Objective To study the risk factors associated with death of patients suffered from acute exacerbation of interstitial pneumonia (AE-IP) with very high mortality and very difficult to handle so as to find the most suitable treatment strategy for these patients.Methods The data of 26 patients with AE-IP admitted to Emergency Intensive Care Unit in Peking Union Medical College Hospital from September 2010 to September 2013 were restrospectively analyzed.Comparison of general condition of patients,treatment strategy and response to non-invasive ventilation of patients was made between survival group and death group.Results There was no significant difference in general condition of patients between death group and survival group.But compared with idiopathic pulmonary fibrosis (IPF),connective tissue disease-related interstitial pneumonia had a tendency to accounting for higher proportion in the survival group.The rats of endotracheal intubation and invasive ventilation were significantly increased in death group (14％ vs.84％,P ＜ 0.05).There was significant improvement in PaO2/FiO2 after non-invasive ventilation for 2 hours in the survival group (176 ± i10 vs.218 ±64,P ＜0.05) while the death group had no significant improvement.Conclusions In patients with AE-IP,connective tissue disease associated interstitial pneumonia might have better outcome than IPF.AE-IP patients have a very high mortality rate once patients intubated,thus the decision of intubation in such patients needs to be very cautious.The patients with negligible improvement in PaO2/FiO2 after non-invasive ventilation for 2 hours may have a poor prognosis.