Approach to Dyspnea

1. Approach to Dyspnea Mani S. Kavuru, MD Professor & Division Chief Pulmonary & Critical Care Medicine Thomas Jefferson University / Hospital

2. Key learning Objectives • Familiarize with eliciting history relevant to dyspnea & scales utilized; • Be able to define a diagnostic approach to dyspnea, with emphasis in the outpatient area; • Develop facility with common pulmonary diagnostic modalities from PFTs, exercise studies, imaging, and biopsy • Apply these concepts in case-based scenarios

3. ATS Definition of Dyspnea • Patient self-reported, subjective • “Breathing discomfort, qualitatively distinct sensations varying in intensity” • Arises from “interactions among multiple physiological, psychological, social, and environmental factors and may induce secondary psychological and behavioral responses” • Prefer “breathlessness” as patient-centric

4. Dyspnea • Subjective • Discomfort associated with the act of breathing in circumstances it is unexpected; • Further characterize by: ◊nature of onset (acute, chronic), duration ◊evolution over time ◊associated symptoms (cough, CP, wheeze, orthopnea) ◊physiologic vs. pathologic ◊quantify (“no SOB” is inadequate)

5. Caveats re. Dyspnea Evaluation • Note discrepancy between patient’s perception (under-reported) and physician’s clinical evaluation (under-recognized); • Be able to recognize fatigue vs. activity intolerance vs. dyspnea; • Be aware of natural history of dyspnea in various clinical settings and disease entities; • Co-existence of multiple causes of dyspnea is common; so need to be able to define relative contribution of lung disease (as opposed to obesity, anemia, LBP); • Be adept at distiguishing cardiac vs. pulmonary causes of SOB; • FEV1 as a surrogate marker can be misleading; • PFTs and exercise studies do not assess severity of feeling of breathlessness (a sensation); but rather impact on functional capacity or physiological consequences; • Don’t be fooled by “normal O2 sat’n”;

6. Pathogenesis of Dyspnea • Dynamic hyperinflation • Increased ventilatory demand relative to capacity • Abnormalities in gas exchange • Inspiratory muscle weakness • Cognitive & psychological influences (i.e. fear, anxiety) • Other

9. The “Oxygen Cost” Diagram “What can you do before you become short of breath?” Patient makes a mark on a 10cm line at the point beyond which they become breathless The oxygen cost diagram is more sensitive to change than the MRC scale - it consists of a 10cm line with everyday activities placed proportionately according to their oxygen cost. Patients place a mark on a 10cm line, beyond which they become breathless. The ability score is the distance in centimetres from the zero point. Reference McGavin CR, Artvinli M, Naoe H, McHardy GJR. Dyspnoea, disability and distance walked: comparison of estimates of exercise performance in respiratory disease. Br Med J 1978; 2: 241–243

13. Bausewein. Respiratory Med 2007

27. A 62 y/o WM smoker (200+ pk-yr) with progressive dyspnea and activity intolerance over past 6 mos; Exam: markedly reduced BSs with prolonged expiration, distant heart tones, 1+ edema; CXR is clear; spiro shows FEV1 to be 30%, FVC 50%, ratio 0.32; RA PaO2 is 78; • A 45 y/o with hx of pred-dependent asthma since childhood; is a smoker; has gained 100+ lbs; several prior admits for resp failure; now presents to clinic with worsened SOB, wheezing; Exam: verbal, no distress, audible wheezing, morbidly obese; RA O2 sat = 98%; • A 75 y/o non-smoker presents with

28. Clinical Evaluation for Lung Disease • PFTs: spirometry (screening/simple), lung volumes, DLCO, O2 assessment; [assess for copd, asthma, UAO, ILD] • Exercise assessment (6MWT, GXT) [assess functional status of any cardiopulm disease] • Bronchoprovocation challenge [assess for asthma] • Serial chest radiographs, CTA / HRCT chest [assess for ILD, cancer, CHF, HP, other] • Targeted Labs: cbc, chems, HPP, CVD, other [assess for anemia, CVD, HP, sarcoid, vasculitis] • Bronchoscopy (BAL, TBBx, EBUS) [assess for any parenchymal lung disease that produces infiltrates on CXR/CT] • Surgical lung biopsy (VATS, mede) [assess for any parenchymal lung disease that produces infiltrates on CXR/CT]

30. “Normal” CXR/CT Asthma COPD & emphysema Occupational “asthma-like” syndromes Cardiac causes Upper airway disease Pulmonary vascular disease Neuromuscular disease Other (anemia, obesity, deconditioning) Abnormal CXR/CT Acute pneumonia syndromes, alveolitis Chronic fibrotic diseases (IPF, CVD, sarcoid) Pneumoconioses Malignancy Cardiac disease PE Other Spectrum of Dyspnea & Respiratory Syndromes

31. Clinical vignette #2 (Q4) a. Empiric therapy with antibiotics b. 24 hour pH monitoring c. Exercise and/or cold air challenge d. Spirometry, if airway obstruction is present, proceed with methacholine challenge test e. Spirometry, if normal, proceed with methacholine provocation test A 45 y/o non-smoker presents with episodic dyspnea and cough of 4 months’ duration. She denies nasal drainage, wheezing or heartburn. A chest x-ray is normal. Which of the following is the next best step?

