Chronic dyspnea of pulmonary origin DIAGNOSTIC IMAGING Chronic dyspnea of pulmonary origin Davide Stoppa

Chronic dyspnea of pulmonary origin Chronic: duration >1 month Dyspnea: breathlessness or shortness of breath Usually caused by cardiopulmonary disease myocardial infarction congestive heart failure Distinction between cardiac and pulmonary causes not always obvious Multifactorial etiology in up to one-third of patients Pulmonary origin - most common causes: Chronic Obstructive Pulmonary Disease (COPD) Interstitial Lung Disease (ILD) Dyer DS, Mohammed TL, Kirsch J, et al. ACR Appropriateness Criteria(R) Chronic Dyspnea: Suspected Pulmonary Origin. J Thorac Imaging. 2013; 28(5):W64-W66.

DYSPNEA ... ?

DYSPNEA Anamnesis - Physical examination

DYSPNEA Anamnesis - Physical examination IMAGING?

Variant 1: any age First imaging approach? X-Ray CT without contrast CT with contrast CT without and with contrast MRI without contrast MRI without and with contrast FDG-PET/CT

Variant 1: any age First imaging approach? X-Ray CT without contrast CT with contrast CT without and with contrast MRI without contrast MRI without and with contrast FDG-PET/CT

ACR appropriateness Criteria® Dyer DS, Mohammed TL, Kirsch J, et al. ACR Appropriateness Criteria(R) Chronic Dyspnea: Suspected Pulmonary Origin. J Thorac Imaging. 2013; 28(5):W64-W66.

Chest X-Ray DYSPNEA IMAGING? Anamnesis - Physical examination negative? positive! Spirometry Pulmonary Cardiac negative Pleural effusion Pneumonitis Pneumothorax Pulmonary edema ILD Vascular pathologies COPD Chest wall deformity Right failure Left failure ABG Other PaO2 < 70 mmHg Mediastinum Great vessels IMAGING?

CT without and with contrast MRI without contrast Variant 2: any age, nonrevealing or nondiagnostic clinical, standard radiography, and laboratory studies Second imaging approach? CT without contrast CT with contrast CT without and with contrast MRI without contrast MRI without and with contrast FDG-PET/CT

CT without and with contrast MRI without contrast Variant 2: any age, nonrevealing or nondiagnostic clinical, standard radiography, and laboratory studies Second imaging approach? CT without contrast CT with contrast CT without and with contrast MRI without contrast MRI without and with contrast FDG-PET/CT

ACR appropriateness Criteria® Dyer DS, Mohammed TL, Kirsch J, et al. ACR Appropriateness Criteria(R) Chronic Dyspnea: Suspected Pulmonary Origin. J Thorac Imaging. 2013; 28(5):W64-W66.

Chest X-Ray Chest CT DYSPNEA Anamnesis - Physical examination positive! negative? Spirometry Pulmonary Cardiac negative Pleural effusion Pneumonitis Pneumothorax Pulmonary edema ILD Vascular pathologies COPD Chest wall deformity Right failure Left failure ABG Other PaO2 < 70 mmHg Mediastinum Great vessels Chest CT

Chronic dyspnea of pulmonary origin How do these Patients present? Let’s see some examples

COPD Represents a spectrum of obstructive airway diseases It includes two key components: chronic bronchitis / small airways disease emphysema http://radiopaedia.org/articles/chronic-obstructive-pulmonary-disease-1

COPD Most important risk factor: tobacco smoking!

COPD Some clinical cases…

COPD – Case 1 No patient information available… Known history of chronic bronchitis

COPD – Case 1

COPD – Case 1

COPD – Case 1 Chronic obstructive pulmonary disease with prevalent chronic bronchitis http://images.md/category/chronic-obstructive-pulmonary-disorder/

COPD – Case 2 Presentation: Patient Data: Chronic, worsening shortness of breath Patient Data: Age: 60 years Gender: Female Race: Caucasian

COPD – Case 2

Chronic obstructive pulmonary disease with marked hyperinflation COPD – Case 2 Chronic obstructive pulmonary disease with marked hyperinflation http://radiopaedia.org/cases/chronic-obstructive-pulmonary-disease-with-marked-hyperinflation

COPD – Case 3 Presentation: Patient Data: History of smoking Age: 55 Gender: Male Race: Hispanic/Latino

COPD – Case 3

Centrilobular pulmonary emphysema COPD – Case 3 Centrilobular pulmonary emphysema http://radiopaedia.org/cases/centrilobular-pulmonary-emphysema-1

COPD – Case 4 Presentation: Patient Data: Severe breathlessness and recurrent infections Patient Data: Age: 49 Gender: Male

