominance 8
5/9/2015 VATS: UIP Explant: HP VATS: UIP Explant: HP UIP vs. NSIP Desquamative interstitial pneumonia Subpleural sparing � Sensitivity for NSIP: 64% Silva et al. Radiology � Specificity for NSIP: 93% 2008; 246: 288 9
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