Pulmonary Fibrosis

Overview

Pulmonary fibrosis means scarring of the lung. Many diseases can cause pulmonary fibrosis including infections, radiation therapy, certain medications, and some diseases such as rheumatoid arthritis, scleroderma, mixed connective tissue disease, and systemic lupus erythematosus. The most common cause of pulmonary fibrosis is idiopathic pulmonary fibrosis (pulmonary fibrosis of unknown cause).

Symptoms and Diagnosis

Most patients with pulmonary fibrosis will first have inflammation. Evidence suggests that inflammation contributes to the scarring process. Once scarring sets in, medications can do very little for the disease. The approach in the medical treatment of pulmonary fibrosis is to stop the inflammation in hopes of stopping future scarring, although new medications are focused at attacking the cells producing the scar. The most common symptoms of pulmonary fibrosis are shortness of breath, particularly with activities, and cough. The fibrosis is usually first suspected by your physician when he or she hears "crackles" when listening to your lungs (sounds which are similar Velcro being torn apart) or when he or she sees scarring on a chest x-ray.

To confirm a diagnosis of pulmonary fibrosis, a high resolution chest CT scan is often performed as it can sometimes determine the cause of the fibrosis and it can also lead the lung specialist to the best part of the lung for biopsy. The next step is frequently a bronchoscopy with bronchoalveolar lavage and transbronchial biopsy. Bronchoalveolar lavage is a technique where the lung is rinsed out and the rinsings are analyzed in the laboratory for the type of inflammation present, evidence of cancer cells, and evidence of certain infections. A transbronchial biopsy is performed through the bronchoscope and yields small, pinhead-sized pieces of lung, which can be looked at under the microscope to determine the cause of fibrosis. All in all, high resolution CAT scan and bronchoscopy will yield a firm diagnosis in less than half of the cases. If the diagnosis remains uncertain, then an open lung biopsy is often performed. This is done by being admitted to the hospital, taken to the operating room, being put under general anesthesia, and having an incision made by a surgeon in the chest to remove thumbnail-sized pieces of lung for microscopic analysis.

To determine the stage of the pulmonary fibrosis, pulmonary function studies, and sometimes pulmonary exercise tests are performed to determine how severely the fibrosis is affecting your lung function. Pulmonary function studies are frequently performed periodically to determine whether the lungs are responding to treatment.

Treatment and Prognosis

The treatment of pulmonary fibrosis varies depending on the cause of pulmonary fibrosis. For the form of idiopathic pulmonary fibrosis called "usual interstitial pneumonitis" (the most severe form of pulmonary fibrosis), we often start with prednisone (an oral corticosteroid medication) and/or cyclophosphamide or azathioprine. These medications are usually given a trial of three to six months and if there is no benefit, then they are discontinued. Colchicine can also be used but in our experience is less effective than the combination of prednisone and cyclophosphamide. The advantage of Colchicine is that it has very few side effects compared to prednisone and cyclophosphamide. N-acetylcysteine is an over-the-counter medication taken 3 times a day that may be helpful in some patients with usual interstitial pneumonitis, particularly if coupled with prednisone and azathioprine.

If the pulmonary fibrosis continues to advance despite medical therapy, lung transplantation is often considered. Persons who are candidates for lung transplant from pulmonary fibrosis are generally those who are under the age of 65 and who have developed the need for supplemental oxygen. As the scarring advances, the lung's ability to transport oxygen from the air we breathe into the blood stream becomes diminished and supplemental oxygen is often necessary. Initially, this usually means oxygen to use with exercise but can advance to the point that oxygen is required at rest as well.

Certain types of pulmonary fibrosis advance more slowly than others. For example, pulmonary fibrosis associated with scleroderma tends to have a more benign course and responds better to therapy; it can often be put into remission permanently. Certain types of idiopathic pulmonary fibrosis have a more favorable prognosis also. There are several types of idiopathic pulmonary fibrosis, which we define by the microscopic appearance of the lung after lung biopsy.

Interferon gamma-1b is a new drug shown to be superior to corticosteroids in preliminary studies. However its current cost (approximately $50-100,000 per year) is prohibitive for most patients unless insurance companies agree to cover the costs. We are currently performing a clinical trial of gamma interferon compared to placebo in patients with pulmonary fibrosis.

Desquamative interstitial pneumonitis has the most favorable prognosis and is almost always caused by tobacco smoke. Smoking cessation and corticosteroids generally result in complete or partial resolution. Respiratory bronchiolitis interstitial lung disease has the next most favorable outcome and is also caused by tobacco smoke. It also is generally treated with smoking cessation with or without corticosteroids. Nonspecific interstitial pneumonitis has a variable response to steroids and/or cyclophosphamide. Usual interstitial pneumonitis occasionally responds to cyclophosphamide and corticosteroids, however, it is the least responsive and often progresses despite early drug treatment. Gamma interferon as well as a number of experimental drugs offer promise for treatment in the future.

In patients who develop worsened lung function despite treatment and for those who develop a low blood oxygen requiring supplemental oxygen, referral for lung transplantation is the best alternative. There are a number of tests that must be done before a patient can be placed on a transplant list so referral is made early if further decline in lung function is anticipated by your doctor.

Living with Pulmonary Fibrosis

Patients with pulmonary fibrosis can develop a number of respiratory and nonrespiratory symptoms which can often be annoying. Cough can be very difficult to control and often requires narcotic-based cough suppressants. Esophageal reflux is common and may play a role in progression of the idiopathic pulmonary fibrosis; it is commonly treated with a class of drugs known as the proton pump inhibitors that block acid production. Depression is quite common in this disease and antidepressants are frequently used when depression is suspected. Supplemental oxygen can greatly improve exercise tolerance and can relieve the sensation of breathlessness. Because of the reduced exercise tolerance, patients often become deconditioned (out of shape), and pulmonary rehabilitation programs can be very useful in order to help you use your muscles most efficiently.

Often what patients need most is information. This includes satisfactory confirmation of the diagnosis, an honest assessment of the prognosis, and risks and benefits to be reasonably expected from treatment. In this regard, community resources such as the American Lung Association can supplement some of information provided by your physician.

For more information:

Go to the Pulmonary Fibrosis health topic, where you can:

Read articles on this topic
Browse the previously asked questions
Ask your own question.

This article is a NetWellness exclusive. NetWellness Article

Last Reviewed: Jan 20, 2006

James N. Allen, Jr., MD
Professor - Clinical
Pulmonary, Allergy, Critical Care & Sleep Medicine
Department of Internal Medicine
College of Medicine
The Ohio State University

Ruthann Kennedy, APRN
Clinical Practice Manager
Pulmonary, Allergy, Critical Care & Sleep Medicine
Department of Internal Medicine
College of Medicine
The Ohio State University