Guideline sources The following summarized guidelines for the evaluation and management of chronic obstructive pulmonary disease are prepared by our editorial team based on guidelines from the Global Initiative for Chronic Obstructive Lung Disease (GOLD 2023), the United States Department of Defense (DoD/VA 2021), the American Association for Thoracic Surgery (AATS 2021), the British Thoracic Society (BTS 2020), the American Thoracic Society (ATS 2020), the Canadian Thoracic Society (CTS 2019; 2012; 2011), the European Respiratory Society (ERS/ATS 2017), the European Respiratory Society (ERS 2017), the U.S. Preventive Services Task Force (USPSTF 2016), the Alpha-1 Foundation (Alpha-1 2016), the International Society for Heart and Lung Transplantation (ISHLT 2015), and the American College of Physicians (ACP 2011). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 16 17 17 17 18 Definition https://web.pathway.md/diseases/recAhwo46uP9QXlBg 1/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway COPD is a chronic lung disease characterized by non-reversible airflow obstruction and persistent symptoms of dyspnea and productive cough. 16 Epidemiology COPD is caused by airway inflammation and remodeling due to environmental exposures, primarily tobacco smoke. Inflammation and oxidative stress in the airway, alveoli, and pulmonary microvasculature causes destruction of lung parenchyma, and loss of elastic recoil in the lung tissue. 17 17 Pathophysiology In the United States, the prevalence of the COPD is estimated at approximately 12,000 persons per 100,000 population. 16 Disease course These pathologic changes lead to manifestations of irreversible airflow obstruction, as well as a progressive decline in lung function, which can progress to chronic respiratory failure. 17 Prognosis and risk of recurrence The standardized mortality rate of patients with COPD is estimated at 2.7 (95% CI, 2.5-3.0) in men and 4.8 (95% CI, 4.2-5.4) in women, respectively. 18 Calculator Calculator Calculator BAP 65 score for acute exacerb BODE index for survival of COPD COPD ass Guidelines 1. Screening and diagnosis Indications for screening: As per USPSTF 2016 guidelines, do not obtain screening for COPD in asymptomatic adults. D As per ACP 2011 guidelines, do not obtain spirometry for screening of airflow obstruction in individuals without respiratory symptoms. D Diagnosis: As per GOLD 2023 guidelines, recognize that: COPD is a heterogeneous lung condition characterized by chronic respiratory symptoms (dyspnea, cough, sputum production, exacerbations) due to abnormalities of the airways https://web.pathway.md/diseases/recAhwo46uP9QXlBg 2/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway (bronchitis, bronchiolitis) and/or alveoli (emphysema) causing persistent, often progressive, airflow obstruction COPD results from gene-environment interactions occurring over the lifetime of the individual that can damage the lungs and/or alter their normal development/aging processes the main environmental exposures leading to COPD are tobacco smoking and the inhalation of toxic particles and gases from household and outdoor air pollution, but other environmental and host factors (including abnormal lung development and accelerated lung aging) can also contribute the most relevant (albeit rare) genetic risk factor for COPD identified to date are mutations in the SERPINA1 gene leading to AAT deficiency; a number of other genetic variants have also been associated with reduced lung function and risk of COPD, but their individual effect size is small. Show 2 more As per VA 2021 guidelines, consider confirming the clinical diagnosis of COPD based on post- bronchodilator spirometry. C 2. Classification and risk stratification Severity assessment: Use the modified MRC dyspnea scale, based on the level of dyspnea, for severity assessment: Situation Guidance I only get breathless with strenuous exercise Grade 0 I get short of breath when hurrying on the level or walking up a slight hill Grade 1 I walk slower than people of the same age on the level because of breathlessness, or I have to stop for breath when walking on my own pace on the level Grade 2 I stop for breath after walking about 100 meters or after a few minutes on the level Grade 3 I am too breathless to leave the house or I am breathless when dressing or undressing. Grade 4 Show 2 more 3. Diagnostic investigations History and physical examination: https://web.pathway.md/diseases/recAhwo46uP9QXlBg 3/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway Elicit a detailed medical history in patients with known or suspected COPD, including: Situation Guidance Smoking Exposures