Idiopathic Pulmonary

Fibrosis

What is Idiopathic Pulmonary Fibrosis?• Idiopathic Pulmonary Fibrosis (IPF) is a disease in which

inflammation of Lung parenchyma results into fibrosis. With the progression of disease, fibrosis builds up to the extent that the lungs are unable to provide oxygen exchange.

• Idiopathic means unknown.• There are however a couple of theories as to its origin. Some

researchers believe that IPF may result from either: 1) an autoimmune disorder; or 2) the chronic sequel of some infection, most likely a virus.Heredity may play a part, possibly making some individuals more likely than others to get IPF.

Epidemiology • IPF affects about 128,100 people in the United States.• 48,000 new cases diagnosed annually. • 40,000 people die each year to IPF, the same as to breast cancer.• There is no known cause, no FDA approved treatments and no

cure for IPF. • Can affect anybody at any age, most common between 50-70

years • IPF is one of about 200 disorders called interstitial lung diseases

(ILDs). IPF is the most common form of ILD• About two-thirds of IPF patients pass away within five years.• IPF occurs more often in men than women. Men tend to be

diagnosed at a later stage in the disease.• A recent study found that IPF may be 5 to 10 times more

prevalent than previously thought.

Causes of IPF• The exact cause is not known hence the term idiopathic. • Inflammation within the alveoli leading to fibrosis is

considered a mechanism.• However, anti-inflammatory treatments are not always

effective. • The current thinking is that somehow the cells that line the

alveoli are damaged in some way. The cells then try to heal themselves. But, this healing process becomes out of control, causing thickening and damage to the walls of the alveoli, and fibrosis (scarring) of the alveoli and lung tissue. The thickening and scarring reduces the amount of oxygen that can pass into the blood vessels from affected alveoli. Therefore, as the disease progresses, less oxygen than normal is passed into the body when you breathe.

Potential triggers of IPF

These include:• Cigarette smoking. The disease is more common in

people who smoke or have been smokers at some time.• Viral infections, such as the Epstein-Barr virus and

hepatitis C virus.• Certain medicines.• Pollutants in the environment.• GERD • Familial tendencies• Occupational exposures

Clinical Features of IPF

• Breathlessness.• Initially exertional• Can affect day-to-day activities such as showering, climbing stairs, getting

dressed and eating. • With progression gets worse, and may prevent any activity.• dry hacking cough, usually lasting longer than 8 weeks

• Chest pain or tightness• Unexplained weight loss• Loss of appetite• Tiredness and loss of energy• Clubbing• Cyanosis

Diagnosis

• Clinical features• X-ray chst • CT Scan• HRCT• Lung biopsy• Bronchoscopy• Echocardiogram

Treatment

• Medications are the primary form of treatment. They are usually prescribed for at least 3 months before an assessment of effectiveness is determined, usually by PFT’s and Chest X-rays. This gives your physician time to see if a particular treatment is effective.

• Prednisone is the drug of choice but other medications can also be used if prednisone is contraindicated. Oxygen is sometimes prescribed in addition to medication if a patients blood oxygenation becomes too low.

• Regular exercise may be useful for some patients with IPF. This can help in improving "muscle" strength and breathing ability and also increase overall strength.

• Lung transplantation is an alternative in some who have progressive disease despite treatment and in the severe, final stages of IPF.

• Living with Idiopathic Pulmonary Fibrosis!• In addition to getting proper treatment, IPF patients just need to follow sensible

healthy lifestyles which include: regular exercise, eat a healthy diet, maintain proper weight, get enough rest and above all Do Not Smoke.

Complications

Prognosis

Prognosis • Disease progression vary greatly. • Some respond to treatment thus may have slowing of

progression.• Others not respond much.• Some remain stable for long periods other deteriorate

rapidly.• So very difficult to predict which patient will go which way.• Lung transplantation has been shown to improve survival

in those people for whom it is suitable and so is being increasingly used as a treatment.

• Newer treatments with medicines may also be shown to be of benefit in the future.

HELPING TIPS FOR IPF PATIENTS• Work with your health-care team to manage and

understand your IPF• Make changes to your lifestyle to stay healthy, and

fight germs to prevent infections• Quitting smoking is the number one thing you can do

to help slow down the progression of any lung disease.• Get a flu shot each year.• Ask your doctor about getting a pneumonia shot.• Keep away from people with chest infections and colds.• Wash your hands properly.• Stay as fit as you can.• Home oxygen

HELPING TIPS FOR IPF PATIENTS• Take IPF medications to prevent additional scaring

and lessen symptoms:Corticosteroid, immunosuppresants, Acetylcysteine, and Pirfenidone to slow the progression of fibrosis.

• Join a pulmonary rehabilitation program• Pulmonary rehabilitation is an exercise and

educational program designed just for patients with lung disease, to teach them, how to breathe more efficiently and to perform their activities of daily living with less breathlessness.

• Talk to your doctor about the option of a lung transplant