Medications

Prednisone

Prednisone (a common corticosteroid medication) has been used to treat IPF/UIP for many years. Prednisone is helpful for the inflammation component of the UIP and may be used as initial treatment if there is extensive inflammation. Prednisone is also used for treatment of an acute episode or exacerbation of inflammation that may develop during the course of the illness. The prednisone dose is usually 60 mg per day with gradual reduction over time as the inflammation subsides. The amount of inflammation can be detected by the "ground glass" shadows seen in high-resolution chest CT scan, or by review of the biopsy.

Side effects and adverse reactions of prednisone can be numerous. Most are reversible, but some are not. Some individuals have no difficulty with prednisone while others may be bothered by some of adverse reactions but can tolerate them. In rare situations, an individual cannot take prednisone. This medication can save a person's life, but can also cause difficulties in some individuals. The common side effects include increased appetite, weight gain, and bruising of the skin. A rounded puffy face, acne-like skin lesions, and "fat pads" below the neck in the front and back may also develop over time. Psychological effects, high blood pressure, diabetes, and osteoporosis (softening of the bones) may develop. Cataracts can occur. A very rare condition known as aseptic necrosis of the hips requiring hip replacement may develop. Prednisone also decreases the immune defenses to certain types of fungal infections and atypical bacterial agents. It is important to obtain the list of adverse effects, review them, and monitor them.

Cyclophosphamide (Cytoxan) 

Cytoxan is one of the original anti-cancer medications and may be used for its immune-suppression properties to slow the fibrosing and scar-forming process. There have been some successful reports on a case-by-case basis for individual patients. Effectiveness has not been confirmed by large epidemiological studies. The major adverse effects include bone marrow suppression with decrease in the white cells and increase in susceptibility to unusual viral infections, fungal infections, and atypical bacterial infections. Bladder inflammation with bleeding may occur. Azathioprine (Imuran)

Imuran is also one of the original anti-cancer medications and may be used for its immune-suppression properties to slow the fibrosing and scar-forming process.

As with Cytoxan, there have been some successful reports for individual patients, yet effectiveness has not been confirmed by large epidemiological studies. The major adverse effect of Imuran is bone marrow suppression with decrease in the white cells and increase in susceptibility to unusual viral infections, fungal infections, and atypical bacterial infections.

Study Protocol - Gamma Interferon (Actimmune)

The interferons (alpha, beta, and gamma) are naturally occurring substances in the body that were initially identified as a defensive mechanism against virus infections. Recently, these agents have been used for their benefit in a variety of fibrosing and scar-forming disease states.

A few years ago, gamma interferon was shown to be effective in the fibrosing process of IPF/UIP in several patients. Gamma interferon appears to be needed to successfully control the fibrosing process of a lung injury. In some individuals with IPF/UIP, there is insufficient gamma interferon in the lungs. Therefore, replacement of the gamma interferon may be effective in stopping the fibrotic process. Because of this possibility, gamma interferon was tested in a large Phase III medication study among 330 patients with IPF/UIP. The study used 200 micrograms of gamma interferon (Actimune) given just beneath the skin, three times weekly for about one year.  In September 2002, preliminary study results of this study suggested that there were trends for less shortness of breath after 48 weeks of treatment and less oxygen use in individuals receiving gamma interferon (Actimune). There was also a trend in improved survival for individuals receiving the gamma interferon, yet this is not statistically significant. Patients with a vital capacity greater than 60% of predicted at the beginning of the study tended to do better that similar individuals not taking the gamma interferon. Additional information is needed to confirm these findings, which will be available when the study extension data collection is completed.  Study Protocol - Pirfenidone Pirfenidone is an anti-fibrotic agent that blocks fibroblastic cell proliferation. Pirfenidone was tested in Europe for several years. A September 2002 preliminary report of a new study in Japan indicates that among 111 patients, there was a trend for improved oxygenation and shortness of breath. The medication was generally well tolerated by patients. InterMune, a company in California, that manufactures gamma interferon, plans to conduct studies of Pirfenidone to confirm these positive findings.

