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1 Pulmonary Medicine Rosemary Adamson, MB BS Assistant Professor, Division of Pulmonary & Critical Care Medicine, UW Attending Physician, Pulmonary & Critical Care, Seattle VA

2 I have no financial disclosures

3 5. Nodules & screening 4. Pulmonary hypertension 3. Restrictive lung diseases 2. Obstructive lung diseases 1. Cough

4 Cough Number 1 reason for PCP visit Acute: <3 weeks Chronic: >8 weeks

5 Cough: Acute bronchitis Cough of less than 3 weeks duration Antibiotics may shorten duration of symptoms BUT Increase adverse events AVOID antibiotic prescription when possible

6 Strategies to avoid prescribing antibiotics for acute bronchitis Prescribe DELAYED antibiotics Recommend symptomatic treatments Recommend returning if not improving 80% patients satisfied with delayed or no antibiotics Spurling et al. Cochrane Database Syst Rev. 2013

7 Cough: Gastroesophageal reflux PPIs suppress acid production but not reflux Lifestyle modifications for GERD: Avoid food for 3 hours before bed Elevate the head of the bed Decrease caffeine, spicey food, mint, alcohol

8 Cough: Upper Airway Syndrome Inhaled nasal steroid Antihistamine Nasal irrigation Really important in all lung diseases

9 Avoid antibiotics for acute bronchitis Recommend symptomatic treatment Recommend returning if not better Consider prescribing delayed antibiotics GERD-induced cough can persist on PPI Upper airway syndrome can require aggressive management 1. Cough

10 5. Nodules & screening 4. Pulmonary hypertension 3. Restrictive lung diseases 2. Obstructive lung diseases 1. Cough

11 Asthma

12 Asthma: outpatient management Short-acting β-agonist Inhaled corticosteroid Review triggers Long-acting β-agonist Discuss lifestyle changes Develop asthma action plan Consider leukotriene receptor antagonist Consider tiotropium, Check inhaler technique Oral corticosteroids, Consider allergy testing Referral to allergy or pulmonary

13 Asthma: action plans Download from NIH.gov. Complete!

14 Asthma: management, additional therapies Omalizumab Moderate-severe persistent asthma with Incomplete control with ICS IgE ve skin or specific IgE test to perennial allergen Dosed by weight & IgE level Subcutaneous injection /2-4wks Mepolizumab Severe asthma with Eosinophilia > 150/microL Subcutaneous injection /4wks Immunotherapy +ve skin or specific IgE test to allergens Subcutaneous injection or sublingual tablets

15 Asthma: management of exacerbations Oral steroids as good as IV Consider magnesium sulfate Early recognition is key! Non-invasive ventilation use increasing Permissive hypercapnia if intubated

16 Increasing exacerbations COPD: evaluation of stable outpatients Increasing Check spirometry for reduced FEV1/FVC ratio symptoms C D A B Vogelmeier et al. Global strategy for the diagnosis, management and prevention of COPD 2017 report. AJRCCM 2017;195:

17 Modified Medical Research Council questionnaire Grade Dyspnea occurs 0 Only with strenuous activity 1 Walking up slight hill or hurrying on flat 2 Walk slower than people of same age, or have to stop, on level ground 3 And have to stop after few minutes or ~100m 4 Performing ADLs; cannot leave house

18 COPD Assessment Tool Rate on scale of 0 (good) to 5 (bad) each of the following: Cough Phlegm Chest tightness Confidence leaving home Dyspnea walking uphill or 1 flight of stairs Limitation to activities in the home Sleep quality Energy level

19 COPD management?+ roflumilast SABA LABA or LAMA LABA + LAMA LABA + LAMA + ICS SABA: short-acting β-agonist LABA: long-acting β-agonist LAMA: long-acting muscarinic agonist ICS: inhaled corticosteroid?+ azithromycin Vogelmeier et al. Global strategy for the diagnosis, management and prevention of COPD 2017 report. AJRCCM 2017;195:

20 COPD management Vaccinations Smoking cessation! Check alpha-1- antitrypsin Pulmonary rehabilitation Opioids & palliative care Consider surgery Vogelmeier et al. Global strategy for the diagnosis, management and prevention of COPD 2017 report. AJRCCM 2017;195:

21 COPD management: recent updates Home oxygen only for severe resting hypoxemia Nocturnal BiLevel may benefit those with hypercapnia Vogelmeier et al. Global strategy for the diagnosis, management and prevention of COPD 2017 report. AJRCCM 2017;195:

22 COPD: management of acute exacerbations 5-7 days of steroids is sufficient Antibiotics not necessary if no sputum purulence Noninvasive ventilation improves outcomes Vogelmeier et al. Global strategy for the diagnosis, management and prevention of COPD 2017 report. AJRCCM 2017;195:

