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 a decrease in total lung capacity, decreased forced vital capacity (FVC), and decreased forced expiratory volume in 1 second (FEV1). Chest CT demonstrates diffuse patchy fibrosis with pleural-based honeycombing.
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)
- 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
- Lung volume decreases in restrictive lung disease and the FEV1/FVC ratio is greater than 80% (increased) due to a significant decrease in forced vital capacity (FVC)
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
Early inspiratory crackles
Late, not early, inspiratory crackles are associated with interstitial lung disease.
Progressive dyspnea on exertion
Productive cough with copious sputum
A productive cough of copious amounts of sputum is most typical of a patient with chronic bronchitis.
Decreased breath sounds with hyperresonant percussion
Physical examination findings of decreased breath sounds with hyperresonant percussion is consistent with a diagnosis of chronic obstructive lung disease.
COPD appears as hyperinflation with flattening of the diaphragm on chest radiograph
Tuberculosis presents with pulmonary infiltrates on chest radiograph most often apical; cavitations may be seen with progressive primary tuberculosis.
Chest x-ray in bronchiectasis shows dilated and thickened bronchi that appear as ring-like markings.