Occupational exposures Environmental exposures (household/outdoor) Early life events (prematurity, low birth weight, maternal smoking during pregnancy, passive smoking exposure during infancy) Past medical history Asthma, allergy, sinusitis, or nasal polyps Respiratory infections in childhood Human immunodeficiency virus Tuberculosis COPD Family history Other chronic respiratory diseases COPD typically develops in adult life and most patients are conscious of increased breathlessness, more frequent or prolonged "winter colds," and some social restriction for a number of years before seeking medical help Pattern of symptom development History of exacerbations or previous hospitalizations for a respiratory disorder Exacerbations CVDs Comorbidities Osteoporosis Musculoskeletal disorders Anxiety and depression Malignancies Limitation of activity Impact on patient's life Missed work and economic impact Effect on wellbeing Effect on family routines Effect on sexual activity Feelings of depression or anxiety Availability of social and family support. Social factors https://web.pathway.md/diseases/recAhwo46uP9QXlBg 4/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway Recognize that physical signs of airflow limitation are usually not present until significant impairment of lung function is present, and a physical examination is rarely diagnostic in COPD. Spirometry: As per GOLD 2023 guidelines, obtain spirometry to establish the diagnosis of COPD by eliciting a post-bronchodilator FEV in 1 second/FVC < 0.70 confirming the presence of persistent airflow limitation. As per VA 2021 guidelines, consider obtaining post-bronchodilator spirometry to confirm the clinical diagnosis of COPD. C As per ACP 2011 guidelines, obtain spirometry to diagnose airflow obstruction in patients with respiratory symptoms. B 4. Respiratory support Long-term oxygen therapy: As per GOLD 2023 guidelines, initiate long-term oxygen therapy to increase survival in patients with severe resting hypoxemia. A Show 2 more Landmark trials: NOTT In patients with COPD and hypoxemia, continuous oxygen therapy was superior to nocturnal oxygen therapy with respect to death at 1 year. Nocturnal Oxygen Therapy Trial Group. Ann Intern Med. 1980 Sep. As per VA 2021 guidelines: Initiate long-term oxygen therapy in patients with chronic stable resting severe hypoxemia (PaO < 55 mmHg and/or SaO 88%) or chronic stable resting moderate hypoxemia (PaO 56-59 mmHg or SaO > 88% and 90%) with signs of tissue hypoxia (hematocrit > 55%, pulmonary hypertension, or cor pulmonale). A Avoid offering routine ambulatory long-term supplemental oxygen in patients with chronic stable isolated exercise-induced hypoxemia without other indications for supplemental oxygen. D Landmark trials: LTOT In patients with stable COPD who had moderate resting desaturation (SpO2 89-93%) and moderate exercise-induced desaturation (during the 6-minute walk test, SpO2 80% for 5 minutes and < 90% for 10 seconds), supplemental oxygen was not superior to no supplemental oxygen with respect to the incidence of death or first hospitalization. https://web.pathway.md/diseases/recAhwo46uP9QXlBg 5/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway Long-Term Oxygen Treatment Trial Research Group et al. N Engl J Med. 2016 Oct 27. As per ACP 2011 guidelines, initiate continuous oxygen therapy in patients with COPD with severe resting hypoxemia (PaO 55 mmHg or SpO 88%). B Updated evidence: AECOPD In patients with acute COPD exacerbation and mild hypercapnia, high-flow nasal cannula was not superior to conventional oxygen therapy with respect to the percentage of patients who needed intubation. Jingen Xia et al. Crit Care. 2022 Apr 15. Noninvasive ventilation: As per GOLD 2023 guidelines: Consider initiating long-term noninvasive positive-pressure ventilation to improve hospitalization-free survival in patients with severe chronic hypercapnia (particularly with pronounced daytime persistent hypercapnia, arterial partial pressure of CO 53 mmHg) and a history of hospitalization for acute respiratory failure. C Initiate noninvasive mechanical ventilation as the first mode of ventilation, in the absence of absolute contraindications, to improve gas exchange, reduce work of breathing and the need for intubation, decrease hospitalization duration and improve survival in patients with acute respiratory failure. A Landmark trials: NIV in COPD with chronic hypercapnia In patients with persistent hypercapnia (arterial partial pressure of CO2 > 53 mmHg) who had an acute COPD exacerbation, home noninvasive ventilation plus oxygen therapy was superior to home oxygen therapy alone with respect to readmission or