Oxygen Therapy

Supplemental oxygen can be very beneficial of individuals with IPF/UIP. The heart has to work harder because of the increased blood pressure in the lungs from the fibrosis. Over time, this extra burden will soon cause the heart to fail. Supplemental oxygen protects the heart by preventing the heart from enlarging and weakening. 

Oxygen is generally used 24 hours daily. This recommendation is based on a large supplemental oxygen study. Using oxygen for 12 hours or less shows no benefit. From 12 to 18 hours shows some benefit. Patients using oxygen over 18 hours daily have total benefit. The use of oxygen 24 hours daily may seem excessive at first, but the oxygen becomes part of the daily routine and helps individuals lead a more active, zestful life as well as preventing a failing heart.

Antibiotic Therapy

Antibiotic treatment for a respiratory infection may be needed during the course of IPF/UIP. Symptoms from a cold in Individuals with IPF/UIP can be treated as usual; however, if a cough develops productive of yellow or especially green phlegm, early antibiotic treatment is usually recommended.

Preventive Therapy

Annual influenza vaccination is generally recommended for individuals with IPF/UIP.

Colds are due to viruses that spread from person to person by touching or contacting mutually used objects such as tissues, doorknobs, computers, telephones, cups, forks, knives, spoons, food, or drink. There are two important preventive measures. First, when near someone with a cold, avoid coming into contact with mutually used objects, and frequent hand washing can be very helpful. Second, do not touch your face with your hands.

Pulmonary Rehabilitation and Routine Exercise Program

A pulmonary rehabilitation program is a fundamental part of managing IPF/UIP. Exercise is very useful for improving conditioning and efficiency of the muscles. The rehabilitation program is useful because an individual can be pushed to a higher level of exertion in a protected environment. The exercise program (e.g., 30 to 45 minutes of daily walking) can be continued on a home basis or at an exercise facility after the rehabilitation program. 

Lung Transplantation

Lung transplantation has become an established treatment in patients who have disabling symptoms from IPF/UIP and other forms of fibrotic lung diseases. There are about 2,000 to 2,500 lung transplants per year. 

Evaluation for lung transplantation is obtained soon after the diagnosis of IPF/UIP, especially for patients under the age of 55 years without complicating medical illnesses and if the diffusing capacity is less than 40% of the predicted value. The age eligibility criterion varies from center to center and is often dependent on other complicating medical conditions. The short-term complications include bleeding and infection while the long-term complications include organ rejection such as bronchiolitis obliterans. It is important to contact the transplant center early to obtain eligibility criteria and to discuss the procedure, benefits, complications, and alternatives with the medical/surgical transplant team. The outcome varies from 70% to 80% one-year survival and 50% to 60% five-year survival with about 20% of patients living 10 years post transplant. These statistical outcomes are improving on a yearly basis. Lung transplantation offers the highest opportunity for restoration of lung function, and is an important option for patients in an appropriate situation.

Smoking Cessation

Individuals with IPF/UIP who smoke have more severe symptoms and a more severe clinical course than individuals who do not smoke. Therefore, all individuals should stop smoking. There are many different ways to stop. If an individual is not able to stop, studies indicate that an organized smoking cessation program is helpful and a program that includes a nicotine substitute is generally recommended.

Outcome of IPF/UIP

IPF/UIP is a progressive disorder with gradual replacement of healthy lung tissue with scar. The progression may be rapid in terms of months or very slow in terms of 15 to 25 years. The average course of the illness is 5 to 8 years. There have been reports of situations where the scarring process has stopped.

Long-term prednisone does not seem to alter the time course of the illness. Active studies such as gamma interferon and Pirfenidone are being conducted to find a medication to alter this course. Successful lung transplantation will change the course of the illness.