Diagnosing Idiopathic Pulmonary Fibrosis on Evidence-Based Guidelines

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2 Diagnosing Idiopathic Pulmonary Fibrosis on Evidence-Based Guidelines Rebecca Keith, MD Assistant Professor, Division of Pulmonary and Critical Care Medicine National Jewish Health, Denver, CO

3 Objectives Develop a differential diagnosis for dyspnea Describe the clinical presentation of IPF Describe the best practices for diagnosing IPF based on the most recent evidence-based guidelines Understand the disease course

4 Case Study: Jerry 67-year-old man Progressive shortness of breath for 3 years Initially, underwent a cardiac evaluation and was found to have critical aortic stenosis Underwent valve replacement Dyspnea improved, but six months later had worsening breathlessness Recent cardiac evaluation normal Non-productive cough for the past 2 years Never-smoker

5 Differential Slide Title Diagnosis for Chronic, Non-Infectious Dyspnea Upper Airways Airway Obstruction Tracheomalacia Lower Airways/Lungs Pleural Effusion Parenchymal Tumors Endobronchial Tumors Asthma/COPD ILD Cardiovascular System Ischemic Heart Disease CHF Valvular Disease Arrhythmias Pulmonary Hypertension Neuromuscular System Amyotrophic lateral sclerosis Multiple sclerosis Myasthenia Gravis Guillain-Barre Metabolic, Renal, Hepatic & Heme Hypo/Hyperthyroidism Cushing s Syndrome Obesity Renal failure/acidosis Psychogenic Disorders Hyperventilation Syndrome Anxiety/Panic Disorders Depression Somatization Pratter MR, at al. Arch Intern Med. 1989;149:2277.

6 Differential Slide Title Diagnosis for Chronic, Non-Infectious Dyspnea One study found that 66% patients presenting to a pulmonologist with chronic dyspnea had one of the following; asthma, COPD, ILD or a cardiomyopathy. Upper Airways Airway Obstruction Tracheomalacia Lower Airways/Lungs Pleural Effusion Parenchymal Tumors Endobronchial Tumors Asthma/COPD ILD Cardiovascular System Ischemic Heart Disease CHF Valvular Disease Arrhythmias Pulmonary Hypertension Neuromuscular System Amyotrophic lateral sclerosis Multiple sclerosis Myasthenia Gravis Guillain-Barre Metabolic, Renal, Hepatic & Heme Hypo/Hyperthyroidism Cushing s Syndrome Obesity Renal failure/acidosis Psychogenic Disorders Hyperventilation Syndrome Anxiety/Panic Disorders Depression Somatization Pratter MR, at al. Arch Intern Med. 1989;149:2277.

7 Interstitial Lung Disease One of the difficulties with understanding this group of disorders is the confusing terminology

8 What is Interstitial Lung Disease? Diverse group of disorders (>150) that result inflammation/scarring of the pulmonary parenchyma Similar presentation Progressive dyspnea and dry cough Abnormal pulmonary physiology Abnormal CXR and/or HRCT Multiple Etiologies Idiopathic Systemic diseases (connective tissue disorders) Drug, environmental, or occupational exposures Genetic

9 Where is The Interstitium? The loose-binding connective tissue (peribronchovascular sheaths, interlobular septa, visceral pleura) The parenchymal interstitium (alveolar wall or alveolar septae) This space is lined by alveolar epithelial and capillary endothelial cells This is the largest component Schwarz MI, et al. Interstitial Lung Disease; 4 th ed., 2003.