death at 12 months. Murphy PB et al. JAMA. 2017 Jun 6. As per AATS 2021 guidelines, consider offering nocturnal noninvasive ventilation in addition to usual care in patients with chronic stable hypercapnic COPD. C Show 4 more 5. Medical management General principles: aim treatment at reduction of symptoms and future risk of exacerbations. Show 3 more Short-acting bronchodilators: https://web.pathway.md/diseases/recAhwo46uP9QXlBg 6/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway As per GOLD 2023 guidelines: Initiate regular and as-needed short-acting -agonists or SAMAs to improve FEV in 1 second and symptoms in patients with stable COPD. Initiate combinations of short-acting -agonists or SAMAs for better improvements in FEV in 1 second and symptoms. A Administer inhaled short-acting -agonists, with or without SAMAs, as the initial bronchodilators for the treatment of acute exacerbations. B Long-acting bronchodilators: As per GOLD 2023 guidelines, initiate regular inhaled bronchodilators to prevent or reduce symptoms in patients with stable COPD. A Show 5 more Landmark trials: UPLIFT In patients with moderate-to-very-severe COPD, tiotropium was not superior to placebo with respect to the incidence of decline in post-bronchodilator FEV in 1 second. Tashkin DP et al. N Engl J Med. 2008 Oct 9. As per VA 2021 guidelines, initiate inhaled LAMAs as first-line therapy in patients with symptomatic COPD. A Show 2 more As per ATS 2020 guidelines, initiate long-acting -agonist/LAMA combination therapy over long- acting -agonist or LAMA monotherapy in patients with COPD complaining of dyspnea or exercise intolerance. B Landmark trials: FLAME (indacaterol-glycopyrronium) In patients who had COPD with a history of at least one exacerbation during the previous year, indacaterol and glycopyrronium were superior to salmeterol and fluticasone with respect to COPD exacerbation. Wedzicha JA et al. N Engl J Med. 2016 Jun 9. As per CTS 2019 guidelines, initiate inhaled long-acting bronchodilators, either LAMAs or long- acting -agonists, to reduce dyspnea, improve exercise tolerance, and improve health status in patients with COPD. A Show 3 more As per ERS 2017 guidelines, initiate a long-acting -agonist in preference to long-acting - agonist monotherapy for the prevention of future exacerbations in patients with COPD with moderate or severe airflow obstruction and a history of 1 exacerbations during the previous year. B https://web.pathway.md/diseases/recAhwo46uP9QXlBg 7/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway As per ACP 2011 guidelines, initiate inhaled bronchodilators in patients with stable COPD with respiratory symptoms and FEV in 1 second < 60% of predicted. B Show 3 more Inhaled corticosteroids: As per GOLD 2023 guidelines, offer ICSs combined with long-acting -agonists to improve function and health status and reduce exacerbations in patients with exacerbations and moderate-to-very severe COPD. A Show 3 more As per VA 2021 guidelines, do not use ICSs as first-line therapy in patients with symptomatic COPD. D Show 3 more As per ATS 2020 guidelines, consider initiating triple therapy with ICS/long-acting - agonist/LAMA over dual therapy with long-acting -agonist/LAMA in patients with COPD complaining of dyspnea or exercise intolerance despite dual therapy with long-acting - agonist/LAMA and with a history of 1 exacerbation in the past year requiring antibiotics or oral corticosteroids or hospitalization. C Show 2 more Landmark trials: WISDOM In patients with a history of exacerbation of COPD receiving triple therapy consisting of tiotropium (at a dose of 18 g once daily), salmeterol (50 g BID), and the ICS fluticasone propionate (500 g BID) during a 6-week run-in period, corticosteroid withdrawal was noninferior to corticosteroid continuation with respect to moderate or severe COPD exacerbation. Magnussen H et al. N Engl J Med. 2014 Oct 2. As per CTS 2019 guidelines, consider adding an ICS in patients with persistent dyspnea and poor health status in the last year despite LAMA/long-acting -agonist dual therapy. B Show 5 more Landmark trials: IMPACT In patients with chronic obstructive lung disease, triple therapy with fluticasone furoate, umeclidinium, and vilanterol resulted in a lower rate of moderate or severe exacerbations than fluticasone furoate-vilanterol or umeclidinium-vilanterol. The risk of clinician-diagnosed pneumonia was significantly higher with triple therapy than with umeclidinium-vilanterol. Lipson DA et al. N Engl J Med. 2018 May 3. Phosphodiesterase-4 inhibitors: https://web.pathway.md/diseases/recAhwo46uP9QXlBg 8/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway As per GOLD 