Pulmonary fibrosis is one of nearly 200 chronic lung conditions that are characterized by inflammation and scarring of the lung tissue, which progressively cause breathing difficulties because the lungs are unable to expand and contract normally. The most common form of the disease is idiopathic pulmonary fibrosis.
All forms of pulmonary fibrosis are progressive and life-threatening, and the prognosis is poor with a median survival of 2.5 to 3.5 years after diagnosis. Respiratory failure is the most common cause of death in pulmonary fibrosis patients but early diagnosis and treatment can significantly improve survival.
Factors affecting prognosis
Three main factors that affect the prognosis of pulmonary fibrosis are:
- Early diagnosis: The severity of pulmonary fibrosis at diagnosis is directly correlated with survival rates. Early diagnosis is associated with better survival.
- Proper treatment: While anti-fibrotic treatments may not cure the disease, they can significantly slow its progression and hence improve prognosis.
- Rate of lung function decline: The progression of pulmonary fibrosis is not constant. In some patients, the disease may progress slowly and then suddenly accelerate and result in respiratory failure. Therefore, disease progression needs to be monitored constantly, which can improve prognosis.
Clinical factors that adversely affect prognosis
Clinical factors associated with poor prognosis are:
- Older age: Patients diagnosed when older usually have a poorer prognosis than younger pulmonary fibrosis patients.
- Smoking history: Overall, smokers and former smokers have a poorer prognosis than nonsmokers.
- Lower body mass index (BMI): Patients with a lower BMI show poorer prognosis than those with higher BMI. Low BMIs may indicate malnutrition or elevated basal energy expenditure.
- More severe physiologic impairment: Physiological parameters that are determined while conducting lung function tests include FVC (forced vital capacity), TLC (total lung capacity), and DLCO (diffusing capacity of the lungs for carbon monoxide). A progressive decline in these parameters indicate a decline in lung function and is associated with a poorer prognosis. A composite physiologic index based on measures of FVC, TLC, and DLCO is used to estimate prognosis.
- Dyspnea scores: Significant changes in chronic dyspnea (shortness of breath) scores are an indicator of declining lung function and poorer prognosis.
- Greater radiologic extent: The extent of fibrosis as measured by high-resolution computed tomography (HRCT) indicates the degree of disease progression. In general, a UIP (usual interstitial pneumonia) pattern on HRCT images is associated with poorer prognosis.
- Other co-existing diseases: The development of other medical complications such as pulmonary hypertension, emphysema, bronchogenic cancer, and gastroesophageal reflux disease can exacerbate pulmonary fibrosis and worsen prognosis.
Last updated: Oct. 6, 2019
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