Interstitial lung disease (ILD) is a risk factor for lung cancer development and is frequently observed in patients with lung cancer. Individuals with ILD have been excluded from most prospective clinical trials of lung cancer therapies because of the risk of ILD acute exacerbation. Thus, the optimal anticancer drug treatment for such patients has yet to be established. Tyrosine kinase inhibitors are avoided for the treatment of advanced non–small cell lung cancer (NSCLC) with ILD because of the concern of acute exacerbation, and information on the effects of immune-checkpoint inhibitors is limited in these patients. Only three prospective single-arm studies of cytotoxic chemotherapies for advanced lung cancer with ILD have been reported. Based on the results of these studies and those of retrospective analyses, carboplatin and either paclitaxel or nab-paclitaxel are often selected in daily clinical practice for patients with NSCLC and ILD, whereas platinum plus etoposide is selected for those with small cell lung cancer and ILD. Although the antitumor activity of first-line platinum-based chemotherapy appears similar in advanced lung cancer patients with and without ILD, its impact on overall survival of the former patients is limited. The risks and benefits of chemotherapy must therefore be carefully explained before treatment initiation, and careful follow-up is necessary for such patients, especially those with the usual interstitial pneumonia pattern, a risk factor for chemotherapy-related exacerbation. Prospective clinical studies with large patient populations are still required to establish the appropriate treatments for advanced lung cancer with ILD.
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