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.