2023 guidelines, offer inhaled bronchodilators over oral bronchodilators. A Do not use theophylline unless other long-term treatment bronchodilators are unavailable or unaffordable. B Show 2 more As per CTS 2019 guidelines, offer oral roflumilast in patients with chronic bronchitis and a high risk of COPD exacerbations despite optimal long-acting inhaled therapy. A Show 2 more As per ERS 2017 guidelines, consider offering roflumilast for the prevention of future exacerbations in patients with COPD with severe or very severe airflow obstruction, symptoms of chronic bronchitis, and exacerbations despite optimal inhaled therapy. C Oral corticosteroids: As per GOLD 2023 guidelines: Consider administering systemic corticosteroids (not more than 5 days) to improve FEV in 1 second, oxygenation, and shorten recovery time and hospitalization duration in patients with acute exacerbation. B Do not use long-term oral corticosteroids in patients with COPD because of numerous side effects D and the lack of benefits. D As per ATS 2020 guidelines, do not use maintenance oral corticosteroids in patients with COPD and a history of severe and frequent exacerbations despite otherwise optimal therapy. D Mucolytics: As per GOLD 2023 guidelines, offer regular treatment with mucolytics, such as erdosteine, carbocysteine, or N-acetylcysteine, to reduce the risk of exacerbations in selected patients with COPD. B As per VA 2021 guidelines, insufficient evidence to recommend for or against the use of N- acetylcysteine in patients with stable COPD continuing to experience respiratory symptoms (dyspnea, cough). I As per CTS 2019 guidelines: Offer oral N-acetylcysteine (600 mg BID) in patients with chronic bronchitis and a high risk of COPD exacerbations despite optimal long-acting inhaled therapy. B Insufficient evidence to determine whether mucolytics confers additional benefit to standard therapy in reducing dyspnea, improving exercise tolerance and activity levels, and/or improving health status. I As per ERS 2017 guidelines, consider offering oral mucolytics for the prevention of future exacerbations in patients with COPD with moderate or severe airflow obstruction and exacerbations despite optimal inhaled therapy. C Long-term antibiotics: As per GOLD 2023 guidelines, consider offering long-term macrocodes, particularly azithromycin, preferentially in, but not limited to, former smokers with exacerbations despite appropriate therapy, C to reduce exacerbations. B Recognize that azithromycin therapy is https://web.pathway.md/diseases/recAhwo46uP9QXlBg 9/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway associated with an increased incidence of bacterial resistance B and hearing test impairment. C As per BTS 2020 guidelines, consider offering long-term macrolide therapy for a minimum of 6 months and up to 12 months to reduce exacerbation rate in patients with COPD with > 3 acute exacerbations requiring corticosteroid therapy and at least 1 exacerbation requiring hospital admission per year. C As per CTS 2019 guidelines, consider offering long-term macrolide maintenance therapy in patients at high risk of COPD exacerbations despite optimal long-acting inhaled therapy. B As per ERS 2017 guidelines: Consider offering a macrolide antibiotic for the prevention of future exacerbations in patients with COPD with moderate-to-very severe airflow obstruction and exacerbations despite optimal inhaled therapy. C Do not use fluoroquinolones for the sole purpose of preventing future COPD exacerbations. D Other pharmacological agents: As per GOLD 2023 guidelines, do not use statins and/or -blockers for the prevention of exacerbations in patients with COPD at increased risk of exacerbation and without indications for statin therapy, D recognizing that statins may have positive effects on some outcomes in patients with COPD receiving statins for cardiovascular and metabolic indications. D Show 3 more Landmark trials: BOREAS In patients with COPD who had a blood eosinophil count 300 cells/mL and an elevated exacerbation risk despite the use of standard triple therapy, dupilumab was superior to placebo with respect to annualized rate of moderate or severe exacerbations. Surya P Bhatt et al. N Engl J Med. 2023 May 21. Online ahead of print. As per CTS 2019 guidelines, insufficient evidence to determine whether statins, anabolic steroids, PDE5 inhibitors, or oral Chinese herbal medicines confer additional benefits to standard therapy in reducing dyspnea, improving exercise tolerance and activity levels, and/or improving health status. I Management of acute exacerbations (setting of care): consider offering a home-based management program (hospital-at-home) in patients with COPD exacerbation presenting to the emergency department or hospital. C Management of acute exacerbations (SABA): administer inhaled short-acting -agonists, with or without SAMAs, as the initial bronchodilators for the treatment of acute exacerbations. B Management of acute exacerbations, corticosteroids: https://web.pathway.md/diseases/recAhwo46uP9QXlBg 10/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway As per GOLD 2023 guidelines, consider administering systemic corticosteroids (not more than 5 days) to improve FEV in 1 second, oxygenation, and shorten recovery time and hospitalization duration in patients with acute exacerbation. B As per ATS/ERS 2017 guidelines: Consider administering a short course ( 14 days) of oral corticosteroids in ambulatory patients with COPD exacerbation. C Consider administering oral (if gastrointestinal access and function are intact) rather than IV corticosteroids in patients hospitalized with COPD exacerbation. C Landmark trials: REDUCE (COPD) In patients presenting to the emergency department with acute COPD exacerbation, short- term treatment was noninferior to conventional treatment with respect to recurrent COPD exacerbation at 6 months. Leuppi JD et al. JAMA. 2013 Jun 5. Management of acute exacerbations, antibiotics: As per GOLD 2023 guidelines, consider administering antibiotics for 5 days to shorten recovery time and reduce the risk of early relapse, treatment failure, and hospitalization duration in patients with acute exacerbation. C As per DoD/VA 2021 guidelines, insufficient evidence to recommend for or against the use of antibiotics for outpatient COPD exacerbations (CRP guided or not). I As per ATS/ERS 2017 guidelines, consider administering antibiotics in ambulatory patients with COPD exacerbation. C Management of acute exacerbations (PDE4 inhibitors): do not use methylxanthines for the management of patients with acute exacerbation. D Management of acute exacerbations, noninvasive ventilation: As per GOLD 2023 guidelines, initiate noninvasive mechanical ventilation as the first mode of ventilation, in the absence of absolute contraindications, to improve gas exchange, reduce work of breathing and the need for intubation, decrease hospitalization duration and improve survival in patients with acute respiratory failure. A As per ATS/ERS 2017 guidelines, initiate noninvasive mechanical ventilation in patients hospitalized with COPD exacerbation associated with acute or acute-on-chronic respiratory failure. B Management of acute exacerbations (pulmonary rehabilitation): Avoid initiating pulmonary rehabilitation during hospitalization in patients hospitalized with COPD exacerbation. D Consider initiating pulmonary rehabilitation within 3 weeks after discharge in patients hospitalized with COPD exacerbation. C https://web.pathway.md/diseases/recAhwo46uP9QXlBg 11/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway Palliative care, nonpharmacological measures: As per GOLD 2023 guidelines, consider offering a fan blowing air onto the face to relieve dyspnea. C As per CTS 2011 guidelines, advise patients with advanced COPD of the potential benefits of pursed-lip breathing and instruct them in its use. B Show 2 more Palliative care (nutritional support): Provide nutritional supplementation to improve respiratory muscle strength and overall health status in malnourished patients. B Consider providing self-management education, pulmonary rehabilitation, nutritional support, and mind-body interventions to improve fatigue. C Palliative care (supplemental oxygen): Consider offering continuous oxygen therapy in hypoxemic (at rest) patients with advanced COPD. C Insufficient evidence to support the routine use of supplemental oxygen to reduce dyspnea in non-hypoxemic patients with advanced COPD. I Palliative care, opioids: As per GOLD 2023 guidelines, consider offering opioids to relieve dyspnea. C Consider offering low-dose long-acting oral or parenteral opioids for the treatment of dyspnea in patients with severe disease. C Landmark trials: BEAMS (morphine 16 mg) In adult patients with COPD and chronic breathlessness, morphine was not superior to placebo with respect to reduction in intensity of worst breathlessness at week 1. Magnus Ekstr m et al. JAMA. 2022 Nov 22. As per ATS 2020 guidelines, consider offering opioid-based therapy, within a personalized shared decision-making approach, in patients with COPD experiencing advanced refractory dyspnea despite otherwise optimal therapy. C As per CTS 2011 guidelines, offer oral (but not nebulized) opioids for the treatment of refractory dyspnea in individual patients with advanced