What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF) is an incurable lung disease in which fibrosis, or scarring, occurs in the lungs making them thick and stiff. As lung tissue thickens, the lungs have difficulty moving oxygen into your bloodstream, resulting in a decreased amount of oxygen to your brain and other organs. Sometimes doctors can determine what’s causing IPF. When the cause is unknown, they call it idiopathic.^[1]

Known Causes of Pulmonary Fibrosis

Known causes of pulmonary fibrosis include:¹

• Exposure to environmental pollutants including silica, hard metal dusts, bacteria and animal proteins.
• Certain medicines such as nitrofurantoin (an antibiotic), amiodarone (a heart medicine), methotrexate and bleomycin (both chemotherapy medicines) and a wide variety of other medications.

Risk Factors for IPF

You’re more likely to get IPF if you:¹

• Smoke cigarettes.
• Have certain viral infections including the Epstein-Barr virus (the cause of mononucleosis), influenza A virus, hepatitis C virus, HIV and herpes virus 6.
• Have a family history of IPF.
• Have gastroesophageal reflux disorder (GERD).

Signs and Symptoms of IPF

The signs and symptoms of idiopathic pulmonary fibrosis are progressive, meaning they develop over time. Initially, you may not even notice them. Once you do, chances are serious lung damage may have already occurred. Because the disease is progressive, symptoms generally worsen over time.¹

The most common signs and symptoms of IPF include the following:¹

• Shortness of breath that initially may only occur when you exercise, but may eventually occur even at rest.
• A dry hacking cough that doesn’t go away. In time, you may experience repeated bouts of coughing that you won’t be able to control.

Other signs and symptoms that may develop gradually include:¹

• Shallow, rapid breathing
• Unintended weight loss
• Fatigue (different than ordinary tiredness) and malaise (feeling generally unwell)
• Aching joints and muscles
• Clubbing (widening and rounding) of the fingertips

Complications of IPF

As IPF worsens, it may lead to the following complications, some of which are life-threatening:¹

• Collapsed lung
• Blood clots in the lung
• Lung cancer
• Respiratory failure
• Pulmonary hypertension (high blood pressure in the blood vessels of the lungs)
• Heart failure

Treatment of IPF

There are 4 treatments used in the management of IPF including:

Medications

To date, there are no medications known to slow the progression of IPF. Prednisone (a corticosteroid), azathioprine (suppresses the immune system) and N-acetylcysteine (a cough medicine) used alone or in combination have been utilized to treat IPF, however, more research is needed to support their use.¹

Other treatments that may help people with IPF include flu and pneumonia vaccines (to prevent infections), cough medicines that include codeine (to control cough) Vitamin D, calcium and bone- building medications (to help prevent bone loss caused by corticosteroids) and anti-reflux therapies to help reduce the symptoms of GERD (because most people with IPF also have GERD).¹

Oxygen Therapy

If the level of oxygen in your blood decreases, you may need supplemental oxygen (oxygen therapy) to restore your blood oxygen level to normal. Oxygen is usually delivered through nasal prongs or an oxygen mask. Initially, you may only need supplemental oxygen during exercise or when you sleep. As your disease progresses, you may need it continuously.¹

Pulmonary Rehabilitation

Pulmonary rehabilitation (PR) is now the gold-standard of care for people who have chronic (ongoing) lung disease. PR uses a multi-disciplinary approach to treatment that usually involves a team of specialists from a wide variety of disciplines including doctors, nurses, respiratory therapists, psychologists, physical therapists, occupational therapists, dietitians and more.¹

PR is used in conjunction with your medical therapy and may include:¹

• Exercise training
• Nutritional counseling
• Education on lung disease and how to manage it
• Energy conservation techniques
• Breathing techniques
• Psychological counseling and/or group therapy
• Help with smoking cessation

Lung Transplant

If your condition progresses rapidly and is unresponsive to standard medical care, your doctor may recommend a lung transplant. A lung transplant may help you live longer and improve your quality of life. Only a select group of patients who meet very specific criteria are eligible for a lung transplant. For more information about lung transplants and to see if you qualify, talk to your primary care provider.¹

Prognosis

IPF usually affects people who are middle-aged or elderly. The progression of IPF varies from person to person. For example, in some people, fibrosis occurs rapidly. In others, the progression of IPF is much slower. Still, in others, the disease stays the same for many years.¹

Because idiopathic pulmonary fibrosis is progressive and has no cure, many people live only about 3 to 5 years after being diagnosed. But everyone’s outlook is different. Some people live 10 years after diagnosis.^[2]

Respiratory failure is the most common cause of death related to IPF. Other causes of death include pulmonary hypertension, pulmonary embolism (blood clot in the lungs), heart failure, pneumonia and lung cancer.¹

For more information about managing idiopathic pulmonary fibrosis and your own, individual prognosis, talk to your primary care provider.

_{[1] National Heart, Lung and Blood Institute. What is Idiopathic Pulmonary Fibrosis? Last updated September 20, 2011.}

_{[2] WebMD. Idiopathic Pulmonary Fibrosis. Last reviewed January 3, 2017.}