COPD – Case 4

Pan-lobular emphysema due to alpha-1-antitrypsin deficiency COPD – Case 4 Pan-lobular emphysema due to alpha-1-antitrypsin deficiency http://radiopaedia.org/cases/pan-lobular-emphysema-due-to-alpha-1-antitrypsin-deficiency

COPD – Case 5 Presentation: Patient Data: 45 year history of smoking with shortness of breath Patient Data: Age: 60 Gender: Male Race: Hispanic/Latino

COPD – Case 5

Paraseptal emphysema and subpleural bullae COPD – Case 5 Paraseptal emphysema and subpleural bullae http://radiopaedia.org/cases/paraseptal-emphysema-and-subpleural-bullae

Different types of emphysema http://radiopaedia.org/cases/emphysema-diagrams

ILD More than 200 chronic lung disorders affecting the interstitium (the tissue between the air sacs of the lungs) by inflammation or scarring (fibrosis) Fibrosis causes lung tissue to permanently lose the ability to breathe and carry oxygen Air sacs, as well as the lung tissue between and surrounding the air sacs, and the lung capillaries, are destroyed by the formation of scar tissue. http://www.uchospitals.edu/specialties/pulmonary/interstitial-lung/faq.html

ILD – Large number of disorders… Idiopathic Major idiopathic interstitial pneumonias Idiopathic Pulmonary Fibrosis / Usual Interstitial Pneumonia (IPF / UIP) Idiopathic Nonspecific Interstitial Pneumonia (NSIP) Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD) Desquamative Interstitial Pneumonia (DIP) Cryptogenic Organising Pneumonia (COP) Acute Interstitial Pneumonitis (AIP) Rare idiopathic interstitial pneumonias Idiopathic Lymphoid Interstitial Pneumonia(LIP) Idiopathic PleuroParenchymal FibroElastosis (PPFE) Unclassifiable idiopathic interstitial pneumonias Other entities Combined Pulmonary Fibrosis and Emphysema (CPFE) Multisystem diseases Autoimmune and collagen vascular diseases Systemic lupus erythematosus Rheumatoid arthritis Progressive systemic sclerosis Dermatomyositis and polymyositis Anti-synthetase syndrome Ankylosing spondylitis Sjögren syndrome Wegener's granulomatosis Mixed connective tissue disease Sarcoidosis Langerhans cell histiocytosis Inherited diseases Neurofibromatosis Tuberose sclerosis (lymphangioleiomyomatosis) Amyloidosis External causes Inhaled substances Inorganic Silicosis Asbestosis Berylliosis Organic Hypersensitivity pneumonitis Drug induced Antibiotics Chemotherapeutic drugs Antiarrhythmic agents Statins Radiation induced Infections Atypical pneumonia Pneumocystis pneumonia (PCP) Tuberculosis Chlamydia trachomatis Respiratory Syncytial Virus Miscellaneous Pulmonary alveolar proteinosis Pulmonary eosinophilia Idiopathic pulmonary haemosiderosis (IPH) Malignancy Primary Lymphoma, other lymphoproliferative diseases Secondary Pulmonary metastases, lymphangitis carcinomatosis http://www.thoracic.org/statements/resources/interstitial-lung-disease/classification-of-IIPs.pdf http://en.wikipedia.org/wiki/Interstitial_lung_disease http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2585700/ http://radiopaedia.org/articles/interstitial-lung-disease

ILD Some other clinical cases…

ILD – Case 1 Presentation: Patient Data: Progressive shortness of breath Patient Data: Age: 50 Gender: Male Race: Hispanic/Latino

ILD – Case 1

ILD – Case 1

ILD – Case 1

Usual interstitial pneumonia (UIP) ILD – Case 1 Usual interstitial pneumonia (UIP) http://radiopaedia.org/cases/usual-interstitial-pneumonia-uip-2

UIP http://valium6.tistory.com/24

ILD – Case 2 Presentation: Dyspnea Patient Data: Age: 47 Gender: Male

ILD – Case 2

ILD – Case 2

Non-specific interstitial pneumonia (NSIP) ILD – Case 2 Non-specific interstitial pneumonia (NSIP) http://radiopaedia.org/cases/non-specific-interstitial-pneumonia

NSIP http://valium6.tistory.com/24

UIP vs NSIP http://valium6.tistory.com/24

UIP vs NSIP http://valium6.tistory.com/24

ILD – Case 3 Presentation: Patient Data: Consolidations (on X-Ray, non available) not responding to multiple courses of antibiotics Patient Data: Age: 33 Gender: Female Race: Caucasian

ILD – Case 3

Cryptogenic organising pneumonia ILD – Case 3 Cryptogenic organising pneumonia http://radiopaedia.org/cases/cryptogenic-organising-pneumonia

ILD – Case 4 Presentation: Patient Data: Shortness of breath Age: Adult Gender: Female

ILD – Case 4

Patient had clinically established scleroderma! ILD – Case 4 Patient had clinically established scleroderma!

