Patients with a scarring disease of the lungs, called pulmonary fibrosis, were recently disappointed when the drug Pirfenidone was not approved by the FDA for its treatment. Unfortunately, this drug has not been found to be substantially effective in stabilizing this disease. Some patients thought that it would dissolve the scars but, unfortunately, once the scars occur they cannot be removed or dissolved.
One of the first things I like to stress when evaluating a new patient is that we must have a correct and accurate diagnosis. Nowhere is this more important than in pulmonary fibrosis. This disease comes in a variety of types, each with its own causes and long term outlooks. Some of these scarring diseases do have treatments, so knowing which variety you may have is very important. Making the correct diagnosis may involve something as simple as checking the medication list for drugs that could be the culprit. Often sophisticated lab tests and studies need to be ordered, and in many cases an actual lung biopsy is needed. Patients do not always want to proceed in this manner, but when necessary, the information found on biopsy can mean the difference between effective treatment and unnecessary treatment.
Keep in mind that no treatment has yet been discovered that will erase the scarring once they occur. However, when treatment is appropriate, slowing or stopping the scar formation can mean the difference in long term comfort and survival. Insist on the most accurate diagnosis possible.
