Pulmonary Fibrosis

Alternative names: Idiopathic Pulmonary Fibrosis

Pulmonary Fibrosis is a serious, progressive disease of the lungs in which lung tissue becomes scarred and thickened, resulting in increasingly impaired lung function and difficulty breathing.

Incidence; Causes and Development; Contributing Risk Factors

Pulmonary Fibrosis usually affects those over the age of 50, men more often than women, and smokers much more often than non-smokers.  Overall, this disease affects about 1 person in every 5,000.

There are many possible causes of the scarring that causes Pulmonary Fibrosis, but in any particular case the specific cause is usually unknown.  Causes include:

• exposure to various airborne substances such as chemicals, gas, asbestos, animal proteins, sawdust, or coal dust
• other medical conditions such as Lupus or Pneumonia
• side-effects from radiation therapy for lung or breast cancer
• certain medications such as chemotherapy drugs or certain antibiotics

Research is suggesting that development of the disease my be triggered by tobacco smoke or certain viral infections.

This scarring occurs between the air sacs in the lungs, making it more difficult for inhaled oxygen to pass from the lungs into the bloodstream.

People who work in jobs that involve exposure to airborne particles or toxins are at risk, as well as those who take certain medications.  Genetics also plays a role, with about 1-in-20 patients having another family member with the condition.

Diagnosis and Tests

Diagnosis involves discussing recent symptoms, reviewing the patient's medical and other history to look for possible risk factors, listening to the lungs with a stethoscope as the patient breathes in and out.  If Pulmonary Fibrosis is suspected then various diagnostic tests can be performed:

• X-ray or high resolution CT Scan to look for scar tissue and lung damage
• Lung function testing to see how much air the lungs can hold and how fast they can move air in and out (spirometry)
• Lung function testing to see how fast they can move (diffuse) oxygen into the bloodstream
• Lung function testing to see how well they perform during activity (exercise stress test)
• Heart function tests such as an echocardiogram to monitor pressure inside the heart
• Pulse oximetry – a small device placed on the end of a finger to measure the blood's oxygen saturation
• Testing a sample of blood for oxygen and carbon dioxide levels (arterial blood gas test)
• Finally, if other tests are inconclusive, a lung tissue biopsy

Treatment and Prevention

The lung damage that occurs as a result of Pulmonary Fibrosis cannot be reversed or repaired, leaving a lung transplant as the only real treatment option.  Unfortunately, donor lungs are rare.  Medications can be used to help reduce the symptoms and slow their progress.

Commonsense lifestyle changes include stopping smoking, eating healthy foods, and exercising regularly, as much as the condition permits.

Patients with rapidly worsening symptoms, such as severe shortness of breath, may receive supplemental oxygen to help with breathing (oxygen therapy), or be placed on a mechanical ventilator and given antibiotics or corticosteroids to reduce inflammation.  Azathioprine and cyclophosphamide also reduce inflammation by suppressing the immune system.  Pirfenidone and Nintedanib reduce the rate of scarring.

New drugs are being developed over time in order to improve the lives and life expectancy of patients.

Prognosis; Complications; Seek medical attention if...

The prognosis varies by person, but life expectancy after diagnosis is usually around 3 to 5 years.  Some people become very ill very quickly, while others have less severe symptoms that worsen slowly.  In some cases, symptoms can remain the same for years.

Possible complications include:

• Serious lung problems such as blood clots, collapsed lung, lung infection, or lung cancer
• Respiratory failure
• Chest infections
• Heart failure and/or pulmonary hypertension, caused by having to pump harder to force blood through narrowed pulmonary arteries

See a doctor if you experience unexplained breathlessness or have had a cough for more than 3 weeks.

People often ignore breathlessness, putting it down to getting old or being out of shape.  As Pulmonary Fibrosis progresses, even light activity (or no activity at all) can cause shortness of breath.  Breathlessness is not normal, except after significant exercise.