COPD. B Palliative care, neuromuscular electrical stimulation: As per GOLD 2023 guidelines, consider offering neuromuscular electrical stimulation to relieve dyspnea. C As per CTS 2011 guidelines, consider offering neuromuscular electrical stimulation and chest wall vibration to reduce dyspnea in patients with advanced COPD. C Palliative care (therapies with no evidence for benefit): https://web.pathway.md/diseases/recAhwo46uP9QXlBg 12/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway Do not use anxiolytics and antidepressants routinely for the management of dyspnea in patients with advanced COPD. D Insufficient evidence to recommend the routine use of acupuncture, acupressure, distractive auditory stimuli (music), relaxation, handheld fans, counseling and support programs, and psychotherapy in patients with advanced COPD. I Palliative care (end-of-life care): discuss with patients and their families about end-of-life care including their views on resuscitation, advance directives, and place of death preferences. B 6. Nonpharmacologic interventions Smoking cessation: As per GOLD 2023 guidelines, offer smoking cessation interventions actively in all patients with COPD. A As per VA 2021 guidelines, advise smoking cessation for the prevention and risk reduction of COPD. A Avoidance of exposures: Advise on efficient ventilation, using non-polluting cooking stoves, and offering similar interventions in patients with COPD. B Advise avoiding continued exposures to potential irritants, if possible. B Physical activity: advise patients to increase their level of physical activity as it is a strong predictor of mortality. A Pulmonary rehabilitation: As per GOLD 2023 guidelines, offer pulmonary rehabilitation in all patients with relevant symptoms and/or high risk for exacerbation, to improve dyspnea, health status, and exercise tolerance in stable patients, A to reduce hospitalizations in patients with a recent exacerbation, B and to reduce symptoms of anxiety and depression. A As per ACP 2011 guidelines: Offer pulmonary rehabilitation in symptomatic patients with an FEV in 1 second < 50% of predicted. B Consider offering pulmonary rehabilitation in symptomatic or exercise-limited patients with an FEV in 1 second > 50% of predicted. C 7. Therapeutic procedures Bronchoscopic interventions: offer bronchoscopic interventions, such as endobronchial valves, A lung coils, B and vapor ablation, B to reduce end-expiratory lung volume and improve exercise tolerance, health status, quality of life, and lung function at 6-12 months following treatment in selected patients with advanced emphysema. B https://web.pathway.md/diseases/recAhwo46uP9QXlBg 13/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway 8. Surgical interventions Lung volume reduction surgery: offer lung volume reduction surgery to improve survival in patients with severe upper-lobe emphysema and low post-rehabilitation exercise capacity. A Landmark trials: NETT In patients with severe emphysema who underwent pulmonary rehabilitation, lung volume- reduction surgery was superior to medical therapy with respect to improvement in exercise capacity at 24 months. Fishman A et al. N Engl J Med. 2003 May 22. Surgical bullectomy: consider offering surgical bullectomy to decrease dyspnea, and improve lung function and exercise tolerance in selected patients with a large bulla. C Lung transplantation: As per GOLD 2023 guidelines, consider offering lung transplantation to improve quality of life and functional capacity in patients with very severe COPD (progressive disease, BODE score of 7-10, and illegible for lung volume reduction surgery) with a least one of the following: history of hospitalization for exacerbation associated with acute hypercapnia (partial pressure of CO > 50 mmHg) pulmonary hypertension and/or pulmonare despite oxygen therapy FEV in 1 second < 20% and either diffusion capacity of the lung for CO < 20% or homogeneous distribution of emphysema. C As per ATS 2015 guidelines, refer patients with COPD for lung transplantation if they meet one of the following criteria: disease is progressive, despite maximal treatment including medication, pulmonary rehabilitation, and oxygen therapy endoscopic or surgical lung volume reduction surgery is not possible BODE score of 5-6 arterial partial pressure of CO > 50 mmHg or 6.6 kPa paO2 < 60 mmHg or 8 kPa FEV < 25% of predicted. 