Question

A 68 year old woman with pulmonary fibrosis presents with worsening exertional dyspnea.

1) Describe pulmonary fibrosis and include the changes to lung structure and function that are expected with this disease.

2) What changes to pulmonary function testing (capacities and volumes) would you expect to see in this type of patient?

3) What are the treatments for pulmonary fibrosis?

Answer 1

1) Pulmonary fibrosis is a condition that causes lung scarring and stiffness. This makes difficulty in breathing. It can prevent body from getting adequate oxygen and eventually can lead to heart failure, respiratory failure, or other complications.    As pulmonary fibrosis progresses, it may lead to complications such as blood clots in the lungs, a collapsed lung or lung infections

2) First section of PFT is is spirometer. The second set of measurements during PFTs are lung volumes. Patients often feel like they are in an old-fashioned phone booth. Patients are instructed to paint while breathing on a mouth-piece.​ The last part of a PFT measures the way in which gases cross through the lung tissue or diffusion capacity. A low DLCO (lung diffusion capacity) may be the first abnormality seen by PFT for idiopathic pulmonary fibrosis patients and is often present before lung volume or spirometry values are abnormal.

3)    Treatments

Pharmacologic management : Corticosteroids , Cyclophosphamide , Azathioprine , N-acetylcysteine , Nintedanib , Proton pump inhibitors , Supplemental Oxygen Therapy.

Non-pharmacologic management ​: Avoid smoking, Home pulse oximetry , Pulmonary rehabilitation , Get vaccinated – be proactive to prevent problems, Achieve and maintain a healthy weight ​and Lung Transplantation .