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What Are the Signs and Symptoms of Idiopathic Pulmonary Fibrosis?

The signs and symptoms of idiopathic pulmonary fibrosis (IPF) develop over time. They may not even begin to appear until the disease has done serious damage to your lungs. Once they occur, they're likely to get worse over time.

The most common signs and symptoms are:

  • Shortness of breath. This usually is the main symptom of IPF. At first, you may be short of breath only during exercise. Over time, you'll likely feel breathless even at rest.
  • A dry, hacking cough that doesn't get better. Over time, you may have repeated bouts of coughing that you can't control.

Other signs and symptoms that you may develop over time include:

  • Rapid, shallow breathing
  • Gradual, unintended weight loss
  • Fatigue (tiredness) or malaise (a general feeling of being unwell)
  • Aching muscles and joints
  • Clubbing, which is the widening and rounding of the tips of the fingers or toes


The illustration shows a cross-section of a healthy heart, including the four heart valves. The blue arrow shows the direction in which oxygen-poor blood flows from the body to the lungs. The red arrow shows the direction in which oxygen-rich blood flows from the lungs to the rest of the body.

The illustration shows clubbing of the fingertips associated with idiopathic pulmonary fibrosis.

IPF may lead to other medical problems, including a collapsed lung, lung infections, blood clots in the lungs, and lung cancer.

As the disease worsens, you may develop other potentially life-threatening conditions, including respiratory failure, pulmonary hypertension, and heart failure.

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September 20, 2011