| Objectives:To explore the clinical features and risk factors in idiopathic inflammatory myopathies(IIM)patients(including classic dermatomyositis(CDM),polymyositis(PM)and clinically amyopathic dermatomyositis(CADM))died in hospital or within 2 weeks of hospital discharge.Methods:We retrospectively analyzed the clinical data of CDM,PM and CADM patients admitted to the First Affiliated Hospital of Zhejiang University from February 2011 to February 2019.The deceased group was defined as the IIM patients who died in hospital or within 2 weeks of hospital discharge,while the survival group was defined as the survival patients during the same period.The deceased and survival groups were age-and sex-matched by a ratio of 1:2.The clinical,serological,therapeutic features and complications were described.Risk factors for the deceased patients wereidentified by logistic regression analysis.Results:The in-hospital mortality rate of CDM,PM and CADM patients in the First Affiliated Hospital of Zhejiang University was 9.4%.The median age of 40 deceased patients was 61.5(48.8-66.0)years,and the ratio of female and male was 1.5:1.The median hospitalization time was longer and anorexia,fever,cough,heliotrope rash,dysphagia were more common in the deceased group than in the survival group(n=80).Serological parameters including ferritin,C-reactive protein(CRP),neutrophil-to-lymphocyte ratio(NLR),C-reactive protein-to-albumin ratio(CAR),AST/CK,red blood cell distribution width(RDW),total bilirubin(T-BIL)and alkaline phosphatase(ALP)concentrations were higher in the deceased group(P<0.05).Proportion of patients with high levels of CEA,glutamyl transpeptidase(γ-GT),fucosidase(AFU),and proportion of patients with complications(involving gastrointestinal hemorrhage,progressive interstitial lung disease,pulmonary infection,hemophagocytic lymphohistiocytosis,low T3 syndrome and newly developed arrhythmia)were higher in the deceased group(P<0.05).Steroid pulse therapy and intravenous immunoglobulin(IVIG)therapy were more common in the deceased group.Multivariate logistic regression analysis revealed that higher on-admission disease activity,progressive interstitial lung disease and pulmonary infection were the independent death risk factors for CDM,PM and CADM patients.It was found in subsection analysis that pulmonary infection and respiratory failure were short-term adverse prognostic factors for IIM patients with progressive interstitial lung disease,while heliotrope rash,progressive interstitial lung disease and increased steroid dose therapy were short-term adverse prognostic factors for IIM patients with pulmonary infection.Conclusions:The in-hospital mortality rate of CDM,PM and CADM patients was relatively high.The complications of IIM were more common and the inflammatory response was more severe in the deceased group.Higher on-admission disease activity,progressive interstitial lung disease and pulmonary infection were the independent death risk factors for CDM,PM and CADM patients.Therefore,it was necessary to closely monitor above indicators during hospitalization for early indentifying adverse prognostic factors and timely adjusting treatment to improve prognosis. |