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We describe three cases of acute exacerbation of interstitial lung diseases (ILDs) in which patients were treated with pulsed-doses of corticosteroids followed by nintedanib and maintenance doses of corticosteroids. All cases responded well to pulsed-dose corticosteroids. However, in conventional practice, corticosteroids can complicate adverse events, including opportunistic infections, diabetes, and osteoporosis. One of the cases reported here involved dermatomyositis-associated ILD with anti-EJ antibodies. Considering possible side effects of corticosteroids and the frequent recurrence of ILDs associated with anti-EJ antibodies, we decided to use nintedanib as a sequential treatment for acute exacerbation of ILDs. Nintedanib has just been approved for treatment of progressive fibrosing ILD, but to date, few reports of acute exacerbation of ILDs treated with nintedanib have been published. This case series may contribute to a more thorough discussion regarding the use and timing of nintedanib in treating acute exacerbation of ILDs.