Pulmonary fibrosis is a disease that causes scarring and damage to lung tissue. Over time, this damage causes breathing difficulty.
Many health conditions can cause pulmonary fibrosis. One of them is rheumatoid arthritis (RA). RA causes inflammation and pain that affects the joints, but it can also affect other organs, like your lungs.
Up to 40 percent of people with RA have pulmonary fibrosis. In fact, breathing problems are the second leading cause of death in people who have RA. But experts still don’t understand exactly the link between RA and pulmonary fibrosis.
Always mention symptoms of discomfort to your doctor, even if breathing troubles only occur during exercise. According to the Arthritis Center, people with RA often underreport breathing problems. This is usually because people with RA are less physically active due to joint pain.
While treatment for RA has improved, treatment for lung disease has not. The goal of treatment is early stage intervention to slow the progression of the disease and improve quality of life.
The most notable symptom of pulmonary fibrosis is shortness of breath. But this symptom doesn’t often appear until the disease has progressed.
Other symptoms of pulmonary fibrosis include:
- a dry, hacking cough
- unintentional weight loss
- widening and rounding of the tips of the fingers or toes
- feeling tired
Shortness of breath may be mild at first and only occur during physical activity. Breathing problems will gradually worsen over time.
The cause of pulmonary fibrosis is unknown, but RA can increase your risk for it because of the inflammation. Research also shows that high counts of RA antibodies are linked to the development of interstitial lung disease (ILD). ILD is the most common lung disease that’s associated with RA. It’s a serious and life-threatening condition that can develop into pulmonary fibrosis.
Other factors can increase your risk for pulmonary fibrosis, including:
- cigarette smoking and exposure to environmental pollutants
- viral infections
- use of medications that damage the lungs (chemotherapy drugs, heart medications, and certain anti-inflammatory drugs)
- a family history of pulmonary fibrosis
- a history of a gastroesophageal reflux disease
You may also develop pulmonary fibrosis if you have a medical condition that damages your lungs, such as polymyositis, sarcoidosis, and pneumonia.
During your visit, your doctor will ask about your symptoms, review your medical and family history, and conduct a physical exam to listen to your breathing. There are also several tests they can do to see if you have pulmonary fibrosis. These tests include:
Imaging tests: A chest X-ray and CT scan can show scarred lung tissue. An echocardiogram can be used to check for abnormal pressures in the heart caused by pulmonary fibrosis.
Pulmonary function testing: A spirometry test shows your doctor the amount of air you can hold in your lungs and the way in which the air flows in and out of your lungs.
Pulse oximetry: A simple test that measures the amount of oxygen in your blood.
Arterial blood gas test: This test uses a sample of your blood to measure oxygen and carbon dioxide levels.
Biopsy: Your doctor may need to remove a small amount of lung tissue to diagnose pulmonary fibrosis. This can be done through a bronchoscopy or a surgical biopsy. A bronchoscopy is less invasive than a surgical biopsy, which is sometimes the only way to get a large enough tissue sample.
Blood tests: Your doctor may use blood tests to see how your liver and kidneys are functioning. This also helps rule out other possible conditions associated with lung disease.
Diagnosing and treating pulmonary fibrosis early is important because of the risks and complications. Pulmonary fibrosis can cause:
- a collapsed lung
- right-sided heart failure
- respiratory failure
- high blood pressure in your lungs
Ongoing pulmonary fibrosis can also increase your risk for lung cancer and lung infections.
Lung scarring from pulmonary fibrosis isn’t reversible. The best therapy is to treat underlying RA and slow the progression of the disease. Treatment options to improve your quality of life include:
- medications such as corticosteroids and immunosuppressants
- oxygen therapy to improve breathing and reduce the risk of complications
- pulmonary rehabilitation to strengthen the lungs and improve symptoms
If your condition is severe, your doctor may recommend an evaluation for a heart-lung transplant to replace your damaged lungs and heart with those from a healthy donor. This procedure can improve breathing and your quality of life, but there are risks with transplantation. Your body may reject the organ, or you may develop an infection due to the immunosuppresant drugs. You will have to take these drugs for the rest of your life to decrease the risk of rejection.
In addition to these treatment options, you’ll want to keep your lungs as healthy as possible. To slow the progression of the disease, it’s important to quit smoking and avoid secondhand smoke or any pollutants that irritate your lungs.
Regular exercise can also improve lung function. Ask your doctor about safe exercises, such as walking, swimming, or biking. You should get an annual pneumonia vaccine and flu shot to lower your risk of infections. If you find that breathing problems worsen after meals, eat smaller, more frequent meals. Breathing is often easier when your stomach isn't full.
A pulmonary fibrosis diagnosis can bring feelings of depression and anxiety. Ask your doctor about local support groups. Sharing your story with people who understand the experience may help. Support groups are also good places to learn about new treatments or coping methods to manage stress.
The outlook and rate of progression for pulmonary fibrosis and RA varies for each person. Even with treatment, pulmonary fibrosis continues to worsen over time. The average survival rate of people with RA who develop ILD is 2.6 years, according to a study in Arthritis & Rheumatism. This may also be because ILD symptoms don’t appear until the disease has progressed to a serious stage.
There's no way to know with certainty how fast the disease will progress. Some people have mild or moderate symptoms for many years and enjoy a relatively active life. Be sure to listen to your doctor and stick with a treatment plan.
Remember to mention dry coughs or breathing difficulties to your doctor. The earlier you treat ILD, the easier it is to slow the progression of the disease.