COPD Case PresentationCOPD Case PresentationCOntinuing MeDiCal eDuCatiOn

1 CMe CreDit

PhysiciansPhysician Assistants

Nurse Practitioners

Valid 4/1/2011 – 7/1/201111/12

42 Year OlD feMale with a COugh

A 42 year old female presents to your office complaining of progressively worsening shortness of breath and cough productive of yellow sputum over the last two years. She complains of occasional wheezing with exertion and occasional nocturnal cough. She does not complain of weight loss or loss of appetite. She quit smoking three years ago after accumulating 21 pack years of tobacco use. Her father and a paternal uncle, both smokers, had “lung disease” while in their forties.

On exam she has mild clubbing with no cyanosis or lower extremity edema. Chest AP diameter is increased with globally diminished breath sounds and end expiratory wheezing without use of accessory muscles of respiration. Office spirometry reveals an FVC at 78% predicted, FEV1 at 56% predicted and FEV1/FVC is 48%.

learning ObjeCtiVes

Those completing this activity will receive information that should allow them to…

• Recognize the impact of Alpha 1 antitrypsin deficiency on COPD;

• Identify patient population at risk of COPD due to Alpha 1 antitrypsin deficiency; and

• Become familiar with treatment options for patients with COPD.

authOr

Sherif Al-Farra, MD, FCCP, DABSMAssociate Professor of Internal MedicineUniversity of North Texas Health Science Center

Dr. Al-Farra discloses that he has received a speaker honoraria from Astra Zeneca within the past 12 months.

hOw tO reCeiVe CreDit

After reading the case presentation, record your responses to the question on the response form and complete the credit request and evaluation. Return the response form, evaluation and credit request to:

PACE Office3500 Camp Bowie BlvdFort Worth TX 76107

Or fax to 817-735-2598.

Your certificate will be mailed within three weeks of receipt.

Questions? Call 800-987-2263.

release anD reView Date

This activity was last reviewed and released on April 1, 2011 and expires July 1, 2011. Credit cannot be awarded after this date.

COMMerCial suPPOrt

This activity is commercially supported by Boehringer Ingelheim and Pfizer. Great care has been exercised to ensure the content is fair and balanced.

aCCreDitatiOn anD CreDit

Physician AccreditationThe University of North Texas Health Science Center at Fort Worth is accredited by the American Osteopathic Association to award continuing medical education to physicians.

The University of North Texas Health Science Center is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to sponsor continuing medical education for physicians.

CreDit DesignatiOn

The University of North Texas Health Science Center has requested that the AOA Council on Continuing Medical Education approve this program for 1.0 hours of AOA Category 2B CME credits. Approval is currently pending.

The University of North Texas Health Science Center at Fort Worth designates this educational activity for a maximum of 1 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

PhYsiCian assistants anD nurse PraCtitiOners

The University of North Texas Health Science Center at Fort Worth is accredited by ACCME to provide continuing medical education for physicians, and will provide physician assistants and nurse practitioners who successfully complete the activity with a Statement of Participation indicating that the activity was designated for 1 AMA PRA Category 1 Credit(s)™.

COPD_CaseStudy11_17x11.indd 1-2 3/31/11 9:18 AM

Pre-assessMent

Because of her relatively young age you decide to screen for alpha 1 antitrypsin deficiency. Which of the following statements is most accurate?

A. PI Null variant is the most common phenotype associated with emphysema.

B. Heterozygous PI SZ phenotype is not associated with emphysema.

C. PI MM phenotype is associated with severe emphysema.

D. PI ZZ is the most common phenotype associated with emphysema.

E. Severe alpha 1 antitrypsin deficiency occurs in 1-2% of the general population.

Emphysema associated with alpha 1 antitrypsin deficiency is characterized by:

A. Centrilobular distribution.

B. Is not associated with bronchiectasis.

C. Bullous disease with upper lobe predominance on chest x-ray.

D. Panacinar distribution.

E. Perihilar fibrosis.

Based on GOLD guidelines she has:A. Mild COPD.

B. Moderate COPD.

C. Severe COPD.

D. Very severe COPD.

E. Minimal COPD.

Based on a plasma AAT level of 40 µmol/L, you should recommend:

A. Intravenous steroids.

B. Intravenous infusion of pooled human AAT.

C. Inhaled corticosteroids alone.

D. Combination therapy including long acting bronchodilators and inhaled corticosteroids in addition to an as needed short acting beta 2 agonist.