23 You evaluated a 54yo man with a chronic productive cough. CXR showed right lower lobe bronchiectasis so you referred him to pulmonary. He returns to review the recommendations from the pulmonologist with you. Assessment: 54M with focal bronchiectasis due to remote severe pneumonia. Plan: - Airway clearance twice daily with nebulized albuterol, then flutter device - Antibiotics for exacerbations according to sputum culture data

24 Bronchiectasis: Abnormal, permanent dilatation of the airways Mucus plugs distend airways Impaired mucociliary clearance BRONCHIECTASIS & mucus hypersecretion Fibrosis pulls on airways Bacterial biofilms

25 Bronchiectasis: pathology

26 Copious sputum production

27 Bronchiectasis: evaluation Causes of bronchiectasis: Chest CT & pulmonary referral Non-tuberculous mycobacterial infection Cystic fibrosis Alpha-1-antitrypsin deficiency Immunoglobulin deficiency Primary ciliary dyskinesia Aspiration Collagen vascular diseases

28 Bronchiectasis: management Airway clearance techniques Mucus-mobilization Anti-inflammatories Aspiration reduction Antimicrobials

29 Asthma: make action plans COPD: avoid ICS when possible Bronchiectasis: airway clearance is key 2. Obstructive lung diseases

30 5. Nodules & screening 4. Pulmonary hypertension 3. Restrictive lung diseases 2. Obstructive lung diseases 1. Cough

31 Asbestosis Diffuse parenchymal lung disease due to years of exposure to asbestos from: Mining for asbestos or vermiculite (Libby, MT), installation or removal of asbestos insulation (ships, pipes, boilers), popcorn ceilings, demolition work, vehicle maintenance (brake pads & clutch) Latency >30yrs Basal predominant interstitial fibrosis Diagnosis of exclusion

32 Diffuse Parenchymal Lung Disease Known cause or association Unknown cause Systemic disease Exposures Genetic Idiopathic Interstitial Pneumonias Other pathologies Connective tissue disease IBD Sarcoidosis Pneumoconioses Hypersensitivity pneumonitis Iatrogenic Familial IPF Tuberous sclerosis IPF NSIP DIP RB-ILD LIP COP LAM LCH PAP Eosinophilic pneumonias

33 Disease Associated occupations Exposure duration Asbestosis Mining, vehicle repair, ship-building, boiler/ pipe fitting, demolition Latency Histopathology Imaging findings >10yrs >30yrs Fibrosis Lower lobe predominant Possible pleural plaques Special points Silicosis Coal-workers pneumoconiosis Mining, sandblasting, glassmaking, ceramics, brick foundries years 20-30yrs Nodules Upper lobe eggshell calcification of hilar LNs PMF (progressive massive fibrosis) possible Coal mining Nodules Upper lobe PMF possible Berylliosis Electronics, manufacturing, ceramics, old fluorescent lightbulbs >2yrs Chronic interstitial pneumonitis Upper lobe Hilar LAD Beryllium lymphocyte transformation test. Rx: steroids

34 Diffuse Parenchymal Lung Disease Known cause or association Unknown cause Systemic disease Exposures Genetic Idiopathic Interstitial Pneumonias Other pathologies Connective tissue disease IBD Sarcoidosis Pneumoconioses Hypersensitivity pneumonitis Iatrogenic Familial IPF Tuberous sclerosis IPF NSIP DIP RB-ILD LIP COP LAM LCH PAP Eosinophilic pneumonias

35 Sarcoidosis Multisystem, inflammatory disorder Characterized by non-caseating granulomas Lungs involved in 90% of cases Epidemiology: Female predominance Highest incidence in US & Sweden More common in African-Americans than Caucasian- Americans BUT rare in people from Africa

36 Sarcoidosis: clinical features Iannuzzi M et al. N Engl J Med 2007;357:

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38 Sarcoidosis: Diagnosis Clinical diagnosis for certain syndromes E.g. Lofgren s syndrome Typically need biopsy Non-caseating granulomas Iannuzzi M et al. N Engl J Med 2007;357:

39 Sarcoidosis: Management Assess severity Evaluate critical systems (eyes, heart, calcium level) Monitor for progression Consider steroids Ask about syncope at every appointment

40 Diffuse Parenchymal Lung Disease Known cause or association Unknown cause Systemic disease Exposures Genetic Idiopathic Interstitial Pneumonias Other pathologies Connective tissue disease IBD Sarcoidosis Pneumoconioses Hypersensitivity pneumonitis Iatrogenic Familial IPF Tuberous sclerosis IPF NSIP DIP RB-ILD LIP COP LAM LCH PAP Eosinophilic pneumonias

