What are IPF and COPD?

Idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD) are both chronic and disabling lung diseases that cause shortness of breath. But IPF and COPD cause different forms of physical damage to your lungs.

In IPF, your lungs become scarred, stiff, and thick, and the progressive damage isn’t reversible. In COPD, the airways and air sacs in your lungs become blocked, but you can control the symptoms even in advanced cases of the disease. The two most common forms of COPD are emphysema and chronic bronchitis.

Both IPF and COPD benefit from early diagnosis. Overall, IPF has a very poor prognosis, with an average survival time of only two to three years after diagnosis. But some people live longer and early treatment can help extend your life. COPD is treatable, with better results if you catch it early. Survival times vary, depending on the severity of the disease, your general health, and your history of smoking.

Prevalence

IPF is a rare disease, affecting an estimated 100,000 people in the United States, with 34,000 new cases diagnosed each year. COPD is much more prevalent and is considered to be a major U.S. medical problem. About 30 million people in the United States have COPD. According to some estimates, it affects about 20 percent of adult Americans.

According to the Centers for Disease Control and Prevention, COPD is the third leading cause of death in the United States. IPF, although rare, is ranked as “seventh on a list of fatal malignancies” according to a 2015 review article.

Causes

The cause of IPF is unknown and the course of the disease is not predictable. In contrast, about 90 percent of COPD cases are caused by smoking and the course of the disease is well studied. IPF is often not diagnosed until permanent lung scarring is present. Many people with COPD have only mild symptoms and aren’t diagnosed until their disease has progressed further.

IPF risk factors

Although the cause of IPF is unknown, several risk factors are associated with the disease:

  • Smoking.
  • Age. About two-thirds of patients with IPF are more than 60 years old when they’re diagnosed.
  • Occupations that involve working around dust, chemicals, or fumes. The Cleveland Clinic notes that farmers, ranchers, hairdressers, and stonecutters have a “moderately increased risk” of developing IPF.
  • Sex. More men than women are diagnosed with IPF.
  • Family history of IPF. Genetic factors are thought to play a role.
  • Radiation treatments for the chest. Radiation therapy near the chest, like that used to treat breast and lung cancers, can lead to scar tissue in the lung.
  • Certain medications. This includes the chemotherapy drugs methotrexate, bleomycin, and cyclophosphamide, as well as some heart medications and antibiotics.

COPD risk factors

The risk factors for COPD are similar to those for IPF:

  • Smoking. Long-term smoking is responsible for 90 percent of COPD cases. This includes pipe, cigarette, and marijuana smokers. Long-term exposure to secondhand smoke is also a risk. People with asthma who smoke have an even higher risk.
  • Age. Most people are about age 40 when they first notice symptoms of COPD.
  • Occupations that involve working around dust, chemicals, or fumes.
  • Sex. Women nonsmokers are more likely to have COPD. A 2007 review suggests that women are more susceptible to physical damage from smoke.
  • Family history of COPD. A rare genetic disorder called alpha-1-antitrypsin deficiency is the cause of about 1 percent of COPD cases. Other genetic factors may also be involved.

Signs and symptoms

IPF and COPD share many signs and symptoms:

  • A primary symptom of both diseases is shortness of breath, which gets progressively worse.
  • Both diseases involve a chronic cough. In IPF, the cough is dry and hacking, while in COPD there’s mucus production and wheezing.
  • Both diseases are marked by fatigue. This is caused by the difficulty of getting oxygen into the blood and carbon dioxide out of the blood.
  • Both diseases can affect your fingertips. In IPF, your fingertips and nails may be enlarged, called clubbing. In COPD, your lips or fingernail beds may turn blue, called cyanosis.
  • Both diseases are made more serious by respiratory infections such as the flu.
  • When severe, both diseases can lead to unintended weight loss because eating becomes difficult.
  • COPD can also involve chest tightness and swelling in your ankles, feet, or legs.

Treatments

There’s currently no cure for IPF or COPD. Treatment aims to alleviate symptoms.

A first step in treatment for both IPF and COPD is for smokers to stop smoking. Another immediate step is to remove air pollutants from the home or workplace. Also, be sure to keep your vaccinations up-to-date to help avoid flare-ups and complications from infection.

Drugs

In the past, anti-inflammatory drugs were prescribed for IPF because it was thought, wrongly, that inflammation led to the lung scarring. These drugs were not effective. Now, researchers are investigating other potential causes, along with specific drugs to target these causes. The lung scarring in IPF cannot be reversed.

COPD treatment includes prescription drugs that relieve the inflammation around the airways to make breathing easier and to help prevent complications from developing.

Bronchodilators can help relax the muscles around your airways. These drugs are used with an inhaler device and can be short-acting or longer-acting, depending on your needs. They can also be combined with inhaled steroids, depending on the severity of the symptoms. Oral steroids are prescribed for short-term use only.

Oxygen therapy

Supplemental oxygen from a small portable oxygen tank is used as a treatment for both IPF and COPD. The oxygen is supplied through a tube or a face mask and helps you breathe more comfortably while you carry out normal daily activities and while you sleep. It can also allow you to exercise. If you have COPD, you may not need the oxygen supplement all the time.

Pulmonary rehabilitation

Pulmonary rehabilitation is a group of programs to help you cope with IPF or COPD. It involves exercises in breathing and stress reduction. It also may include nutritional and psychological counseling and disease management. The goal is to help you stay active and keep up with daily activities. If your disease is very severe, you may need help at home with your daily routine.

Lung surgery

A lung transplant is a possibility for those with either IPF or COPD. It can extend your lifespan, but it also has risks. Depending on the type of lung damage, other surgeries are possible for COPD. In a bullectomy, enlarged air spaces in the air sacs, called bullae, can be removed to help you breathe. For some people with COPD, lung volume reduction surgery can remove damaged tissue from the lungs to improve breathing.

Outlook

Both IPF and COPD are life-threatening diseases with severe discomfort and physical and emotional challenges. Early detection is key. If you have any of the signs or symptoms, it’s important to get screened. Once you’re diagnosed, stick to your treatment plan, including exercising as prescribed, which will help extend your lifespan.

Join a support group where you can discuss the problems of IPF or COPD and find resources to help you cope. Support groups can also alert you to any new developments in treatment. Research is ongoing for both diseases to find new medications and possible ways of preventing the disease.