Idiopathic Pulmonary Fibrosis(IPF)

What Is Idiopathic Pulmonary Fibrosis?

Idiopathic Pulmonary Fibrosis (IPF) is a condition where the lungs become progressively scarred.

Many clinical and radiological syndromes are described on the basis of the pattern of involvement of the lungs.

It is rare before the age of 50; usually the disease starts after age 60 and is most prevalent in the age group after 70 – 75 years.

It is hitherto unknown what causes the disease but several hypotheses including immune mechanism to lungs and environmental toxins have been postulated.

What Are The Causes Of Idiopathic Pulmonary Fibrosis?

In IPF the tiniest air sac of the lungs called alveoli get progressively damaged and eventually get scarred.

The lung therefore gets progressively stiff. It becomes difficult for Oxygen to pass from the lungs to the blood stream through the damaged alveoli.

It also becomes more and more difficult for the lungs to expand during breathing as it becomes scarred and stiff. As a result patients start to get breathless on exertion.

The cause of IPF, i.e. what causes the process of damage in the alveoli is as yet unknown. Several mechanisms are postulated and are believed to be associated with the disease.

However a direct causal relationship has not been established.

The risk factors based on the Postulated mechanisms are:

  • Viral infections of the lungs.
  • Exposure to a toxin like metal or wood dust.
  • Autoimmune mechanism i.e. the body’s own immune system does not recognize the lungs and causes damage to it.
  • Familial/genetic.
  • Gastro esophageal reflux disease
  • Smoking

 

As detailed above the reason as to why the process of damage begins, why it happens after a certain age and whether one of the factors above directly causes IPF is not known.

Stages Of Idiopathic Pulmonary Fibrosis (IPF)

There are several ways IPF has been staged as per several authorities and various research protocol. The most practical one is the one which guides treatment. This is done on the basis of Forced Vital Capacity (FVC), a parameter measured in Lung function tests.

Patients with FVC < 50% of predicted are probably in mild to moderate phase of the disease. There is good evidence for novel agents in this stage of disease which shows that they are effective in slowing down the progression of the disease.

Patients with FVC > 50% of predicted are in advanced or severe phase of disease. The evidence for novel agents is not as robust in this stage of disease, but general consensus at this stage is that they are probably effective.

Diagnosis Of Idiopathic Pulmonary Fibrosis

Diagnosis of IPF is based on a combination of clinical history & examination, laboratory tests, tests of lungs and its functions and radiological investigations.

  • Clinical Examination: General examination may reveal anaemia and clubbed fingers.

Examination of the lungs often shows reduced expansion capacity and added sounds called crepitations on auscultation with a stethoscope.

The crepitations are typically heard at the base of the lungs and at the end of inspiration.

 

  • Laboratory Investigations: Common laboratory investigations may show anaemia (low Haemoglobin).

 

  • Arterial Blood Gases: These tests can be done at rest and after walking for a few minutes to see whether your lungs are transferring oxygen normally to the blood stream.

 

  • Lung/Pulmonary Function Tests or Spirometry: The patient is asked to blow into a pipe and the machine/computer analyses amounts of air breathed in and out and along with some other calculations can analyse whether the lung is functioning within its normal limits.

If the result is abnormal it can also be predicted what kind of lung disease is causing the problem by looking at the pattern of the abnormality.

 

  • CXR: This may show white opacities suggestive of scarring of lungs.

 

  • HRCT (High Resolution CT) Chest: This is the most sensitive and useful radiological investigation that is able to identify lung fibrosis, scarring, damage to the alveoli and also the areas and pattern of lung damage being encountered.

 

  • Bronchoscopy: This test involved putting a small tube down the airway into the lungs and collecting fluid or taking a small biopsy. It is not needed commonly in the diagnosis of IPF and is only recommended if above tests fail to identify a diagnosis.

 

  • Lung Biopsy: A small lung tissue may be biopsied from the outside guided by a CT scan. This is again rare to use in the diagnosis of IPF and is only recommended if other common tests fail to get a diagnosis.

Treatment Of Idiopathic Pulmonary Fibrosis

Currently there is no treatment to cure IPF. The treatments available aim to reduce the progression of the disease or relieve symptoms.

Medicine: There are 2 medicines currently available which has been shown to be of some value in IPF and is able to slow down the progression of the disease.

They are useful once the function of the lungs have gone down to 50 -80% of normal.

The medicines are called Pirfenidone and Nintedanib. In addition N-Acetylcysteine, a medicinal product with antioxidant properties have shown some evidence to be effective in IPF, although this is controversial.

 

Pulmonary Rehabilitation: Several programs with breathing exercises and physical activity can increase the capacity of the lungs and improve symptoms of breathlessness.

 

Oxygen Therapy: IPF causes the levels of Oxygen in the blood stream to fall resulting in breathlessness. In advanced stages of disease therapy with oxygen can improve oxygen levels in the blood and improve symptoms.