41 DPLD: Idiopathic Pulmonary Fibrosis DPLD characterized by findings of Usual Interstitial Pneumonia Natural history: Slowly progressive Median survival 2-3yrs from diagnosis Risk factors: Prevalence & incidence increase with age More common in men Association with gastroesophageal reflux

42 IPF: diagnosis Exclude other causes Characteristic chest CT Characteristic histopathology Biopsy not always necessary Radiographic & histopathologic patterns termed Usual Interstitial Pneumonia

43 IPF: management Supportive care: Vaccinations Pulmonary rehabilitation GERD reduction Oxygen Consider lung transplant Palliative care Specific therapies: Pirfenidone (anti-fibrotic) Nintedanib (TK-inhibitor) Slow rate of decline in PFTs Multiple drug interactions Side effects: GI, rash, hepatotoxicity

44 Restrictive Lung disease: pathophysiology Reduced TLC Increased lung recoil Decreased chest wall recoil Diffuse Parenchymal Lung Disease Chest wall abnormality Neuromuscular weakness

45 Neuromuscular Lung Disease: causes Nervous System Phrenic nerve injury Guillain-Barré syndrome Poliomyelitis Amyotrophic lateral sclerosis Spinal cord injury Neuromuscular Junction Myasthenia gravis Muscle Post-polio syndrome Muscular dystrophy

46 Neuromuscular Lung Disease: clinical features & management Weak cough, recurrent pneumonias Respiratory failure Pulmonary function testing used to assess severity: MIP = Maximum Inspiratory Pressure bedside MIP = NIF = Negative Inspiratory Force MEP = Maximum Expiratory Pressure Cough assist may be required Ventilatory support may be required First at night Then daytime as well

47 3. Restrictive lung diseases Pulmonary restriction is Diagnosed by lung volumes Due to parenchymal or chest wall pathology Evaluation involves either High-resolution chest CT or Neuromuscular strength evaluation For patients with DPLD on chest CT Look for cause or association

48 5. Nodules & screening 4. Pulmonary hypertension 3. Restrictive lung diseases 2. Obstructive lung diseases 1. Cough

49 Pulmonary Hypertension: pathophysiology Pulmonary Vascular Resistance Right Ventricle Left heart overload Left Atrium

50 Pulmonary Hypertension: WHO classification Group 1: pulmonary arterial hypertension Group 2: due to left heart disease Group 3: due to lung disease or hypoxemia Group 4: due to chronic thromboembolic disease Group 5: unclear multifactorial mechanisms

51 Pulmonary hypertension: WHO functional classes Class Description 1 No limitation to activity 2 Ordinary activity causes some symptoms 3 Less than ordinary activity causes symptoms 4 Any activity causes symptoms Syncope

52 Pulmonary Hypertension: management Oxygen Coumadin Calcium Channel Blockers PDE5 Inhibitors Endothelin-Receptor antagonists Prostacyclin Analogues Right Ventricle Diuresis Digoxin Exercise Diuresis Left Atrium

53 4. Pulmonary hypertension PFTs with isolated DLCO reduction are concerning for pulmonary hypertension Pulmonary hypertension should be Screened for with TTE Diagnosed by right heart catheterization Left heart failure is most common cause in US Syncope indicates decompensation

54 5. Nodules & screening 4. Pulmonary hypertension 3. Restrictive lung diseases 2. Obstructive lung diseases 1. Cough

55 Pulmonary nodules: talking to patients Give them a leaflet Show them the size Encourage smoking cessation

56 Pulmonary nodule management: Fleischner guidelines Refer to: Incidentally discovered nodules in patients who are Older than 35 Immunocompentent Without cancer 2017 update: NO follow-up for solid nodules <6mm FIVE years follow-up for sub-solid nodules

57 Lung cancer screening Give them a leaflet Refer to program if possible Encourage smoking cessation Discuss risks

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61 Benefit of quitting smoking is greater than screening

62 Lung cancer screening USPSTF recommends: Annual low-dose chest CT for 55-80year olds with >30 pack-year smoking history who have quit for <15 years

63 Lung nodules Are very common Need to be explained to patients Lung cancer screening Significant pre-screening counselling Encourage smoking cessation 5. Nodules & screening

64 5. Nodules & screening 4. Pulmonary hypertension 3. Restrictive lung diseases 2. Obstructive lung diseases 1. Cough

65 Pulmonary medicine: final take-home points Upper airway syndrome & GERD are common exacerbate all lung diseases may not be obvious Everyone needs to QUIT smoking!

66 Thank you & any questions? Rosemary Adamson, MB BS