The prognosis of patients with dermatomyositis with interstitial pneumonia is related to the condition of interstitial pneumonia, co-infections, and respiratory distress, and cannot be generalized. Early active and effective treatment can improve the prognosis of patients. The prognosis of patients with dermatomyositis with interstitial pneumonia is related to the condition of interstitial pneumonia. Interstitial pneumonia can be categorized into acute, acute and chronic according to the changes in the condition, and patients with chronic interstitial pneumonia have a better long-term prognosis. Respiratory failure and lung infection are the main causes of death in patients with dermatomyositis with interstitial pneumonia, and patients are susceptible to lung infections because of low immunity due to long-term glucocorticoid and immunosuppressant therapy. Therefore, patients with acute interstitial pneumonia, co-infections, and respiratory distress should be treated more aggressively and effectively to improve the prognosis as much as possible. Early detection, diagnosis and treatment can help improve the prognosis of patients. Patients are advised to maintain a positive and optimistic mindset and follow the doctor’s instructions for regular treatment as early as possible.