 

• Self Care: There are several things the patients can do to improve their symptoms. These include

  1. Regular exercise
  2. Stopping smoking
  3. Eating a healthy diet
  4. Taking yearly influenza vaccinations
  5. Taking precaution to stay away from people with chest infections.

 

Lung Transplant: Lung transplant can significantly improve life expectancy in patients with IPF. However it’s major operation of significant undertaking.

The success of the procedure depends on the patient’s general health and condition of the lungs. Donor lungs compatible to the patient’s body have always been scarce to find.

 

• Palliative care: IPF can sometimes be very aggressive and not respond to any forms of treatment. Sometimes there is nothing more for doctors to do other than offer symptom control through palliative care.

FAQs On Idiopathic Pulmonary Fibrosis (IPF)

 

1. Is Idiopathic Pulmonary Fibrosis (IPF) Curable?

Ans: Currently there are no agents that can cure IPF. Novel agents are now available which can slow down the progression of the disease.

The effect of these drugs over long term survival is as yet unknown.

Studies are ongoing on use of combination of these agents and novel agents and we may know more in the future.

Lung transplant is an option in patients and if complication free can potentially get rid of the disease.

 

2. What is the Prognosis Of Idiopathic Pulmonary Fibrosis (IPF)?

Ans: At present Idiopathic Pulmonary Fibrosis (IPF) does not have a treatment that can cure the disease.

Although it will usually not be a threat to life immediately, unfortunately the lung function does deteriorate slowly over time leading to respiratory failure and death.

One situation which significantly reduces survival and can be lethal is acute exacerbation (severe infection with respiratory failure including or not including mechanical ventilation).

Median life expectancy for IPF varies between 2 to 5 years with average being 4 years. However there is considerable variability in these figures and it must be acknowledged that these are average figures.

Several patients with IPF has lived long beyond 10 years.

Several factors like age, male gender, baseline lung function and its trajectory of decline over 6 – 12 months etc. are predictors of survival and life expectancy.

Novel agents are now available which can slow down the progression of the disease. The effect of these drugs over long term survival is as yet unknown.

Lung Transplant is an option in IPF.

 

4. Is idiopathic pulmonary fibrosis(IPF) fatal?

At present IPF does not have a treatment that can cure the disease.

Although it will usually not be a threat to life immediately, unfortunately the lung function does deteriorate slowly over time leading to respiratory failure and death.

One situation which significantly reduces survival and can be lethal is acute exacerbation (severe infection with respiratory failure including or not including mechanical ventilation).

Novel agents are now available which can slow down the progression of the disease. The effect of these drugs over long term survival is as yet unknown.

 

5. Is Idiopathic Pulmonary Fibrosis (IPF) a terminal illness?

IPF will not be a lethal condition to start with. However over time the lung function gradually deteriorates to cause respiratory failure and death.

Median life expectancy for IPF varies between 2 to 5 years with average being 4 years.

However there is considerable variability in these figures and it must be acknowledged that these are average figures.

Several patients with IPF has lived long beyond 10 years.

Several factors like age, male gender, baseline lung function and its trajectory of decline over 6 – 12 months etc. are predictors of survival and life expectancy.

Novel agents are now available which can slow down the progression of the disease. The effect of these drugs over long term survival is as yet unknown.

 

6. What Is The Life expectancy of IPF?

Median life expectancy for IPF varies between 2 to 5 years with average being 4 years.

However there is considerable variability in these figures and it must be acknowledged that these are average figures.

Several patients with IPF has lived long beyond 10 years.

Several factors like age, male gender, baseline lung function and its trajectory of decline over 6 – 12 months etc. are predictors of survival and life expectancy.

Novel agents are now available which can slow down the progression of the disease. The effect of these drugs over long term survival is as yet unknown.

Lung transplant remains an option in IPF.

 

7. Idiopathic Pulmonary Fibrosis (IPF) vs Pulmonary Fibrosis (PF)

Ans: Pulmonary Fibrosis is essentially inflammation and scarring of the lungs which can happen from various causes.

Some of them are associated with other connective tissue diseases like Rheumatoid Arthritis and SLE.

However a group of these diseases happen primarily in lung and is not associated with any other disease or cause. These are called Idiopathic Interstitial Pneumonia (IIP).

There are several forms of IIP which has different radiological and tissue/histological characters and affects different patterns of areas in lungs.

Idiopathic Pulmonary Fibrosis (IPF)/Usual Interstitial Pneumonitis (UIP) is the most common form of IIP which principally affects peripheral, basal parts of lug and has characteristic features in histology from lung biopsy if performed.

Previously it was called Cryptogenic Fibrosing Alveolitis (CFA)

 

Have any more question about this disease?Drop an email to [email protected]

Dr. Dwaipayan Sen (FRCP(Glasgow) MRCP Geriatrics)


Consultant Stroke Physician

Dr. Dwaipayan Sen is a Consultant Stroke Physician and Clinical Lead for Comprehensive Stroke Services, Manchester Centre for Clinical Neurosciences.

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