PANCE Blueprint Pulmonary (12%)

Idiopathic pulmonary fibrosis

Patient will present as → a 55-year-old female who is a current smoker presents with a 9-month history of respiratory symptoms, including dyspnea on exertion, thoracic pain and dry cough, which were preceded by a pulmonary infection. On auscultation you hear inspiratory crackles. Pulmonary function tests (PFTs) show only mild impairment of vital capacity with decreased lung volume and a normal to increased FEV1/FVC ratio

Idiopathic pulmonary fibrosis is the most common of all interstitial lung diseases

In order to be considered "idiopathic" you must be sure to rule out other common causes such as drugs, and environmental or occupational exposures

Common "non-idiopathic" causes of pulmonary fibrosis which must be ruled out

  • Cigarette smoking
  • Certain viral infections
  • Exposure to environmental pollutants, including silica and hard metal dusts, bacteria and animal proteins, and gases and fumes
  • The use of certain medicines (methotrexate, amiodarone, nitrofurantoin, rituximab, bleomycin, and cyclophosphamide)
  • Genetics
  • Radiation treatment
  • Gastroesophageal reflux disease (GERD)

CXR shows fibrosis

CT chest: diffuse patchy fibrosis with pleural based honeycombing

PFTs will demonstrate a restrictive pattern - opposite of what you would see with asthma

  • Decreased lung volume with a normal to increased FEV1/FVC ratio.

Treatment includes the judicial use of corticosteroids, O2, and eventually lung transplant

Chest CT showing classic "honeycombing"

Chest CT showing classic "honeycombing"

Question 1
What is the diagnostic study of choice for pulmonary hypertension?
A
right heart catheterization
B
spirometry
Hint:
Patients initially undergo chest x-ray, spirometry, and ECG to identify more common causes of dyspnea,
C
chest X-ray
Hint:
Patients initially undergo chest x-ray, spirometry, and ECG to identify more common causes of dyspnea,
D
echocardiography
Hint:
pulmonary artery pressure is estimated by echocardiography
Question 1 Explanation: 
Gold standard for diagnosis is right heart catheterization. Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels become constricted. Severe pulmonary hypertension leads to right ventricular overload and failure. Symptoms are fatigue, exertional dyspnea, and, occasionally, chest discomfort and syncope. Diagnosis is made by finding elevated pulmonary artery pressure (estimated by echocardiography and confirmed by right heart catheterization). Treatment is with pulmonary vasodilators and diuretics. In some advanced cases, lung transplantation is an option. Prognosis is poor overall if a treatable secondary cause is not found.
Question 2
An O2 saturation of 90% corresponds to what PO2 value?
A
90 mmHg
Hint:
See D for explanation.
B
80 mmHg
Hint:
See D for explanation.
C
70 mmHg
Hint:
See D for explanation.
D
60 mmHg
Question 2 Explanation: 
O2 sat values above 90% correspond with a PO2 >70 mmHg and values less than 94% represent hypoxemia. Less than 90% O2 sat warrants measurement of arterial blood gasses.
Question 3
Pulmonary hypertension is defined as a mean pulmonary arterial pressure of
A
≥ 5 mm Hg
Hint:
See D for explanation
B
≥ 10 mm Hg
Hint:
See D for explanation
C
≥ 15 mm Hg
Hint:
See D for explanation
D
≥ 25 mm Hg
Question 3 Explanation: 
Blood pressure in the lungs is usually very low < 15 mm HG. In pulmonary hypertension the pressure increases > 25 mmHG at rest!
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