32. Clinical vignette #3 (Q8) 60 y/o non-smoker with progressive dyspnea over 8 mos; hx is remarkable for prior thyroid cancer, s/p 2 surgeries, XRT to neck. W/up shows normal ABGs, spirometry, lung volumes/DLCO, HRCT chest, V/Q scan. Which is next best test to establish a diagnosis? • Open lung biopsy • Flow volume loop • 100% shunt study • 2D echocardiogram with bubbles • Methacholine provocation study

33. Clinical Vignette #4 (Q9) 50 y/o male ex-smoker (20 pk-yrs) with DOE, daily cough +/- sputum, activity intolerance, wheezing; Exam: reduced breath sounds; CXR: hyperinflated, clear; spiro: severe reduction in FEV1 with no BD response and reduced DLCO; several family members died prematurely. Which of the following is the best approach? Blood test to prove diagnosis, then optimize inhalers, home O2, and start intermittent monthly infusions Proceed to lung transplantation Bronchoscopy with BAL, biopsy Liver biopsy Serum cotinine levels

34. Clinical vignette #5 (Q11) • 66 y/o WF smoker with 6-12 mos hx of progressive dyspnea, cough, activity intolerance. Exam shows clubbing of fingers, bibasilar crackles, trace edema. PFTs show reduction in DLCO and volumes with normal ratio and flows; RA PaO2 55; CXR is diffusely abnormal. What is the most likely abnormal compartment? • Upper airways • Pulmonary vasculature • Small airways, tethering structures • Interstitium • “Bellows” or resp muscles/nerves

36. Vignettes, Questions • Clinical vig #1 (Q 1-3) • Clinical vig #2 (Q4-7) • Clinical vig #3 (Q8) • Clinical vig #4 (Q9-10) • Clinical vig #5 (Q11) • Clinical vignette #6 (Q12)

37. Typical Symptoms Dyspnea Cough +/- sputum Activity intolerance Fatigue Chest pain Wheezing Note: pattern of sx History Temporal aspects acute or chronic Assoc systemic sx Response to therapies Family history IPF, CTD Exposures Sick contacts Tobacco Occupational Hobbies Pets Drugs Radiation Initial Approach to Respiratory Disease

38. Classification of Lung Diseases • Obstructive Disease: asthma; chronic bronchitis; emphysema; CF; • Restriction--Intra-parenchymal disease (lung tissue is abnormal, e.g. HP, pulmonary fibrosis) • Restriction--Extra-parenchymal disease (lung tissue is normal); chest wall deformities, kyphosis, scoliosis, obesity, pleural effusions, ascites • Neuromuscular disorders (“bellows”)

39. Clinical Vignette #1 22 y/o woman non-smoker with episodic chest tightness, wheezing, SOB, coughing with exposure to cold air, exercise FMHx: allergies, eczema, asthma Exam: tachypnea; bilat wheezes CXR: clear Spirometry: reduced flows (i.e. FEV1)

40. Question 1 a. There has been a recent upward trend b. Death occurs frequently c. Excessive use of -agonists has been implicated d. Subgroups of patients are at greatest risk e. Inadequate use of certain medications have been implicated Which of the statements regarding this disease is incorrect?

41. Question 2 a. Airway hyperreactivity b. Severe hypoxemia c. Obstructive impairment with reversibility d. Mild increase in diffusing capacity (DLCO) e. Airway inflammation All of the following features are typical for bronchial asthma except:

43. Spirometry • Two main measurements: • total volume exhaled (FVC) • lung/thorax expansion • HPP, IPF - restrictive lung diseases • volume exhaled in 1st second of exhalation (FEV1) • airway diameter • obstructive lung diseases • asthma, emphysema, chronic bronchitis, etc.

45. Question 3 What is the single best maintenance therapy for chronic moderate asthma? Prednisone Albuterol or epinephrine Inhaled steroids Cromoglycates Leukotriene blockers

47. Question 5 a. Send home with -MDI q 4 hours b. Send home with inhaled steroids and PRN - MDI c. Begin IV aminophylline, admit for observation d. Administer p.o. steroid in ED, discharge home with steroid taper over two weeks and PRN -MDI e. Assess ABGs and CXR prior to further treatment A 20 y/o with a history of episodic asthma presents to the emergency department (ED) with dyspnea, wheezing, and chest tightness of several days duration. A PEFR was 200 1/min. Inhaled -agonists were administered 30 minutes apart x 3. She became asymptomatic. Which of the following is the next best step?

48. Question 6 a. Misdiagnosis of asthma b. Presence of a co-morbid disease (i.e., sinusitis, GERD, etc.) c. Steroid receptor polymorphism d. Patient non-compliance with medications e. Poor control of environmental triggers Reversible factors that may contribute to "steroid-dependent" asthma include all of the following except:

49. Question 7 a. Establish the correct diagnosis (i.e., obtain a methacholine provocation test) b. Carefully quiz the patient / family about compliance issues c. Instruct regarding proper MDI technique, spacer device, home PEFR monitoring d. Add inhaled corticosteroids e. Check theo level and optimize the dose A 30 y/o non-smoker presents to the clinic with a 3 year history of episodic cough, chest tightness, and wheezing. She meticulously uses a -MDI at least 2 puffs every 6 hours with good relief of symptoms. She takes several additional puffs at night to help with sleep. A month ago, after an acute URI, she required ER care. She was very aggressively treated and discharged home on nebulized albuterol, atrovent, humibid, and theo/albuterol tablets. What is the single most important intervention now?

50. Asthma "Mimics": Differential Diagnosis Overlapping airway disorders Chronic bronchitis and emphysema (COPD) Cystic fibrosis Bronchiectatic syndromes Anatomical airway obstruction Foreign bodies Laryngospasm, edema Vocal chord paralysis Laryngotracheobronchomalacia Benign / malignant endobronchial tumors Other conditions associated with wheezing Congestive heart failure ("cardiac asthma") Pulmonary embolism Aspiration (gastroesophageal reflux) Loffler's syndrome Factitious asthma (vocal chord dysfunction)