COPD and Alpha-1 Antitrypsin Deficiency

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Questions and Answers

Which characteristic is NOT part of the diagnosis for mild intermittent asthma?

Short acting beta agonist use ≤ 2 days/week

Daytime symptoms ≤ 2 days/week

Exacerbations needing oral steroid: 0-1/year

Nighttime symptoms > 2 nights/month (correct)

What is the FEV1 requirement for moderate persistent asthma?

FEV1 < 60% predicted

FEV1 > 50% predicted

FEV1 > 60%, but < 80% (correct)

FEV1 > 80% predicted

In stepwise therapy for asthma, which treatment is recommended for mild intermittent asthma?

Long-acting beta agonist

SABA PRN (correct)

ICS regimen

Oral corticosteroids

What defines a significant exacerbation for moderate persistent asthma?

Exacerbations needing oral steroid: ≥ 2/year Signup and view all the answers

Which symptom is characteristic of severe persistent asthma?

Daytime symptoms continuously all day Signup and view all the answers

Which of the following is NOT considered a risk factor for severe asthma exacerbations?

Using SABA PRN more than 2 days/week Signup and view all the answers

What is the primary role of short-acting beta agonists (SABAs) in asthma management?

To provide immediate relief of acute symptoms Signup and view all the answers

At what stage of asthma treatment is a short course of oral steroids considered?

Step 4 to 6 Signup and view all the answers

What may indicate significant hypercapnia in a patient with COPD?

Fall in FEV1 below 1 liter without respiratory depressants Signup and view all the answers

Which of the following is NOT a recommended screening practice for COPD patients?

Routine lung imaging every year Signup and view all the answers

Which statement about the effects of hypercapnia is true?

It can cause delirium and respiratory depression. Signup and view all the answers

What is a possible consequence of untreated COPD?

Development of cor pulmonale Signup and view all the answers

Which characteristic is associated with alpha-1 antitrypsin deficiency related to COPD?

Development of emphysema at a young age Signup and view all the answers

What is the purpose of weekly infusion of alpha-1 antitrypsin in COPD management?

To improve lung function and survival Signup and view all the answers

According to GOLD guidelines, what spirometric grade indicates very severe COPD?

FEV1 < 30% of predicted Signup and view all the answers

Which patient characteristic suggest a diagnosis of alpha-1 antitrypsin deficiency?

Family history of early onset emphysema Signup and view all the answers

Which consideration is essential for initiating lung cancer screening in COPD patients?

Patients with significant hyperinflation and air trapping with FEV1 < 45% Signup and view all the answers

What is the primary goal of long-term oxygen therapy for stable COPD patients?

To achieve oxygen saturation ≥ 90% Signup and view all the answers

In what circumstance is lung volume reduction surgery indicated for COPD patients?

For patients with upper lobe emphysema and low baseline exercise capacity Signup and view all the answers

Which of the following factors may indicate the need for supplemental oxygen in COPD patients?

Oxygen saturation below 88% on room air at rest Signup and view all the answers

What does the INOX trial suggest regarding nocturnal O2 supplementation in COPD patients?

It shows no survival benefit in isolated nocturnal hypoxemia cases Signup and view all the answers

Which treatment option is associated with improved survival in COPD patients experiencing persistent daytime hypercapnia?

Noninvasive positive pressure ventilation plus oxygen Signup and view all the answers

Which of the following best reflects the findings from the LOTT trial regarding oxygen therapy in COPD patients?

No survival benefit was demonstrated in patients with moderate desaturation Signup and view all the answers

What is the purpose of reevaluating COPD patients shortly after initiating oxygen therapy?

To assess the therapeutic effectiveness of oxygen saturation Signup and view all the answers

Study Notes

COPD

Persistent, severe hypoxia due to COPD (PaO2 less than 60 mmHg) leads to pulmonary hypertension, erythrocytosis, limited exercise capacity, and impaired mentation.
Significant hypercapnia can cause delirium and respiratory depression.
Avoid hypnotic agents, anxiolytics, and narcotic agents in hypercapnic patients.
Significant hypercapnia can be ruled out if the O2 saturation is normal on room air (> 96%).
If hypoxemia is present, then ABG is required to diagnose and quantify hypercapnia.
Hypercapnia is seen when FEV1 falls below 1 liter in the absence of respiratory depressants.

Alpha-1 Antitrypsin Deficiency

COPD developing at a young age, i.e., < 45 years of age, with a strong family history without significant cigarette smoking suggests this disorder.
Z-allele causes production of protein that is not transported out of the liver.
Homozygous ZZ has a serum alpha-1 antitrypsin level of 10 to 15% of normal and predisposes the patient to emphysema.
Cirrhosis of the liver develops in some patients.
Heterozygotes with MZ have serum alpha-1 antitrypsin level at 50% of normal and they are not predisposed to the development of emphysema.
Weekly infusion of alpha-1 antitrypsin at a dose of 60 mg/kg has been shown to slow the rate of decline of lung function and improve survival.
Candidates for this infusion are patients with alpha-1 antitrypsin deficiency with moderate airflow obstruction.
WHO recommends screening all patients diagnosed with COPD for alpha 1 antitrypsin deficiency, especially in areas with high prevalence.