E. Supplemental oxygen if saturations are 90% with exertion.

Discussion of Question three

GOLD guidelines apply to patients with COPD regardless of cause including AAT. Spirometry is indicated in patients with COPD to facilitate a diagnosis and determine severity. An FEV1/FVC ratio less than 70% is diagnostic of obstruction. The severity of obstruction is based on the FEV1. If the FEV1 is above or equal to 80% of predicted the patient has mild COPD. If the FEV1 is between 50-80% of predicted the patient has moderate COPD. Severe COPD is diagnosed if the FEV1 is between 30-50% of predicted (which is the case in our patient) and very severe COPD is diagnosed when the FEV1 is below 30% or below 50% with evidence of respiratory failure. Minimal COPD is not part of the GOLD guidelines. C is the correct answer.

Discussion of Question four

Normal plasma levels of AAT are 20 to 53 µmol/L. A minimum AAT plasma threshold of 11 µmol/L is required to protect the lung. Below this level, patients are at a significantly increased risk of

developing emphysema and may benefit from Intravenous infusion of pooled human AAT. Our patient has severe COPD and should be on combination therapy including long acting

bronchodilators and inhaled corticosteroids in addition to an as needed short acting beta 2 agonist. Supplemental oxygen is indicated when saturation drops below 89%. There is no indication for intravenous steroids at this time and an inhaled corticosteroid alone would not be sufficient as monotherapy. Therefore, D is the right answer.

Please COMPlete the resPOnse fOrM befOre PrOCeeDing tO the Case DisCussiOn.

suMMarY

It is important to entertain alpha 1 antitrypsin deficiency as a risk factor for COPD especially in when severe disease occurs at a relatively young age (4th and 5th decade). Screening should be considered in all patients with COPD. PI ZZ is the most common phenotype associated with disease. In addition to therapy as recommended by GOLD, patients with a minimum AAT plasma threshold of 11 µmol/L may benefit from Intravenous infusion of pooled human AAT.

referenCes

American Thoracic Society/European Respiratory Society Statement: Standards for the Diagnosis and Management of Individuals with Alpha-1 Antitrypsin Deficiency Available at: http://www.thoracic.org/statements/ Accessed 01/19/2011.

Global strategy for the Diagnosis, Management, and prevention of Chronic Obstructive Disease. Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2009. Available at: http://www.goldcopd.org Accessed: 01/03/2011.

Continuous or nocturnal oxygen therapy in chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy Trial Group (Petty TL, Chairman) Ann Intern Med 1980;93:391-398.

baCKgrOunD

Chronic obstructive pulmonary disease (COPD) is responsible for one death every four minutes in the US. While 12 million Americans have been diagnosed with the disease, it is estimated that at least that many have COPD but are undiagnosed. Several COPD risk factors converge in East Texas, resulting in some of the highest rates of unnecessary hospitalizations in the state. Since primary care physicians, PAs and NPs in the region are the most likely to diagnose and manage COPD, they should be made aware of the most current information available on how to improve patient outcomes and overcome clinical barriers to diagnostic testing and treatment.

1

2

3

4

1

2

3

4

DisCussiOn

Discussion of Question One

Alpha 1 antitrypsin deficiency is a rare inherited disease affecting the lungs, liver and skin with severe deficiency impacting 0.02-0.05% of the general population. It is important to screen individuals with COPD for AAT deficiency as up to 1-2% of patients with COPD may have severe AAT deficiency. Screening is more important in individuals who develop COPD at a younger age (in their 40’s). The normal phenotype is PI MM. While PI ZZ is the most common phenotype associated with emphysema, PI SZ may lead to AAT deficiency. PI Null variant causes severe life threatening disease and is fortunately rare. Therefore, the answer is D.

Discussion of Question two

Patients with emphysema secondary to AAT deficiency develop panacinar rather than the centrilobular distribution usually seen in emphysema secondary to tobacco use. Bullous disease usually involves the lung bases on chest x-ray in individuals with AAT, while upper lobe involvement is more common in smokers. Even though the mechanism is not well understood, bronchiectasis (abnormal dilation of the proximal and medium-sized bronchi) occurs in patients with AAT with estimates varying between 10-40%. Based on this information, the answer is D.

COPD_CaseStudy11_17x11.indd 3-4 3/31/11 9:18 AM