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- Patient Comments: Pulmonary Fibrosis - Treatments
- Patient Comments: Pulmonary Fibrosis - Experience
- Patient Comments: Pulmonary Fibrosis - Symptoms
- Patient Comments: Pulmonary Fibrosis - Cause
- Find a local Pulmonologist in your town
- Pulmonary fibrosis definition and facts
- What is pulmonary fibrosis?
- What is idiopathic pulmonary fibrosis?
- What causes pulmonary fibrosis?
- What are signs and symptoms of pulmonary fibrosis?
- What are the signs and symptoms of idiopathic pulmonary fibrosis?
- What are the stages of idiopathic pulmonary fibrosis?
- Which specialties of doctors treat pulmonary fibrosis?
- How is pulmonary fibrosis diagnosed?
- What is the treatment for pulmonary fibrosis?
- What about pulmonary rehabilitation for pulmonary fibrosis?
- What are the complications of pulmonary fibrosis?
- Can pulmonary fibrosis be prevented?
- What is the prognosis and life expectancy for a person with pulmonary fibrosis?
- Where can I get information to improve the quality of life for those with pulmonary fibrosis?
What are signs and symptoms of pulmonary fibrosis?
Signs and symptoms of pulmonary fibrosis include:
- Shortness of breath (dyspnea), including shortness of breath with everyday activities
- Coughing (chronic, dry, hacking cough)
- Diminished exercise tolerance
- Fast, shallow breathing
- Chest discomfort
- Muscle and joint aches
- Loss of appetite
- Unexplained/unintended weight loss
- "Clubbing" of the tips of the fingers and toes (thickening of flesh under the nails, causing a widening and rounding appearance
Symptoms vary depending on the cause of the pulmonary fibrosis. The severity of symptoms and the progression (worsening) of symptoms over time can vary.
What are the signs and symptoms of idiopathic pulmonary fibrosis?
The most common form, idiopathic pulmonary fibrosis, has a slow and relentless progression.
- Early on in the disease, patients often complain of a dry, unexplained cough.
- Often, slow and insidious onset of shortness of breath can set in.
- With time, shortness of breath (dyspnea) worsens. Dyspnea initially occurs only with activity and is often attributed to aging. Over time, the dyspnea occurs with little or no activity. Eventually, the shortness of breath becomes disabling, limiting all activity and even occurring while sitting still.
- In rarer cases, the fibrosis can be rapidly progressive, with shortness of breath (dyspnea and disability occurring in weeks to months from the onset of the disease. This form of pulmonary fibrosis has been referred to as Hamman-Rich syndrome.
What are the stages of idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis has been loosely staged and categorized as mild, moderate, or severe, or early versus advanced. The staging is usually based on pulmonary function tests, although these stages are considered arbitrary.
There are some newer proposed staging systems:
- Mortality risk scoring system: This is
based on four predictors
- recent respiratory hospitalization,
- baseline forced vital capacity (FVC, a measure of lung function), and
- 24-week change in FVC.
- Taking these factors into account gives a score, which helps predict the expected 1-year probability of death.
- The GAP index: This staging system is
based on four baseline variables
- gender (G),
- age (A) and
- two lung physiology variables (P) (FVC plus the diffusing capacity of the lung for carbon monoxide, or DLCO,).
- The GAP index uses these variables to categorize patients in three different stages, which estimate the individual risk for patients.