10 The Interstitium The Largest Component

11 Interstitial Lung Disease Inflammation Cellular non-specific interstitial lung disease (NSIP)

12 Interstitial Lung Disease Combination of inflammation and fibrosis Mixed non-specific interstitial fibrosis (NSIP)

13 Interstitial Lung Disease Fibrosis Usual Interstitial Pneumonia (UIP) Cool CD et al. Am J Respir Crit Care 2006;174:

14 Interstitial Lung Disease

15 Interstitial Lung Disease

16 Idiopathic Slide Title Interstitial Pneumonias: No Identifiable Cause Major IIP Idiopathic Pulmonary Fibrosis (IPF) Idiopathic Nonspecific Interstitial Pneumonia (NSIP) Respiratory Bronchiolitis-ILD (RB-ILD)* Desquamative Interstitial Pneumonia (DIP)* Cryptogenic Organizing Pneumonia (COP) Acute Interstitial Pneumonia (AIP) Rare IIP Idiopathic lymphoid interstitial pneumonia (LIP) Idiopathic pleuroparenchymal fibroelastosis (IPPFE) Unclassifiable IIP Travis WD, et al. Am J Respir Crit Care Med 2013;188:

17 Idiopathic Pulmonary Fibrosis Chronic, fibrosing interstitial pneumonia of unknown etiology Incidence = 3 to 9/100,000; or up to 90,000 new cases per year in the US Of the idiopathic ILDs, IPF accounts for ~50% Of the idiopathic ILDs, worst prognosis Median survival 2.5 to 3.5 years from diagnosis

18 Idiopathic Pulmonary Fibrosis Presentation Patients are typically > 50 years old Men > Women Many with a smoking history; smoking is a risk factor Exertional dyspnea +/- Cough +/- Fatigue NO extrapulmonary features

19 Diagnostic Slide Title Algorithm For Patients With Suspected IPF Reprinted with permission of the American Thoracic Society. Copyright 2017 American Thoracic Society. Raghu G, et al./2011/an Official ATS/ERS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management/Am J Respir Crit Care Med/183/ The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

20 Current Definition of IPF Exclusion of other known causes of ILD Presence of UIP pattern on HRCT imaging in those patients without a surgical lung biopsy ATS/ERS/JRS/ALAT Statement. Am J Respir Crit Care Med. 2011;183:

21 Diagnostic Slide Title Algorithm For Patients With Suspected IPF Reprinted with permission of the American Thoracic Society. Copyright 2017 American Thoracic Society. Raghu G, et al./2011/an Official ATS/ERS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management/Am J Respir Crit Care Med/183/ The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

22 Interstitial Lung Disease HRCT Patterns UIP Pattern Possible Inconsistent Subpleural, basal predominance Subpleural, basal predominance Upper or mid-lung predominance Reticular abnormality Reticular abnormality Peribronchovascular predominance Honeycombing with or without traction bronchiectasis Absence of features that are inconsistent NO HONEYCOMBING Absence of features that are inconsistent Ground glass > Reticular Micronodules Cysts Mosaic attenuation/airtrapping Consolidation

23 UIP or Possible UIP?

24 UIP or Possible UIP?

25 Is This IPF?

26 Diagnostic Slide Title Algorithm for Patients With Suspected IPF I don t know. I don t have enough information. Reprinted with permission of the American Thoracic Society. Copyright 2017 American Thoracic Society. Raghu G, et al./2011/an Official ATS/ERS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management/Am J Respir Crit Care Med/183/ The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

27 Making Slide Title The Diagnosis In ILD, Requires That You Become A Detective History Physical Exam Pulmonary physiology Radiography +/- Surgical lung biopsy

28 History Typical presentation for IPF Age > 50 Dyspnea subacute, insidious onset I first noticed I was breathless playing with my grandchildren 2 years ago, but I thought I was just out of shape. +/- Dry cough +/- Fatigue/Low stamina NOT pain, weakness, swollen joints, rash, or other systemic symptoms

29 History The Focus Of The History Taking Is To Identify Features That Suggest This Is NOT IPF Rheumatologic Illness Eyes Mouth Skin Joints Muscles Esophagus Raynaud s Exposures Birds Mold in the home