Differential Diagnoses of COPD

Asthma
CHF
Bronchiectasis
TB
Obliterative bronchiolitis
Diffuse Panbronchiolitis

GOLD Guidelines for Management of COPD

Assess various aspects of COPD separately:
- Symptoms: Use COPD Assessment Test (CAT) or COPD Control Questionnaire (CCQ)
- Spirometric Grade: FEV1/FVC must be < 70%
 - GOLD 1: Mild - FEV1 ≥ 80% of predicted
 - GOLD 2: Moderate - FEV1 ≥ 50%, but < 80% of predicted
 - GOLD 3: Severe - FEV1 ≥ 30% but < 50% of predicted
 - GOLD 4: Very severe - FEV1 < 30% of predicted
- Exacerbations: ≥ 2 or ≥ 1 that led to admission to hospital/year (E) or 0-1 exacerbations that did not need hospital admission/year (A and B)
- Comorbidities: Cardiovascular disease, anxiety/depression, osteoporosis, metabolic syndrome, skeletal muscle dysfunction, and lung cancer.

Asthma

Poor inhaler technique, nonadherence, symptoms due to alternative diagnoses such as vocal cord dysfunction, comorbidities such as GERD/OSA/obesity/rhinosinusitis, continuous exposure to irritants at home/work should be ruled out before severe asthma diagnosis.

Asthma Definition Based on Symptoms and SABA Frequency (Before ICS Containing Regimen Has Been Started):

Mild Intermittent Asthma:
- Daytime symptoms ≤ 2 days/week
- Nighttime symptoms ≤ 2 nights/month
- FEV1 > 80% predicted, FEV1/FVC normal
- Normal FEV1 between exacerbations
- No interference with daily activity
- Exacerbation that needs oral steroid: 0-1/year
- Short acting beta agonist use to control symptoms: ≤ 2 days/week
- Treatment step: Step 1
Mild Persistent Asthma:
- Day symptoms: 3 - 6 days per week (not daily)
- Nighttime symptoms: 3-4 nights per month
- FEV1 > 80% predicted, FEV1/FVC normal
- Minor limitation in daily activity
- Exacerbations that need oral steroid: ≥ 2/year
- Short acting β agonist to control symptoms: > 2 days/week, not daily, not > 1x/day
- Treatment step: Step 2
Moderate Persistent Asthma:
- Daytime symptoms occur daily
- Nighttime symptoms > 1x/week, but not every night
- FEV1 is > 60%, but < 80%, FEV1/FVC reduced 5%
- Some limitation of daily activity
- Exacerbations that need oral steroid: ≥ 2/year
- Short acting β agonist to control symptoms: Daily
- Treatment step: Step 3 (and consider short course oral steroid)
Severe Persistent Asthma:
- Daytime symptoms continuously throughout the day
- Nighttime symptoms: nightly, 7x/week
- FEV1 < 60% predicted, FEV1/FVC reduced > 5%
- Severe limitation of daily activity
- Exacerbations that need oral steroid: ≥ 2/year
- Short acting β agonist to control symptoms: Many times/day
- Treatment step: Step 4 - 6 (and consider short course oral steroid)

Stepwise Therapy of Asthma

Step 1: SABA PRN. For patients with mild intermittent asthma only.
Step 2 – 6: For persistent grade asthma is given based on response.

CT Chest in COPD

Patients with persistent exacerbations, disproportion between PFT findings and symptoms (other differential diagnoses), significant hyperinflation and air trapping with FEV1 < 45% (lung volume reduction and other interventions), meet criteria for lung cancer screening.

Criteria for Long-Term Oxygen Therapy for Stable COPD

PaO2 ≤ 55 mmHg or oxygen saturation ≤ 88% on room air at rest with or without hypercapnia, confirmed twice over 3 weeks.
PaO2 > 55 but < 60 mmHg or O2 sat 88% + pulmonary HTN, edema suggesting CHF or hematocrit > 55% (right heart failure or erythrocytosis).

Reevaluation After Starting O2

Reevaluate after starting O2 in 60-90 days to see if O2 sat is therapeutic and if O2 is still needed.

Goal for O2 Therapy

Goal for O2 therapy is O2 saturation ≥ 90%.

Oxygen Therapy Prescription in COPD

For patients with stable COPD and only moderate resting oxygen desaturation (O2 sat 89-93%) or only moderate exercise induced desaturation (O2 sat 80-89%), O2 should not be routinely prescribed.
Patients with moderate exertional desaturation (80-89%) had no survival benefit when given oxygen in the LOTT trial.

Nocturnal Oxygen Supplementation

The INOX trial showed that nocturnal O2 supplementation had no survival benefit in patients with isolated nocturnal hypoxemia (OSA patients excluded), but the trial was underpowered, so cannot definitively rule out benefit.

Noninvasive Positive Pressure Ventilation + O2

Improves survival and decreases hospitalization after recent hospital admission in patients with persistent daytime hypercapnia (PaCO2 ≥ 53 mmHg).

Other COPD Treatments

Lung volume reduction surgery: Only for patients with upper lobe emphysema with low baseline exercise capacity.
Surgical Bullectomy (for large bulla), and bronchoscopic interventions to decrease end expiratory lung volume, improve exercise tolerance, quality of life and lung function (endobronchial valves, lung coils, vapor ablation) are other options.
Lung transplant is an option in the management of very severe COPD.

Bronchiectasis

Abnormal, permanent dilatation of bronchi.
This may be focal or diffuse.

USPSTF Recommendations for COPD Screening

USPSTF does not recommend screening for COPD in asymptomatic adults.

Emerging Concepts/Treatments in COPD

Eosinophil directed prednisolone treatment for acute exacerbation of COPD
Anti-eosinophil monoclonal antibody for COPD
Other Phosphodiesterase inhibitors

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Description

This quiz covers critical information regarding Chronic Obstructive Pulmonary Disease (COPD), focusing on its complications like hypoxia and hypercapnia. It also discusses Alpha-1 Antitrypsin Deficiency, emphasizing diagnosis and implications for young patients. Test your knowledge on these respiratory conditions and their management.