Abstract

Management of interstitial lung disease, especially idiopathic pulmonary fibrosis, is both difficult and unsatisfactory. In many patients, only supportive therapy can be instituted. Attempts have been made to use anti-inflammatory therapy to reverse inflammation, provide symptomatic relief, stop disease progression, and prolong survival; the results of such treatment have varied from no improvement to significant prolongation of survival. Corticosteroids are the most frequently used anti-inflammatory agents. Cytotoxic drugs, such as oral azathioprine or intermittent intravenous cyclophosphamide, have also been shown to be effective both alone and in combination with low-dose oral corticosteroids. Of the other antifibrotic drugs that have been used, colchicine seems to provide some benefit. It is especially useful in aged persons and those with corticosteroid-induced problems or concomitant illnesses that are likely to be worsened by steroids. Anti-inflammatory therapy is costly to administer and monitor, particularly in the developing world. It is therefore important to consider these issues before instituting treatment. Younger patients and patients with less-severe disease of recent onset are most likely to respond to treatment. Similarly, patients with lymphocytic alveolitis or desquamative interstitial pneumonia respond better. Despite the use of newer strategies for treatment, the overall prognosis for patients with interstitial lung disease has not really changed, and the median population survival remains almost the same as it was about 30 years ago.

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