PANCE Blueprint Pulmonary (9%)

Patient will present as → a 67-year-old male with a 6-month history of progressive shortness of breath on exertion and a persistent dry cough. He has a past medical history of hypertension, controlled with medication, and a 30-pack-year smoking history, although he quit smoking 10 years ago. He denies any significant occupational exposures or a family history of lung disease. On examination, he appears tachypneic with a respiratory rate of 22 breaths per minute. Auscultation of the lungs reveals bilateral fine inspiratory crackles at the lung bases. His oxygen saturation on room air is 92%. Pulmonary function tests show a restrictive pattern with reduced forced vital capacity (FVC) and a preserved FEV1/FVC ratio. Diffusion capacity for carbon monoxide (DLCO) is significantly reduced. High-resolution computed tomography (HRCT) of the chest demonstrates bilateral reticular opacities, honeycombing, and traction bronchiectasis predominantly at the lung bases, without significant lymphadenopathy or pleural effusion. A diagnosis of Idiopathic Pulmonary Fibrosis is made. The patient is counseled about the nature of the disease, including its progressive course and the current lack of a cure. Treatment options, including antifibrotic therapy with nintedanib or pirfenidone to slow disease progression, are discussed. The importance of pulmonary rehabilitation, vaccinations, and supplemental oxygen therapy as needed is emphasized. The patient is also advised on lifestyle modifications, including regular exercise and smoking cessation support for his prior smoking habit. Referrals to a support group for patients with IPF and a palliative care consultation to manage symptoms and improve quality of life are provided.

Chronic progressive lung disorder characterized by increasing scarring, which ultimately reduces the capacity of the lungs; etiology unknown

• 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 - the opposite of what you would see with asthma
  • Decreased total lung capacity, decreased forced vital capacity (FVC), decreased forced expiratory volume in one second (FEV1)
  • The FEV1/FVC ratio may be normal or greater than 80% (increased) due to a significant decrease in forced vital capacity (FVC)
  • Despite the reduction in FVC, the FEV1/FVC ratio is preserved or even greater than 80% (increased) because both FEV1 (forced expiratory volume in the first second) and forced vital capacity (FVC) are proportionally reduced

Pulmonary fibrosis induced by amiodarone.

Chest CT showing classic “honeycombing”

Treatment may include antifibrotic drugs (pirfenidone or nintedanib), oxygen therapy, and eventually lung transplant

• Most patients deteriorate, and the median survival is about 3 years from diagnosis