Prognosis

The prognosis for interstitial lung diseases and non-idiopathic pulmonary fibrosis is extremely variable. However, in general the prognosis for non-idiopathic ILDs and pulmonary fibrosis is better than the prognosis of IPF. The clinical course of ILDs and non-idiopathic pulmonary fibrosis largely depends on the cause of the lung disease, whether a specific treatment for the underlying cause is available and how each individual responds to treatment. Having an accurate diagnosis and treatment plan is critical.

Idiopathic Pulmonary Fibrosis

Prior to the development of the anti-fibrotic drugs, the median survival of all patients with IPF was about 2.9 years from the time of diagnosis. This meant that 50% of people diagnosed with IPF died within 3 years of their initial diagnosis. Another way to interpret this statistic is that 50% of people diagnosed with IPF live longer than three years from the time of their initial diagnosis. Importantly, this statistic does not take into account several important aspects of IPF.

1. The development of the anti-fibrotic medications. Although neither anti-fibrotic medication cures IPF, both have been shown to significantly reduce the rate of progression. Because of their recent arrival to the market, their impact on mortality has yet to be determined.
2. The severity of IPF at the time of diagnosis. We are now able to diagnose IPF earlier in the disease course and if we start treatment at an earlier time point in the disease process, we may be able to have a larger impact on disease progression.
3. Not everyone with IPF experiences the same rate of lung function decline. We now recognize that some patients with IPF experience a very slow rate of lung function decline while others progress much more rapidly. We are not yet able to determine definitively which patients are more or less likely to experience a rapid progression of disease. Here again, the implementation of anti-fibrotic medications may significantly alter the course of disease progression.