The evolution of thinking and approach in patients with Idiopathic Pulmonary Fibrosis (IPF) is undergoing some fundamental change, specifically as it applies to treatment.  The first era in this evolution of thought was when IPF was recognized as a separate and specific entity apart from other diseases causing progressive scarring in the lung tissue. The ability to make this diagnosis accurately remains challenging and may require tissue biopsy in more difficult cases.

The next evolutionary era came with the development of drugs for the treatment of IPF.  Since IPF is a relatively uncommon disease, it took some time for pharmaceutical companies to invest in drugs which could alter the progression of scarring in the lungs.  There are two drugs currently on the market for patients with IPF.  These drugs come with some baggage.  Both drugs are, as you might expect, costly.  The main side effect associated with these drugs is gastrointestinal, making it difficult for many patients to tolerate full daily doses.

Last in the sequence of events is the development of data from clinical studies showing that combination therapy, i.e. using both drugs together, may be more effective than one drug, alone. This era of development brings with it more challenges in terms of cost and tolerability.  Unfortunately, neither drug, either alone or in combination, can cure this disease, making it hard to make the case with patients to take the drugs and with insurers to pay for them.  Let’s hope the next era is more promising.