Idiopathic pulmonary fibrosis
was first described in the English medical literature in 1933 by Dr. Hamman and Dr. Rich. They described the abnormalities of advanced pulmonary fibrosis. For the next 80 years there were many rounds of refinement in the clinical and pathologic description of IPF but no progress in treatment. In the published literature, the average life expectancy in the absence of treatment is about 3 years. Less than 30% of patients will survive 5 years after diagnosis. However, caution must be used in applying this information to any specific patient as patients that were included in this analysis had symptomatic disease. Patients that are identified incidentally or with very early disease would be expected to survive much longer. Furthermore, we have entered a new era of IPF where we finally have medications to offer patients, OFEV (nintedanib) and Esbriet (pirfenidone). These medications appear to improve survival. We are very optimistic that patient outcomes will continue to improve at a rapid pace.
A variety of studies have contributed to our current understanding about factors affecting prognosis for idiopathic pulmonary fibrosis patients. In general patients with IPF lose about 150 to 200 ml/year of lung capacity. Many patients may have long periods of stability while others will have a more progressive course. There is no single pattern. Overall, most patients experience a very gradual decline that is punctuated by more abrupt episodes of increased symptoms (such as cough and shortness of breath). These episodes are called “exacerbations” and can be life-threatening.
Factors Affecting Prognosis in Idiopathic Pulmonary Fibrosis
- Older Age: older age at diagnosis is associated with a worse prognosis
- Gender: Women tend to have better survival than men
- Lower body mass index: declining weight is associated with a worse prognosis
- Lower exercise capacity: more severe symptoms that are associated with reduced exercise capacity is associated with a worse prognosis
- Pulmonary Function Testing: the worse the pulmonary function test results are in general the worse the prognosis. Also, declining pulmonary function tests indicate a worse prognosis
- Severity of radiographic fibrosis: more severe fibrosis on CT scanning is associated with worse outcomes
- Certain medical conditions: the presence of emphysema or pulmonary hypertension are associated with a worse prognosis
- History of respiratory exacerbations: patients with periods of increased symptoms or hospitalization are at increased risk for progressing
What can I do to improve my prognosis?
As with any chronic medical problem, some things are out of your control and others are under your control.
First, find a medical team that you trust and follow their advice. Your doctor should be knowledgeable about IPF especially the recent advances. We have learned a few important things in the past 2 years. For example we now know there is no role for immunosuppression as primary treatment for IPF. This means that if your doctor is prescribing high dose prednisone, azathioprine, Imuran, CellCept or mycophenolate then you should consider a second opinion.
Second, you should have an active discussion with your team about the two newly approved therapies Esbriet (pirfenidone) and OFEV (nintedanib). While they are not appropriate for every patient, every patient should at least be considered.
Third, get in shape and find a healthy lifestyle. Most patients with chronic lung disease feel better when they engage in an exercise program. Discuss pulmonary rehab (a structured supervised exercise program) with your team. Eat right—a balanced healthy diet. Obesity is an added stress on the respiratory system. Seek your healthy weight. Of course, never smoke!
Fourth, don’t isolate yourself. Social contact is an important part of good health. Depression is common in patients with chronic lung disease. Don’t be afraid to discuss this with your team. Don’t isolate yourself. Talk to your partner about your feelings. A support group for patients with chronic lung diseases may be useful.
Fifth, think positively but plan ahead. Everyone should have an advanced directive and a will or trust. Take care of these items so that you don’t need to worry.
Lastly, be a joiner. Participate in a clinical research study or join an IPF support group. The progress we have made in idiopathic pulmonary fibrosis treatment is due to the generosity of patients that were willing to participate in clinical research studies. Without their contribution, we would still have no treatments.
Research and Idiopathic Pulmonary Fibrosis
Why is research important? Without research we would not make any progress in understanding or treating IPF. There are several different types of 
research. Basic science research seeks to understand idiopathic pulmonary fibrosis at a cellular or molecular level. For example, what types of enzymes or proteins are abnormal in patients with IPF. Animal studies offer another avenue to study IPF. There are rodent models of IPF that allow scientists to study the disease. Clinical research involves patients with disease. This maybe a treatment trial or a genetic study that seeks to identify a gene that contributes to the disease process.
Patients may participate in many ways. In a clinical trial, a drug company usually seeks to study a specific medication. There are strict guidelines called inclusion and exclusion criteria for who may participate. Most studies are randomized placebo controlled. This means that neither the doctor nor the patient knows if they are getting active treatment or placebo (sham treatment or sugar pill). This study design is required to bring new drugs to market.Patients are required to sign a consent form acknowledging that they have been educated about the trial and are willing to participate. The participant can withdraw their consent at any time. Patients may search on the internet to learn about active research studies. A government website clinicaltrials.gov lists all FDA sanctioned studies.
Doctors with particular interest and expertise in IPF tend to be involved in clinical research. So finding a specialist that is involved in clinical trials related to idiopathic pulmonary fibrosis is often a good start to finding the right doctor.