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
• Fatigue/tiredness
• Weakness
• 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.

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.

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
  1. age,
  2. recent respiratory hospitalization,
  3. baseline forced vital capacity (FVC, a measure of lung function), and
  4. 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
  1. gender (G),
  2. age (A) and
  3.  two lung physiology variables (P) (FVC plus the diffusing capacity of the lung for carbon monoxide, or D_LCO,).
  • The GAP index uses these variables to categorize patients in three different stages, which estimate the individual risk for patients.