Idiopathic Pulmonary Fibrosis
The following is a brief overview of this condition. For more information, please refer to the guidelines from the For further guidelines, please refer to the British Thoracic Society and the British Lung Foundation.
What Is Idiopathic Pulmonary Fibrosis?
Pulmonary fibrosis describes a group of diseases which lead to interstitial lung damage and ultimately fibrosis and loss of the elasticity of the lungs. It is a chronic condition characterised by shortness of breath. There are more than 100 related diseases of the lung known as interstitial lung diseases (ILD), and idiopathic pulmonary fibrosis (IPF) is the most common of all the interstitial lung diseases (ILD).
IPF is a condition in which the lung tissue becomes thickened, stiff and scarred over a period of time. The development of the scar tissue is called fibrosis. As the lung tissue becomes scarred and thicker, the lungs lose their ability to transfer oxygen into the bloodstream. As a result, the brain and other organs don’t get the oxygen they need. The scarring typically starts at the edges of the lungs and progresses towards the centre making it more difficult to breathe.
What Causes IPF?
Idiopathic means unknown and hence the cause of IPF is unknown. In some patients, the disease is genetic. Environmental factors (particularly exposure to certain types of dusts) may also play a role. What is known is that IPF changes the lung’s ability to function normally. Typically, mild scarring of the lung tissue occurs first, but, over months and years, the normal lung tissue is replaced by more heavily scarred lung tissue, which makes it difficult to breathe and deliver needed oxygen to the body.
Even though the cause of IPF is still being investigated, the following are considered to be risk factors for disease development:
- Viral Infections.
- Exposure to dust / fumes and smoking.
- Age /gender (diagnosis is more common in men and older people).
- Acid reflux from the stomach into the lungs.
IPF affects each person differently and the disease progresses at varying rates. For some, the scarring occurs quickly, while in others, it happens over a longer period of time. Symptoms also vary from moderate to severe and may stay the same for a while.
What Are The Symptoms Of IPF?
Symptoms of IPF usually develop gradually and may not be noticed until the disease is well established. Because the disease may develop later in life, a common assumption is that the breathlessness is just part of middle age. If you feel breathless, you should see your doctor. Symptoms may include:
- Dry cough (can be chronic, dry, hacking coughing).
- Shortness of breath, especially during or after physical activity.
- Lasting tiredness.
- Weight loss.
- Bulb-like development of the fingertips and nails (a condition called clubbing).
How Is IPF Diagnosed?
Diagnosis of IPF can be difficult since the cause is unknown. The symptoms are similar to those of other diseases such as asthma, chronic obstructive pulmonary disease (COPD) and congestive heart failure. Additionally, IPF can co-exist with these diseases.
In order to confirm the diagnosis of IPF, doctors must rule out all known causes of fibrotic lung disease and therefore it will often take some time to explore and eliminate other possible diagnoses.
Confirming diagnosis may involve one or more of the following tests:
- Blood tests to rule out other diseases.
- Pulmonary function test to measure breathing capacity.
- X-ray and CT (“CAT”) scan of your chest to identify the pattern of scarring in your lungs.
- Lung cell sample by passing a small flexible telescope (a bronchoscope) down the breathing tubes in order to examine cells and look for signs of inflammation.
- Lung biopsy.
Sometimes, during examination of the lungs with a stethoscope, a doctor may hear crackling sounds in the chest. These crackles have a very characteristic sound. The chest X-ray may or may not be abnormal, but a high resolution CT scan will often show abnormalities. The classic findings in idiopathic pulmonary fibrosis show scarring of the lungs with small bubbles that have a characteristic appearance called “honeycomb” on the CT scan.
The lung function test may show a reduction in the volume of the lungs and the transfer factor (or diffusing capacity) is a measure of the ability of the lungs to exchange gases (oxygen and carbon dioxide) into and out of the blood stream.
In many cases, the diagnosis of IPF can be made on CT scan but, in other cases of lung fibrosis, a lung biopsy may be necessary to obtain enough tissue to make an accurate diagnosis. The biopsy specimen is examined microscopically by a pathologist to confirm the presence of fibrosis. Often, a biopsy is not necessary.
Can IPF Be Treated Or Prevented?
At this point, there is no cure for IPF which is why Breathing Matters is supporting the Centre for Respiratory Research.
The treatment options are limited since scarring is permanent once it has developed. Lung transplantation is one of the few options available. Research trials using different drugs that may reduce fibrous scarring are ongoing.
Some types of lung fibrosis may respond to corticosteroids and other medications that suppress the body’s immune system by decreasing the processes that lead to fibrosis. The goal is to decrease lung inflammation and subsequent scarring.
The side effects of treatments can be serious and a lung specialist is required to determine what treatment is appropriate.
Pulmonary fibrosis causes decreased oxygen levels in the blood which can lead to a condition known as pulmonary hypertension and patients with pulmonary fibrosis are frequently treated with supplemental oxygen to prevent heart failure.
In addition to the medicines, there are also non-drug approaches available that can help manage IPF symptoms. Pulmonary rehabilitation, often combined with oxygen therapy, may help improve the ability to function without severe breathlessness.
Pulmonary rehabilitation involves nutritional advice, exercise and breathing techniques, whilst oxygen therapy allows the patient to continue being as active as possible.
Living With IPF
Please consult your doctor before making changes to your lifestyle.
- Quit smoking to help increase oxygen levels in your blood and lower blood pressure and heart rate.
- Eat less saturated fat and more fruits and vegetables.
- Exercise to help keep your body working as efficiently as possible.
- Lose excess weight to help improve your breathing capacity.
- Avoid situations in which you might catch infections from others.
- Avoid excessive pollutants and dust.
- Try to stay mentally active.
- Seek emotional support through family, friends, healthcare providers and support groups.
- Don’t over exert yourself and ensure you get enough rest.
At UCL, we are:
- Studying the role of auto-antibodies (antibodies that the body makes against its own organs) in idiopathic pulmonary fibrosis. In particular, we are developing ways to looking for auto-antibodies that attack patients’ own blood vessels in patients with interstital lung diseases including IPF.
- Investigating the role of platelets in IPF and other ILDs. We have studies looking at whether platelets accumulate in the lungs of patients with lung fibrosis, and whether platelets are abnormally ‘sticky’ in these patients.
- Investigating the ability of special scans, called PET scans, to light up active areas of lung fibrosis to help us decide on treatment courses.
- Developing and defining the role of minimally invasive bronchoscopic cryoscopic lung biopsy in the diagnostic pathway in idiopathic interstitial lung disease.
- Investigating the effect of low oxygen concentration on white cell activation in IPF and other diseases.
- Investigating whether new drugs for IPF can be delivered directly to the lungs via inhalation and therefore avoiding toxic side-effects.