30 When To Suspect ILD? If a patient has exertional dyspnea and/or chronic cough Perform a physical examination, listen carefully for basilar crackles Examine the hands and joints for evidence of autoimmune disease and/or clubbing Order PFTs, restrictive pattern Order a chest radiograph Perform an informal hallway walk test; 3% is abnormal If any of these suggest ILD, order a High Resolution CT

31 Let s Get Back to Jerry History of Present Illness 67-year-old man Progressive shortness of breath for 3 years Initially, underwent a cardiac evaluation and was found to have critical aortic stenosis Underwent valve replacement Dyspnea improved, but six months later had worsening exertional breathlessness. Recent cardiac evaluation normal Non-productive cough for the past 2 years

32 Let s Get Back to Jerry Medications Metoprolol Famotidine prn He has no other medical/surgical history He has no history of smoking He has no history of lung problems in the family. He has no exposures, and no autoimmune concerns

33 Let s Get Back to Jerry VS: BP 112/78, HR 68, RR 16, Oxygen Saturation on Room Air 93% (Altitude 5280) Gen: Healthy appearing male Pulm: Late bi-basilar crackles Card: RRR, no m/r/g Ext: Query early clubbing, no other joint abnormalities Skin: No rashes Neuro: Normal strength

34 What Testing Should We Order? 1. PFT s 2. Walk test to assess for hypoxemia 3. HRCT 4. All of the above 5. Only 1 & 2

35 What Testing Should We Order? 1. PFT s 2. Walk test to assess for hypoxemia 3. HRCT 4. All of the above 5. Only 1 & 2

36 Findings From Jerry s Initial Evaluation PFTs TLC = 75% of predicted FVC = 78% of predicted FEV1 = 81% of predicted Ratio = 90 Diffusion ins 69% of predicted Oxygen titration study His oxygen falls from 93 to 87% at six-minutes

37 Findings From Jerry s Initial Evaluation HRCT report Moderately extensive, basilar predominant reticulation, with honeycombing and traction bronchiectasis Consistent with a UIP pattern

38 At This Point What Would You Do? 1. You diagnose Jerry with IPF 2. You order autoimmune serologies to assess for occult connective tissue disease 3. You send Jerry for a surgical lung biopsy 4. You order precipitans looking for an exposure that may lead to chronic hypersensitivity pneumonitis

39 At This Point What Would You Do? 1. You diagnose Jerry with IPF 2. You order autoimmune serologies to assess for occult connective tissue disease 3. You send Jerry for a surgical lung biopsy 4. You order precipitans looking for an exposure that may lead to chronic hypersensitivity pneumonitis

40 Jerry s Diagnosis His autoimmune panel was negative We typically do not order precipitans Given his UIP pattern, he does not need a surgical lung biopsy Jerry was diagnosed with IPF

41 Extremely Slide Title Difficult To Predict An Individual s Disease Course Reprinted from The Lancet, 378, King TE Jr, et al., Idiopathic Pulmonary Fibrosis, , Copyright 2011, with permission from Elsevier.

42 What Slide Will Title Jerry s Disease Course Look Like? 1. Slow, progression loss in lung function 2. Rapidly accelerated disease 3. Prolonged (> 5 years of stability) 4. Death due to an acute exacerbation within the next year 5. Any of the above

43 What Slide Will Title Jerry s Disease Course Look Like? 1. Slow, progression loss in lung function 2. Rapidly accelerated disease 3. Prolonged (> 5 years of stability) 4. Death due to an acute exacerbation within the next year 5. Any of the above

44 Summary There is a large differential diagnosis for dyspnea Of patients referred to a pulmonologist, the most common diagnoses are asthma, COPD, cardiomyopathy, and ILD IPF Presents in older patients with exertional breathlessness +/- cough No extrapulmonary manifestations If you have no identifiable cause and a UIP pattern on HRCT = IPF If you have no identifiable cause and a possible UIP pattern (no honeycombing), consider surgical lung biopsy The disease course is highly variable

45 For additional resources and IPF patient education materials, please visit