9. Specific circumstances Patients with alpha-1 antitrypsin deficiency: As per GOLD 2023 guidelines, consider initiating IV augmentation therapy to slow down the progression of emphysema in patients with AAT deficiency and COPD (with never or ex- https://web.pathway.md/diseases/recAhwo46uP9QXlBg 14/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway smokers with an FEV in 1 second of 35-60% of predicted and patients with severe hereditary AAT deficiency with established emphysema being the most suitable candidates). C As per ERS 2017 guidelines: Initiate IV augmentation therapy to reduce the progression of emphysema in patients with severe AAT deficiency. E Insufficient evidence to support the use of augmentation therapy in PiSZ or PiMZ subjects or current smokers of any protein phenotype. I As per Alpha-1 2016 guidelines, initiate IV augmentation therapy in patients with AAT deficiency and an FEV in 1 second in the range of 30-65% of predicted. A Show 10 more As per CTS 2012 guidelines, consider initiating AAT augmentation therapy in non-smoking or ex-smoking patients with COPD with a FEV in 1 second in the range of 25-80% of predicted attributable to emphysema and documented low levels of AAT, i.e. 11 mcmol/L, receiving optimal pharmacological and nonpharmacological therapies, including comprehensive case management and pulmonary rehabilitation, for the benefits in CT lung density C and mortality. C 10. Patient education General counseling: As per GOLD 2023 guidelines: Provide education in patients with COPD, although education alone has not been shown to be effective to change the patient's behavior. B Provide self-management intervention skills to be communicated with a healthcare professional, with or without the use of a written action plan, to improve health status and prevent exacerbation complications decrease (such as hospitalizations and emergency department visits). B As per VA 2021 guidelines: Offer a supported self-management program including a written action plan with exacerbation management, smoking cessation, and exercise. B Consider offering telehealth support including telemonitoring and/or mobile applications. C 11. Preventative measures Routine immunizations: As per GOLD 2023 guidelines, offer influenza and SARS-CoV-2 vaccination in patients with COPD. B Show 3 more As per VA 2021 guidelines, consider offering routine vaccination for influenza and pneumococcal pneumonia for prevention and risk reduction of COPD exacerbations. C https://web.pathway.md/diseases/recAhwo46uP9QXlBg 15/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway 12. Follow-up and surveillance Follow-up: monitor symptoms, exacerbations, and objective measures of airflow limitation during routine follow-ups of patients with COPD to determine when to modify management and to identify any complications and/or comorbidities that may develop. References 1. Alvar Agusti, Richard Beasley, Bartolome R. Celli et al. Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Pulmonary Disease: 2023 Report. GOLD. 2023. Open 2. Qaseem A, Wilt TJ, Weinberger SE et al. Diagnosis and management of stable chronic obstructive pulmonary disease: a clinical practice guideline update from the American College of Physicians, American College of Chest Physicians, American Thoracic Society, and European Respiratory Society. Ann Intern Med. 2011 Aug 2;155 3 179 91. Open 3. US Preventive Services Task Force USPSTF , Siu AL, Bibbins-Domingo K et al. Screening USPSTF for Chronic Obstructive Pulmonary Disease: US Preventive Services Task Force Recommendation Statement. JAMA. 2016 Apr 5;315 13 1372 7. Open 4. O'Donnell DE, Aaron S, Bourbeau J et al. VA/DoD clinical practice guideline for the management of chronic obstructive pulmonary disease. VA/DoD. 2021 Apr;Version 3.0. Open 5. Wedzicha JA Ers Co-Chair, Miravitlles M, Hurst JR et al. Management of COPD exacerbations: a European Respiratory Society/American Thoracic Society guideline. Eur Respir J. 2017 Mar 15;49 3 . Open 6. Bourbeau J, Bhutani M, Hernandez P et al. 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A consensus document for the selection of lung transplant candidates: 2014 an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015 Jan;34 1 1 15. Open 11. Linda Nici, Manoj J Mammen, Edward Charbek et al. Pharmacologic Management of Chronic Obstructive Pulmonary Disease. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020 May 1;201 9):e56-e69. Open 12. Jadwiga A Wedzicha, Peter M A Calverley, Richard K Albert et al. Prevention of COPD exacerbations: a European Respiratory Society/American Thoracic Society guideline. Eur Respir J. 2017 Sep https://web.pathway.md/diseases/recAhwo46uP9QXlBg 16/18 6/23/23, 3:26 AM Chronic obstructive pulmonary disease Pathway 9;50 3 1602265. Open 13. Robert A Sandhaus, Gerard Turino, Mark L Brantly et al. The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult. 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