Scleroderma with interstitial fibrosis ILD – Case 4 Scleroderma with interstitial fibrosis http://radiopaedia.org/cases/scleroderma-with-intertitial-fibrosis

ILD – Case 5 Presentation: Patient Data: Dyspnea and dry cough Age: 42 years old Gender: Male Race: Middle eastern

ILD – Case 5

ILD – Case 5

ILD – Case 5

ILD – Case 5 Sarcoidosis - stage II http://radiopaedia.org/cases/sarcoidosis-stage-ii-1

ILD – Case 6 Presentation: Patient Data: 1-month history of sore throat and 9-month history of worsening nonproductive cough; dyspnea only after prolonged coughing episodes Provisionally diagnosed with asthma 9 months earlier, but not response to fluticasone Admission of smoking one pack of cigarettes per day for the past 3 years Patient Data: Age: 21 Gender: Female

ILD – Case 6

ILD – Case 6

Pulmonary Langerhans Cell Histiocytosis ILD – Case 6 Pulmonary Langerhans Cell Histiocytosis http://pubs.rsna.org/doi/full/10.1148/rg.271065044

ILD – Case 7 Presentation: Patient Data: Increasing shortness of breath over several months Patient Data: Age: 32 Gender: Female

ILD – Case 7

ILD – Case 7

Lymphangiomyomatosis ILD – Case 7 Lymphangiomyomatosis http://radiopaedia.org/cases/lymphangiomyomatosis

ILD – Case 8 Presentation: Patient Data: Shortness of breath Age: 64 Gender: Male Race: Caucasian

ILD – Case 8

ILD – Case 8

ILD – Case 8

Patient has an occupational history of sand blasting! ILD – Case 8 Patient has an occupational history of sand blasting!

ILD – Case 8 Silicosis with acute findings of congestive heart failure and pulmonary alveolar edema http://radiopaedia.org/cases/silicosis-2

ILD – Case 9 Patient Data: Age: 80 years Gender: Male

ILD – Case 9

ILD – Case 9

ILD – Case 9

Patient was previously exposed to asbestos fibres! ILD – Case 9 Patient was previously exposed to asbestos fibres!

Asbestos related lung and pleural disease ILD – Case 9 Asbestos related lung and pleural disease http://radiopaedia.org/cases/asbestos-related-lung-and-pleural-disease

ILD – Case 10 Presentation: Patient Data: Hypoxia with lung infiltrates on CXR Steroid responsive: asthma? Patient Data: Age: 12 Gender: Female Race: Caucasian

ILD – Case 10

ILD – Case 10

Further history: patient slept with a pet parrot! ILD – Case 10 Further history: patient slept with a pet parrot!

Hypersensitivity pneumonitis ILD – Case 10 Hypersensitivity pneumonitis http://radiopaedia.org/cases/hypersensitivity-pneumonitis-1

ILD – Case 11 Patient data: Elderly age Cardiac arrhythmia

ILD – Case 11

ILD – Case 11

Patient was using Amiodarone! ILD – Case 11 Patient was using Amiodarone!

ILD – Case 11 Amiodarone lung http://radiopaedia.org/cases/amiodarone-lung-1

ILD – Case 12 Presentation: Patient Data: Shortness of breath of 4 days duration Patient Data: Age 35 Gender: Male

ILD – Case 12

ILD – Case 12

Pulmonary alveolar proteinosis ILD – Case 12 Pulmonary alveolar proteinosis http://radiopaedia.org/cases/pulmonary-alveolar-proteinosis

ILD – Case 13 Presentation: Patient Data: Acute exacerbation of asthma, requiring hospitalisation Patient Data: Age: 56 Gender: Female Race: Caucasian

ILD – Case 13

ILD – Case 13

Patient had blood eosinophilia! ILD – Case 13 Patient had blood eosinophilia!

Eosinophilic lung disease - chronic ILD – Case 13 Eosinophilic lung disease - chronic http://radiopaedia.org/cases/eosinophilic-lung-disease-chronic-1

ILD – Case 14 Presentation: Patient Data: Previous history of Age: 60 breast carcinoma non-Hodgkin lymphoma MALToma Patient Data: Age: 60 Gender: Female

ILD – Case 14

ILD – Case 14

Lymphangitis carcinomatosis ILD – Case 14 Lymphangitis carcinomatosis http://radiopaedia.org/cases/lymphangitis-carcinomatosis-1

Lymphangitis carcinomatosis

Take Home Points Patient with chronic dyspnea of pulmonary origin: what to do? 1° approach: X-Ray 2° approach: CT without contrast (HRCT) CT with contrast in selected cases Different pathologies may appear similar on X-ray and even on CT Importance of correlation to Patient data (don’t forget occupational anamnesis) Clinical presentation Laboratory studies

Thank You for Your Attention!