What are the Treatments?

Once scar tissue is laid down in the lung, no surgery or medication can remove or dissolve the scar.  It becomes a permanent part of the lung.  Therefore, the goal of current treatments is to prevent the formation of this scar tissue in the first place.  There is no evidence that any treatment improves survival or the quality of life for patients with IPF.  However, the poor prognosis and the potential for a positive outcome have prompted physicians to treat these patients anyway.  Most patients will probably be eligible for at least a trial of treatment when first diagnosed.  Given the limited success of current treatments, however, some patients may find that the potential benefits of any treatment are outweighed by the known risk of treatment-related complications.  These might include those over age 70, extremely obese patients, those with major heart disease, diabetes or osteoporosis, those with severe impairment of pulmonary function, and those with endstage fibrosis or scarring on their X-rays. 

For those who will be offered treatment, it is not clear when this should be started.  It is believed that the earlier treatment is started, the better the chances are of a beneficial response.  However, given the lack of proven benefit and the known risk of treatment-related complications, there is no reason to treat someone without any symptoms or evidence of impairment.  Therefore, these patients may be followed for several months to look for evidence of worsening before starting treatment.

Prednisone, cyclophosphamide, azathioprine and colchicine are some of the more common drugs used to treat IPF.   These are immunosuppressants which may help slow down the progression of disease by suppressing the inflammation which appears to lead to the scarring.  Corticosteroids (prednisone) are the primary treatment for IPF.  Corticosteroids are taken as a pill every day.  Common minor side effects include increased appetite, weight gain, salt and water retention, mood disturbances, and muscle and joint aches, among others.  It is important to be closely monitored by a physician while taking corticosteroids, and they should not be stopped suddenly.  It requires about 3 to 6 months before the effectiveness of any treatment can be assessed.  About one third of patients overall experience a beneficial response to corticosteroids, and any improvement which may occur is usually noted within 3 months.  It’s not clear why some patients respond to corticosteroids and some do not.  However, responses are usually partial and transient.  Those patients who exhibit objective stabilization or improvement are then maintained on prednisone chronically (sometimes indefinitely).  Other immunosuppressive or cytotoxic agents (eg. cyclophosphamide or azathioprine) have been considered as second-line treatments for those whose disease progresses despite corticosteroids or those who cannot tolerate corticosteroids.  A few small trials have noted initial improvement in 15 to 50 percent of cases with these agents.  All of these drugs have potentially serious side effects and must be closely monitored.  Finally, drugs which inhibit the formation of scar tissue in the lab are now being tried in patients.  Colchicine, for example, appears to have efficacy similar to corticosteroids.

Until adequate studies are done to define the best treatment for patients with IPF, the current recommendations are to treat appropriate patients with a combined regimen of corticosteroids and either azathioprine or cyclophosphamide.  Patients should be treated for at least six months, barring any complications or adverse effects of the medications.  Studies should be done every six months to assess response, and treatment should be stopped or changed if the patient is worse.  Treatment should be continued only if there is objective evidence of continued improvement or stabilization.

In addition to drug treatment, all patients will benefit from quitting smoking.  Cigarette smoking has been associated with more progressive disease.  Patients should also be encouraged to enroll in a pulmonary rehabilitation program.  Although there are again no carefully defined studies proving the benefit of exercise, experts agree that routine exercise will likely improve symptoms and quality of life. 

Lung transplantation has been offered to patients with IPF and holds promise for young persons without other significant illnesses and with progressive severe disease that is unresponsive to other forms of treatment.  Single lung transplant is currently the preferred surgical operation.  Placing a patient on the list early is important, since waiting time may exceed two years due to limited donors.  Approximately 50% of patients will survive for five years after transplantation. 

Table 2.  Guidelines for the selection of patients with IPF for lung transplantation

General Criteria

• Severe impairment with oxygen dependency

• Medical therapy ineffective

• Deteriorating course of disease

Relative contraindications to transplantation

• Presence of systemic disease that will complicate recovery

• Presence of significant psychosocial dysfunction that prevents compliance with treatment

• Presence of previous major cardiothoracic surgery, due to scarring

• Presence of high-dose corticosteroid therapy that prevents wound healing

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