Pulmonary Fibrosis

  • Medical Author:
    George Schiffman, MD, FCCP

    Dr. Schiffman received his B.S. degree with High Honors in biology from Hobart College in 1976. He then moved to Chicago where he studied biochemistry at the University of Illinois, Chicago Circle. He attended Rush Medical College where he received his M.D. degree in 1982 and was elected to the Alpha Omega Alpha Medical Honor Society. He completed his Internal Medicine internship and residency at the University of California, Irvine.

  • Medical Author: John P. Cunha, DO, FACOEP
    John P. Cunha, DO, FACOEP

    John P. Cunha, DO, is a U.S. board-certified Emergency Medicine Physician. Dr. Cunha's educational background includes a BS in Biology from Rutgers, the State University of New Jersey, and a DO from the Kansas City University of Medicine and Biosciences in Kansas City, MO. He completed residency training in Emergency Medicine at Newark Beth Israel Medical Center in Newark, New Jersey.

  • Medical Editor: Melissa Conrad Stöppler, MD
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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
  • 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.

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
    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 DLCO,).
    • The GAP index uses these variables to categorize patients in three different stages, which estimate the individual risk for patients.
Medically Reviewed by a Doctor on 9/14/2016

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